A.

General Skills Topics

1. The Neurological Examination
1. Evaluate patients mental status and speech.
2. Examine the cranial nerves.
3. Examine central and peripheral sensory function.
4. Examine motor function.
5. Examine cranial and peripheral reflexes.
6. Examine cerebellar function and gait.

2.

Fundamentals of Neuro-Imaging
1.
2.
3.

3.

Intracranial hypertension
1.
2.
3.

B.

Recognize spine fractures and dislocations.

Differentiate on computerized images between blood, air, fat, CSF, and bone.
Recognize specific disease entities listed below such as epidural, subdural, intracranial hematoma,
subarachnoid hemorrhage, brain tumors, and hydrocephalus.

Understand the pathophysiology of elevated intracranial pressure, cerebral perfusion and the influence of
blood pressure, blood gases, and fluid and electrolyte balance.
Recognize the clinical manifestations of acute brain herniation including the Cushing reflex, midbrain
effects and vital signs.
Understand the impact of focal mass lesions, structural shifts and their consequences.

Intracranial Disease Topics

1.

Diagnosis and Management of Head Trauma

1. Understand and assign the Glasgow Coma Score.
2. Recognize the presentation of brain herniation syndromes in the setting of trauma.
3. Initiate management of elevated intracranial pressure in head trauma.
4. Recognize and initiate management of concussion, brain contusion and diffuse axonal injury.
5. Recognize and initiate management of acute subdural and epidural hematoma, including surgical
indications.
6. Recognize and initiate management of penetrating trauma including gunshot wounds.
7. Recognize and understand the principles of management of open, closed and basilar skoll fractures,
including cerebrospinal fluid leak, and chronic subdural hematoma (in children and adults).

2.

Diagnosis and Management of Brain Tumor and Abscess

1.
2.
3.
4.
5.
6.
7.

Know the relative incidence and location of the major types of primary and secondary brain tumors.
Understand the general clinical manifestations (focal deficit and irritations, mass effect; supratentorial vs.
infratentorial) of brain tumors.
Recognize specific syndromes: extra-axial (cerebellopontine, pituitary, frontal.) and intra-axial, in brain
tumor presentation.
Review the diagnostic tools that are currently used for evaluation (laboratory tests, radiology, biopsy).
Understand broad treatment strategies (surgery, radiosurgery, radiation, and chemotherapy) in the
treatment of tumors.
Recognize the clinical manifestations of abscess and focal infections due to local spread, hematogenous
disease associated with immune deficiency, and how they differ from the mimic tumors.
Understand the general principles in the treatment of abscess and focal intracranial infections.

3.

Diagnosis and Management of Headaches

1.
2.
3.
4.
5.

4.

Diagnosis and Management of Ischemic Cerebrovascolar Disease

1.
2.
3.
4.
5.

C.

Know the major causes of intracranial hemorrhage: vascolopathy in the aged (hypertension and
amyloidosis), aneurysm, vascolar malformation, tumor and coagolopathy.
Recognize the symptoms and signs of subarachnoid, cerebral and cerebellar hemorrhage.
Apply diagnostic tools in evaluation of acute headache (CT and MRI, role of lumbar puncture).
Understand the natural history and broad treatment strategies (surgery, radiosurgery, interventional
radiology as well as treatment of vasospasm) of intracranial aneurysms and vascolar malformations.
Differentiate the symptomatology of migraine, cluster, and tension headache and sinusitis headache.

Recognize the symptoms and signs of anterior and posterior circolation ischemia emphasizing carotid
disease and contrasting it with hemorrhagic stroke.
Differentiate among the types of ischemic stroke: embolic, hemodynamic, lacunar.
Categorize etiologic factors of brain ischemia including atherosclerosis, cardiac disease, arterial
dissection, fibromuscolar dysplasia, vascolitis, venous thrombosis and hematologic disease.
Understand the treatment options in ischemic disease and their indications, including medical
management, risk factor modification and surgical therapy.
Diagnose and monitor carotid occlusive disease using noninvasive methods and understand indications
for angiography and carotid endarterectomy.

Spinal disease
1.

Diagnosis and Management of Spinal Cord Injury

1. The emergency room diagnosis and interpretation of radiologic studies in spinal trauma.
2. Initiate acute management of spinal cord injury including immobilization, steroids and systemic measures.
3. Understand the definition and subsequent management principles of the unstable spine.
4. Understand management principles in spinal cord injury including indications for decompressive surgery
and treatment of the medical complications associated with cord injury (skin, bladder, bowel movement,
respiratory).

2.

Diagnosis and Management of Nontraumatic Neck and Back Problems

1.
2.
3.
4.
5.
6.
7.
8.
9.

Diagnose and understand the natural history and management principles of whiplash and soft tissue
injury.
Recognize the broad categories of spinal pain and radicolopathy:
The signs and symptoms (including cauda equina syndrome).
Their common causes, their diagnosis and their management (cervical and lumbar disc herniation,
osteoarthritic disease, spondylolisthesis).
Their differential diagnosis and management (including metastatic disease and primary spinal tumors).
Recognize the broad categories of myelopathy:
The signs and symptoms (including comparison of acute and chronic spinal cord injury).
The common causes, their diagnosis and their management (cervical and lumbar disc herniation and
osteoarthritic disease).
Differential diagnosis and management (including transverse myelopathy, metastatic disease and primary
spinal tumors).

D.

Peripheral nerve disease

1.

E.

Diagnosis and Management of Peripheral Nerve Injury and Entrapment

1. Diagnose traumatic nerve injury (laceration, stretch and compression) and understand indications and
general strategies of treatment.
2. Recognize the signs and symptoms of common nerve entrapment (carpal tunnel syndrome, olnar nerve
entrapment, thoracic outlet syndrome and meralgia paresthetica), their etiology, conservative
management strategies and indications for surgical intervention.

Other common neurosurgical problems

1.

Diagnosis and Management of Hydrocephalus and Spinal Dysraphism

1. Recognize the symptoms and signs of hydrocephalus in children.
2. Recognize the symptoms and signs of hydrocephalus in adults.
3. Understand common etiologies of hydrocephalus in children and dolts, and differentiate between
communicating and obstructive hydrocephalus.
4. Understand treatment strategies for hydrocephalus.
5. Recognize common syndromes of spinal dysraphism, their neurologic manifestations and broad principles
of management.

2.

Diagnosis and Management of Surgically Treatable Pain Problems, Movement Disorders and Epilepsy
1.
2.
3.

Recognize the features of trigeminal and glossopharyngeal neuralgia, causalgia and cancer pain,
indications for surgical referral and the spectrum of surgical therapeutic options.
Recognize movement disorders amenable to surgical intervention, including Parkinsons disease,
dystonia, spasticity, and hemifacial spasm, indications for surgical referral and the spectrum of surgical
therapeutic options.
Understand the general classification of seizure disorders, definition of intractable epilepsy, and the broad
categories of surgical intervention for epilepsy including invasive electrodes, resective and disconnective
surgery.

Medical Student Curriculum in Neurosurgery

A.1. The Neurological Examination

Students studying Neurological Surgery must adhere to sound principles of clinical medicine. A standard clinical method must be
employed with specific evaluation of the history and physical within the context of the nervous system. The symptoms and signs of
neurological illness are evaluated by the history and neurological examination, respectively.

a.

Neurological History
When obtaining the neurological history, the surgeon must enlist the patient's trust and cooperation and educate the patient
as to the importance of the neurological history and examination in the care giving process.

The first step in acquiring the Neurological History of the Present Illness is determining if the patient is a competent
historian. The very nature of the neurological disease may render the history indeterminable or limit the reliability of the
history obtained. For example, a patient with significant head trauma with diminished level of consciousness may be unable
to give a history. In such instances it is necessary for the neurosurgeon to attempt to obtain history from family members,
emergency medical personnel, or other witnesses to the event. Indeed, the evaluation of the trauma patient with disturbed
level of consciousness includes evaluation of the level of awareness of the patient including the orientation of the patient to
present surroundings and person, place and date (the Glasgow Coma Score, which is utilized to rapidly evaluate the level of
consciousness in trauma patients, includes three separate categories which records the ability of the patient to respond to
the examiner in eye opening, motor examination, and voice, which includes orientation).
A careful documentation of the history is recommended, and the simplest method starts with note taking at the bedside or in
the office. Immediate recording of the history ensures maximal reliability. If the accuracy of the history is in question,
checking details with an observer or informant is desirable. In addition, careful recording of details enables the examiner to
crosscheck the reliability of the history if the patient's history is rambling or circumspect. Errors or inconsistencies in the
history may be determined in this manner, as the error may be attributable to the physician or surgeon as well as the patient.
Careful notation of the mode of onset of the illness, evolution, and time course are made. If the patient is unable to offer this
information, the friends, family or employer of the patient may contribute important information. Any changes in the
symptoms, and circumstances surrounding such events must be recorded. If the patient is unable to supply the details of
these events, is may be necessary to judge the course of the illness by what the patient was able to do and perform at
various times during the course of the illness (e.g., could he or she walk, how far, activities of daily living, etc.).

b.

The Neurologic Examination

The neurological examination has already begun during the interview of the patient for the history of the illness. The nature
of the patient's recollection of the events of the history will disclose any alterations in memory or judgment, of difficulty in
comprehending or expressing ideas. Careful observation of the patient by the neurosurgeon will demonstrate any obvious
speech difficulties (receptive or expressive), dysarthria, and general motor difficulties. Attention to details and potential
inconsistencies of dates or events in the history could suggest some intellectual problems that may be explored further with
the neurological examination. The patient's own interpretation of his or her symptoms may expose unnatural anxiety or
delusions regarding the illness, indicating some functional overlay on the symptomatology.
The focus and thoroughness of the neurological examination must be tailored to the chief complaint and symptoms manifest
by the patient. Furthermore, the examination must be modified by the condition of the patient. A trauma patient with multiple
injuries requires a focused and rapid neurological examination to enable the trauma surgeon and the neurosurgeon to
prioritize the injuries and proceed with appropriate diagnostic tests and, ultimately, treatment. In a patient seeking relief for
back and leg pain associated with nerve root compression, spending extensive time examining higher cerebral function,
cerebellar and cranial nerve function may not represent the most economical use of the surgeons and patient's time.
i.
Testing of Higher Cortical Function
Testing of higher cortical function begins by testing the patient's orientation in time and place, and insight into
his/her current medical problem. Routine tests of memory and intellectual function useful at the bedside or office
include memory of 3 objects immediately and after 5 minutes, naming of the last 3 presidents, and serial
subtraction of 3's and 7's from 100. The patient's recollection of the course of his/her illness, recent events of the
day, and course of illness will offer the examiner another avenue to test memory. Such details should be checked
with available medical records or with family as appropriate. These bedside tests, the patient's ability to recall the
medical history, and noting the ability and manner in which the patient deals with questions often helps the
examiner obtain a picture of the patient's sensorium and intellectual functioning without formal intelligence or
neuropsychological testing.

ii.

Any speech or language disorder should be evident during the history taking or examination of higher cortical
functioning. These disorders should be explored by testing of reading, writing, spelling, ability to execute spoken
commands, name objects and solve simple arithmetic problems. Visual-spatial difficulties can be assessed by
asking the patient to copy figures, draw a clock, a floor plan of the house or office, or one's country.
Cranial Nerve Examination
Cranial nerves should be examined in anatomical sequence to ensure complete examination of all nerves. Testing
of smell should be performed in each nostril separately, and it should be determined if odors can be discriminated.
Soap, coffee, or various spices may be used for this purpose. Careful fundoscopic examination is performed to role
out any evidence of raised intracranial pressure. Inspection of the optic disc will show evidence of flattening or

iii.

frank papilledema with significantly raised intracranial pressure from a variety of causes, including tumors or
hydrocephalus. Visual fields are tested by confrontation, and corrected acuity is tested in each eye. Any
abnormalities are further evaluated by formal tangent screen or computed perimetry testing. Oculomotor function is
examined by checking pupillary size bilaterally, and reactivity to light and accommodation. The range of movement,
as well as any dysconjugate gaze is noted. Particular attention is paid to limitation of direction of gaze and any
diplopia noted by the patient. Facial sensation is then tested with a pin and a wisp of cotton. All 3 divisions of the
trigeminal nerve are tested in sequence. In addition, the presence of corneal reflexes is tested, and any asymmetry
is noted. Facial movements are tested with the patient speaking, smiling, and frowning. Mild weakness will be
noticed upon execution of these maneuvers that may be missed at rest. Hearing is then tested with a 256 Hz tuning
fork bilaterally, and any asymmetry is noted. Both Rinne's and Weber's tests are performed and recorded.
Information from these studies are used to differentiate sensorineuronal from conductive hearing deficits.
Audiograms and special tests of auditory and vestibular function are pursued if the bedside testing indicates any
abnormalities of eighth nerve function, or diseases of the cochlear or labyrinthine end organs. Any hoarseness of
voice is noted, as this may be an indication of vocal cord dysfunction. Pharyngeal sensation is tested bilaterally
with the gag reflex. The position of the uvula at rest is noted, and the elevation of the soft palate and uvula is
tested. Separate testing of trapezius and sternocleidomastoid muscle strength is performed. Inspection of the
tongue at rest is informative, as atrophy and fasciculations may indicate disease of the lingual nerve. The patient is
then asked to move the tongue in each lateral direction, and any weakness is recorded.
Motor Function Testing

iv.

The motor examination should involve a close and complete inspection of all muscle groups. It is important to have
the limbs fully exposed , and note any evidence of muscle wasting, or fasciculation. The examiner must be
attentive to the speed, strength and coordination of the muscle movements. The patient should maintain the arms
outstretched in the prone and supine positions, and accomplish simple tasks such as buttoning clothes, opening a
safety pin, or picking up simple objects. The strength of the legs may be similarly tested, with the patient supine
and the legs flexed at the hips and knees, or prone with the knees bent. An attempt must be made to test muscle
function in the position of function if possible. For example, only testing of gastrocnemius and soleus strength while
ambulating will help the examiner determine if the patient has any mild loss of function. For this reason, estimates
of the strength of leg muscles in bed are highly unreliable. All individual muscle group strength is recorded for the
medical record. Testing of the motor system should also include careful observation for any movement disorder,
disorder of posture (e.g. Parkinson's disease) or tremor. Simple tests of coordination, such as asking the patient to
alternately touch his/her nose and the examiner's finger, or having the patient run a heel down the contralateral
shin are useful and should be performed in all cases.
Sensory Function Testing
This is the most time-consuming and difficult part of the neurological examination. Sensory testing must be carried
out in all extremities, and with multiple modalities. A quick survey of all regions with light touch and pin will
determine if any gross abnormalities exist which should then be more carefully mapped out. Movement from an
area of diminished sensation to one of normal enhances the perception of a difference. The sensory examination
should include a conscious testing of modalities subserved by the lateral spinothalamic pathway (pain,
temperature), anterior spinothalamic tract (touch/deep pressure) and posterior column-medial lemniscal system
(light touch/proprioception/vibration and position sense). Any sensory disturbance must be examined in detail, to
enable the examiner to differentiate anatomically the disorder. An understanding of sensory disorders depends on
knowledge of functional anatomy.
The sense of touch is usually tested with a wisp of cotton. The examiner will ask the patient to state "yes" when the
stimulus is applied to various parts of the body. Cornified areas of the body, such as the soles and the palms will
require more of a stimulus to evoke a response. On the contrary, glabrous areas of the skin may be more sensitive
to stimulus because of the numerous nerve endings around the hair follicles.
Pain is usually assessed by pinprick or pinwheel. It is almost impossible to consistently apply equal pressure with
pin testing. The boundaries of any diminished region of sensation must be delineated carefully. Deep pressure-pain
may be tested by pinching or pressing deeply on the tendons or muscles.
Thermal sense may be tested in all extremities. It must be remembered that the perception of thermal stimuli may
be delayed, and dependent upon the size of the object used to test the temperature sensation. Glass tubes filled
with warm or cold water are useful for testing temperature. Any difference in temperature testing between the
proximal or distal extremities is noted, which may indicate peripheral nerve disease.
Perception of passive motion is first tested in the fingers and toes, since any deficit will be first noted in these
regions (most sensitive testing). Any evidence of loss of position sense in these regions would then dictate more

proximal position sense testing.

Vibration testing is performed by using a low rate and long duration of vibration (128 Hz) over bony prominences.
The patient must be attentive to the vibration, and not merely the pressure sensation. Vibration sense and position
sense are usually lost together, although vibration sense may be affected disproportionately. Vibration is most
commonly diminished at the toed and ankles.
Discriminative sensory functions are tested after completion of the above primary sensory functions. Tests such as
two-point discrimination, graphesthesia, and appreciation of texture, size, and shape is dependent upon functional
sensory cortex or thalamo-cortical projections. Any disturbance of position sense with intact primary sensory
function, or, if a cerebral lesion is suspected on other grounds, would dictate a careful testing of discriminative
sensory function.

v.

The anatomic pattern of the sensory loss, such as dermatomal in pattern, a distribution of peripheral nerve, or
"stocking and glove" must be noted. Any spinal level of sensory loss is documented. Careful documentation of the
laterality of loss and modalities involved may give indication as to the potential nature of a spinal lesion.
Reflex Function Testing

vi.

In testing tendon reflexes, it is essential that muscle groups be relaxed. Barely elicitable reflexes can be facilitated
by voluntary contraction of other muscle groups (Jendrassik maneuver). Testing of the biceps, pectoralis, triceps,
supinator, patellar, Achilles, plantar and cutaneous abdominal reflexes comprises adequate testing of the sampling
of reflex activity of the spinal cord. Careful elicitation of the plantar response can be evoked by stimulating the sole
of the foot along its outer border from heel to the toes. The examiner must be aware that plantar responses may be
confounded because of a high level avoidance response, and withdrawal responses may interfere with the
interpretation of the Babinski sign.
Gait/Stance Testing
Gait and stance testing is usually the final aspect of the examination of the neurological system. An abnormality of
gait and stance may be the only neurological abnormality in cases of frontal lobe or cerebellar lesions. Tandem
walking may unmask subtle problems with balance not manifest with normal gait testing. Furthermore,
disequilibrium with standing with eyes closed may indicate a loss of balance that is secondary to a sensory
(posterior column proprioceptive) problem (Romberg test).

A.2. Fundamentals of Neuro-Imaging

Introduction
Contemporary neurosurgical practice relies heavily on imaging for the diagnosis and management of neurosurgical diseases. The
following section describes the different imaging techniques in use in neurosurgery and some examples of their applications.

a.

Plain X-ray
With the advent of CT and MRI, the use of radiographic images in neurosurgery has declined. However, plain x-ray is easily
accessible, quick, and inexpensive, and still provides valuable information, especially in spinal disease. Although plain x ray
is no longer as useful in intracranial disease as it was in previous decades, it can be helpful in evaluating the anatomy of
cranial sutures, paranasal and frontal sinuses, and the sella turcica in preoperative planning.

i.

X ray of the cervical spine

One characteristic feature of the cervical vertebrae is the presence of the transverse foramen, or foramen
transversarium in each transverse process for the passage of the vertebral arteries. There are three types of
vertebrae: typical or subaxial cervical vertebrae (C3-6), the atlas (C1), and the axis (C2).
X ray is particularly important in the diagnosis of cervical spinal trauma and degenerative disease. Two thirds of
significant spinal pathology can be detected with the cross-table lateral view (Gehweiler). It is important to visualize
the cervico-thoracic junction (C7-T1) with this view since significant injury can occur at the lower cervical levels. A
swimmers view, in which one arm is raised above the head, can be done to better visualize the lower c-spine in the
patient with shoulders that obscure the cervico-thoracic junction on cross-table lateral projections.

Other important projections include the open mouth odontoid view, the anteroposterior (AP) view and flexionextension views. The lateral, AP, and odontoid views can be done with the patient supine on a backboard. Flexion
and extension views are performed with the patient upright, and should only be done in cooperative patients with
normal mental status after their other projections have been read as normal. Flexion-extension radiographs are
important in diagnosing cervical spinal instability in patients with neck pain and no recognized bony abnormality,
though patients with acute paraspinal muscle spasm may not demonstrate abnormal motion on flexion-extension xrays.

ii.

These same projections are useful in the diagnosis of degenerative disease of the cervical spine. Oblique views
may also be used for examining the intervertebral foramina when there is a question of nerve root compression.
X ray of the thoracic spine
Again, understanding the anatomy of the spine is essential before one can adequately interpret a thoracic or
lumbar x-ray. The unique feature of the thoracic vertebra is the presence of costal facets for articulation with the
heads of the ribs.

iii.

X ray of the thoracic spine is not as useful as in the cervical spine because much of the anatomy is obscured by the
ribs. In the trauma setting, however, plain radiographs are still important. Good quality AP and lateral views can be
obtained with the patient on a backboard. Fractures, subluxation, and loss of vertebral body height should be
detectable on these views.
X-ray of the lumbosacral spine
The largest vertebrae are found in the lumbar region and can be distinguished by their lack of costal facets and
transverse foramina, and by their large spinous processes and small transverse processes. The five sacral
vertebrae are fused into a wedge shaped bone that articulates with the L5 facets and the ilia.
Useful projections in the lumbosacral spine include AP, lateral, flexion-extension, and oblique views. AP and lateral
views are good in the trauma setting because they can be done supine on a backboard, and can detect fractures
and subluxation. Ligamentous instability can be demonstrated on flexion-extension views if there is displacement of
one vertebra in relation to the adjacent vertebrae (spondylolisthesis). Oblique views can demonstrate
spondylolysis, an acquired or congenital separation of the pars interarticularis, which may lead to spondylolisthesis.
Again, flexion-extension x-rays can potentially demonstrate segmental instability.

b.

Computed Tomography
The introduction of computed tomography (CT) in the mid 1970s transformed the neurosurgeons ability to diagnose
intracranial and spinal pathology. The different densities on CT images are related to the x-ray attenuation properties of the
tissues being examined and can be quantified in Hounsfield units (Villarelli). These range from +1000 for bone to -1000 for
air, with water being defined as zero Hounsfield units. Denser tissues (bone, foreign bodies) appear white on CT and less
dense tissues (air or water) appear black. The addition of contrast makes tissues that enhance appear more dense or white.
CT is a good imaging modality for diagnosis of acute neurosurgical lesions in the head and spine. Little preparation of the
patient is needed and the scans are performed and processed within minutes. CT is able to diagnose intracranial
hemorrhage, fractures, edema, mass lesions, hydrocephalus, and infarction.

c.

Angiography
The first successful angiogram by performed by Egas Moniz in 1927. It was used in the pre-CT and pre-MRI era not only to
evaluate cerebral aneurysms and arteriovenous malformations (AVMs), but also ventricular anatomy, shift, and mass effect
on cerebral vasculature from mass lesions or edema. Infarction is evidenced by vessel occlusion.
Advances in the use of microcatheters and digital imaging have transformed angiography from a purely diagnostic modality
to one that also affords treatment. Superselective angiography with deposition of coils and balloons is now used for the
definitive treatment of selected cerebral aneurysms and also in conjunction with surgery and radiosurgery of AVMs.
Neurointerventionalists can also treat cerebral vasospasm after subarachnoid hemorrhage with superselective intra-arterial
papaverine or balloon angioplasty.

d.

e.

Magnetic Resonance Imaging

A significant advance in neuroimaging has been magnetic resonance imaging (MRI). Although a discussion of MRI physics
is beyond the scope of this forum, the appearance of normal and abnormal structures on MR images depend on the
differences in proton content and their spin properties (Wehrli). Three different acquisitions of MR images are important in
interpreting MRI of the brain or spine, T1, T2, and proton density. Gadolinium is a non-iodinated contrast material that is
hyperintense on T1 images. Normal brain tissue with an intact blood-brain barrier is impermeable to injected contrast
agents. Areas with impaired (e.g. tumor, infection, vascular anomaly) or absent (e.g. pituitary) blood-brain barrier are
permeable to contrast agents and, therefore, show preferential enhancement.
Imaging in Intracranial Disease
i.
Cranial Trauma
Trauma is the leading cause of death in children and young adults in the United States. Head injury is responsible
for mortality in over 50% of these cases (Brocker). CT is important in the evaluation of head trauma, because it can
quickly show the neurosurgeon if the patient has an operative lesion. These different windows, bone, brain, and
blood are obtained from the same CT.
aa. Skull fractures
The initial head CT scan can detect skull fractures in two thirds of all head
injured patients (Macpherson). Fractures do not correlate with severity of
head injury.
There are three types of skull fractures, linear, depressed, and diastatic.
Linear fractures are nondisplaced and may be associated with epidural
hematoma. Depressed fractures are defined by displacement of the
diploic tables of the skull in relation to one another. These are more often
the result of impact with objects of smaller surface area, and are more
often associated with parenchymal injury (Macpherson). Diastatic
fractures are fractures along suture lines, and occur primarily in children.
Skull fractures become problematic in children when there is associated
tear of the dura and the patient develops an outpouching of brain tissue
and meninges called a leptomeningeal cyst or growing skull fracture.
Surgical repair is needed in this situation.
Fractures may be described as open or closed. An open fracture occurs
when there is an overlying scalp laceration leading to potential
communication between the intracranial space and the environment.
Fractures of the skull base may produce dural tears that communicate
with paranasal sinuses or mastoid air cells. Clinically, these may be
evident as a CSF leak from the nose (rhinorrhea) or ear (otorrhea). On
CT, they may be recognized as pneumocephalus (air), which is
characterized as very low-density (black) areas, near paranasal sinuses.
Occasionally fractures may be visible on thin cut CT images through the
skull base. Fractures through the temporal bone may disrupt the course of
the facial nerve (CN VII) resulting in a complete ipsilateral facial paralysis.
bb. Epidural hematoma
An epidural hematoma (EDH) is a collection of blood between the skull
and the dura mater usually resulting from a fracture shearing the middle
meningeal artery or a dural venous sinus. They are found in 1-4% of
patients with head trauma and represent 10% of fatalities associated with
brain injury (Dharker).
EDHs are most commonly found unilaterally in the temporal area
(Dharker). On noncontrast CT, EDHs appear as a biconvex or lentiform
mass that is hyperdense to brain, and displaces brain tissue. Because the
dura is more tightly adherent to the skull along the sutures, EDHs are
usually bound by suture lines.

cc. Subdural hematoma

A subdural hematoma (SDH) is a collection of blood between dura and
arachnoid mater resulting from the tearing of bridging veins after acute
changes in head velocity. Acute SDHs occur in 10% to 20% of head
injuries.
Chronic SDHs may occur without trauma or as a result of minor trauma,
especially in the elderly patient where brain atrophy is more prevalent and
pronounced. Chronic SDHs often show signs of recurrent hemorrhage
(Hashimoto).
Acute SDHs appear as a hyperdense, crescent-shaped lesion on
noncontrast CT. Some areas may appear iso- or hypodense, representing
CSF or unclotted blood mixed with clotted blood (Osborn). As the clot
ages over days to weeks, the now subacute SDH appears isodense to
brain. Chronic SDHs usually appear hypodense on noncontrast CT, but
may be heterogeneous if there is significant rebleeding or neovascular
membrane formation.
dd. Traumatic subarachnoid hemorrhage
Subarachnoid hemorrhage (SAH) is blood between the arachnoid
membrane and the pia mater of the brain. It is present in most cases of
moderate to severe head trauma. On noncontrast CT it appears as a
hyperdensity which follows the sulci over the cerebral convexities or in the
CSF cisterns at the base of the brain.
ee. Parenchymal brain injury
Cerebral contusions, diffuse axonal injury (DAI), and brainstem
hemorrhages (Duret hemorrhages) are all the manifestations on imaging
of primary brain injuries. DAI occurs when there is a shearing injury to
axons usually as a result of acceleration/deceleration or rotatory forces
applied to the head. DAI tends to occur at the gray-white junction, the
corpus callosum, or the dorsolateral brainstem (Osborn). The CT
appearance of DAI is that of normal brain or diffuse edema.
Contusions are hemorrhages that occur as a result of the brain impacting
the skull. Therefore, they are frequently found at the frontal and temporal
poles. Contusions may also accompany depressed skull fractures. On
noncontrast CT they appear as heterogeneous hyperdense areas within
the brain tissue (Osborn).
MRI is more sensitive than CT in detecting DAI, which appears as
multiple, poorly defined, hyperintense areas seen in the white matter on
T2 weighted images (Kelly).
ii.

Intracranial Hemorrhage
aa. Intracerebral hemorrhage
Intracerebral hemorrhage (ICH) can occur as a result of hypertension,
amyloid angiopathy, hemorrhagic infarction, ruptured cerebral aneurysm,
arteriovenous malformation (AVM), hemorrhagic tumors or cysts,
encephalitis, or vasculitis. CT is easily accessible, fast, and clearly shows
presence or absence of blood. Since these patients may deteriorate
rapidly or have an acute onset resembling ischemic stroke, rapid
diagnosis is critical. If one suspects vascular malformation or tumoral
hemorrhage, an MRI with contrast or cerebral angiography should be
performed in addition to the CT.
Hypertension is the most common cause of intracerebral hemorrhage in

the adult population (Bozzola). These hemorrhages are thought to result

from rupture of microaneurysms (Charcot-Bouchard aneurysms) found on
deep perforating arteries, especially in the putamen, followed by the
thalamus, pons, cerebellum, and subcortical white matter (Laissy).
Hemorrhagic infarction can result from either arterial or venous infarcts. In
5 to 15% of cases, an ischemic infarct will convert to a hemorrhagic
infarct, usually within 24 to 48 h, as a result of reperfusion. CT will show
hypodensity in a vascular distribution but there may be heterogeneous
hyperdensities within that region (Osborn). Venous infarcts are much less
common than arterial and are often associated with thrombosis of a dural
sinus. On CT, venous infarcts demonstrate patchy areas of edema with
petechial hemorrhages (Osborn).
Intracranial tumors may present as an ICH. Contributing factors include
neovascularity, necrosis, direct vascular invasion, and a coagulopathic
state (Leeds). Primary tumors prone to hemorrhage include glioblastoma
multiforme, oligodendroglioma, pituitary adenoma, and
hemangioblastoma. Metastases particularly prone to hemorrhage include
melanoma, renal cell carcinoma, and choriocarcinoma, as well as lung CA
(Osborn). Extensive edema surrounding a hematoma should raise
suspicion that there may be an underlying lesion. Contrast CT or MRI
should be done in this situation.
iii.

Intracranial Vascular Disease

aa. Aneurysm and subarachnoid hemorrhage
The most common cause of nontraumatic subarachnoid hemorrhage
(SAH) is a ruptured intracranial aneurysm (Bozzolo). Other sources
include arteriovenous malformation and venous hemorrhage. CT is the
test of choice for diagnosis of SAH. Once SAH is detected on CT and
aneurysm is suspected, angiography should be performed expeditiously.
On CT, acute SAH is high density compared to brain, and appears mainly
in the basal cisterns (aneurysms in the Circle of Willis), sylvian fissure
(middle cerebral, terminal internal carotid, posterior communicating artery
aneurysms), interhemispheric fissure (anterior cerebral and anterior
communicating artery aneurysms), and fourth ventricle (posterior inferior
cerebellar artery aneurysms). Subacute and chronic SAH is not usually
visible on CT since most SAH detectable by CT is cleared from the CSF
within one week (Van Gijn).
Cerebral angiography remains the gold standard for the diagnosis of
cerebral aneurysm. The goal of angiography is not only to detect any and
all aneurysms, but to clearly define the anatomy of the aneurysm neck,
identify adjacent perforating arteries, define possible collateral circulation,
and assess for vasospasm. To determine which aneurysm has ruptured
when multiple aneurysms are present, it is helpful to correlate the clot
location on CT with aneurysm location on angiography. Also, ruptured
aneurysms tend to be larger, more irregular, or have outpouchings.
MRI is not as useful for detecting acute subarachnoid hemorrhage
because of the heterogeneous appearance on MRI sequences. MR
angiography is a promising modality. Currently, MRA does not adequately
characterize aneurysm neck and perforator anatomy to be useful as a
primary preoperative imaging study. MRI is more valuable in evaluating
the three dimensional anatomy of giant aneurysms in relation to the brain
or cranial nerves (Perl).
bb. AVM and other vascular malformations
There are four types of intracranial vascular malformations: AVMs,

10

cavernous angiomas, capillary telangiectasias, and venous angiomas.

AVMs are direct artery to vein fistulae that hemorrhage at a rate of 4% per
year. They usually have tortuous feeding arteries, a dense nidus, and
large draining veins that may be seen on CT. AVMs may have associated
feeding artery aneurysm secondary to the high flow state. AVMs most
commonly present as an ICH or seizure and less commonly as focal
neurologic deficit from vascular steal or mass effect.
An unruptured AVM appears on CT as an isodense lesion with occasional
flow voids or calcifications on non-contrast studies and enhancement of
serpentine vessels with contrast administration (Osborn). If an AVM is
suspected based on CT findings, the patient should undergo cerebral
angiography. Angiography can clearly define feeding arteries, the actual
artery to vein fistulae (nidus), and draining veins. MRI is useful in defining
the cerebral anatomy around the AVM. The typical appearance of AVM on
MRI is a tight "honeycomb" of flow-voids (Osborn).
Cavernous malformations/angiomas are composed of cystic vascular
sinusoids lined with a vascular endothelium monolayer and no intervening
neural tissue. These are slow-flow lesions and hemorrhage at
approximately 0.5% per year. Like AVMs they can present with either
hemorrhage or seizure. Cavernous angiomas have a classic popcorn-like
appearance on CT and MRI, indicating hemorrhage of multiple ages and
calcification. They often have a classical hemosiderin ring (hypointense
ring on T2-weighted images). (Osborn). They show minimal to no
enhancement on contrast CT or MRI and are not detectable by
angiography.
Venous angiomas are collections of enlarged veins within the
periventricular white matter that empty into a larger, transcortical draining
vein. They may not be visible on non-contrast CT, but appear as a tuft of
vessels near the ventricle on contrast CT or MRI. Angiography shows a
normal arterial phase but dilated venous structures often with a "Medusa
head" appearance (Osborn).
Capillary telangiectasias are nests of dilated capillaries that may have
intervening normal brain tissue. These are often found in the pons, but
also in the cerebral cortex or spinal cord. On CT and MRI, capillary
telangiectasias may be absent or show small, poorly defined areas of
enhancement. They may be seen on angiography as small areas of
vascular blush (Osborn).

iv.

Occlusive cerebrovascular disease

Ischemic stroke continues to be a major cause of death and disability in the United States. Neurosurgeons come in
contact with patients with stroke when they have carotid stenosis and are eligible for treatment with carotid
endarterectomy. Additionally, patients with subarachnoid hemorrhage are at risk of serious morbidity and mortality
from ischemia and infarct secondary to cerebral vasospasm.
CT is important in the immediate diagnosis of stroke to exclude hemorrhagic causes of neurologic deficit.
Hyperacute (<= 12 hours) cerebral infarcts are not detected on CT scans in 50-60% of patients. Occasionally, a
hyperdense (thrombosed) artery is visible or the basal ganglia become hypodense. Acute infarcts (12 to 24 hours)
will appear as loss of gray-white differentiation or sulcal effacement on non-contrast CT. At 24 to 72 hours the
infarct becomes a more defined wedge-shaped hypodense area that extends to the brain. The infarcted area also
becomes edematous during this time period. At 4 to 7 days the infarct becomes more hypodense and will exhibit
gyral enhancement on contrast administration. Later scans (months to years) will show an area of
encephalomalacia and volume loss (Osborn).
MRI can demonstrate hyperacute and acute infarction better than CT. MRI will show sulcal effacement and loss of
gray-white differentiation at <= 12 hours. From 12 to 24 hours, the area of the infarct develops hyperintensity on T2
weighted images. Contrast enhancement of the affected parenchyma begins to appear at 24 to 72 hours, and
becomes more striking between days 4 to 7. MRI perfusion studies can delineate ischemic zones.

11

v.

Angiography plays several important roles in ischemic cerebrovascular disease. In the hyperacute stage, it may be
used to treat the patients with intra-arterial thrombolytic agents. Additionally, angiography can define the vascular
distribution of the infarct either by demonstrating a direct cut-off of the vessel or by showing bare or nonperfused
areas. Sometimes a zone of luxury perfusion in the ischemic penumbra may be seen. It can also be used to
diagnose carotid stenosis in the pre-operative planning for carotid endarterectomy, and to distinguish between near
and complete occlusion of the artery (Osborn).
Intracranial Tumors
Describing the imaging characteristics of each type brain tumor is beyond the scope of this discussion. We will,
however, provide an overview of the characteristic imaging features of common primary brain tumors in the brain
parenchyma, metastatic tumors, and those tumors outside the brain parenchyma.
aa. Intra-axial Tumors
Intra-axial tumors can be divided into two subgroups, primary brain
tumors and metastatic tumors. The primary tumors include gliomas
(astrocytoma, anaplastic astrocytoma, glioblastoma multiforme,
oligodendroglioma, and ependymoma), neuronal origin tumors, pineal
region tumors, and CNS lymphomas. Metastatic disease may affect not
only the brain parenchyma, but also the leptomeninges and calvarium.

Primary tumors. Gliomas represent

approximately 40% of all intracranial tumors (Russell). Astrocytic
tumors are the most common of the glial tumors. Typically, they
are infiltrative or diffuse but some specific subtypes are
circumscribed. Infiltrative astrocytic tumors include astrocytomas,
anaplastic astrocytomas, glioblastoma multiforme,
oligodendroglioma, and ependymoma. Circumscribed tumors of
astrocytic origin include pilocytic astrocytomas and
subependymal giant cell astrocytoma. On non-contrast CT,
gliomas may be evident only as white matter edema or an illdefined isodense white matter lesion. They are usually found
supratentorially in adults but more frequently infratentorially in
children. With the addition of contrast, low-grade astrocytomas
enhance poorly. Anaplastic astrocytomas and glioblastomas
show stronger enhancement patterns and may have areas of
heterogeneity. These high-grade gliomas often show spread of
edema or enhancement along white matter tracts such as the
corpus callosum that indicate infiltration of tumor into normal
brain. They may also show central areas of hemorrhage or
necrosis. On MRI, low-grade astrocytomas are iso- or
hypointense on T1 weighted images and homogeneously
hyperintense on T2. They may show minimal to no enhancement
with gadolinium. Anaplastic astrocytomas and glioblastoma
multiforme are iso- to hypointense on T1 and heterogeneous on
T2, with strong heterogeneous enhancement (Osborn). The T2
signals are more sensitive for edema, which often correlates with
the degree of tumor infiltration.
Pilocytic astrocytomas are tumors of glial origin that are more
common in children and young adults. These are wellcircumscribed tumors located near the third or fourth ventricles
and arise from the cerebellum and optic chiasm/hypothalamus.
On CT and MRI, they frequently appear cystic with an
enhancing, solid mural nodule (Osborn).
Oligodendrogliomas are relatively slow growing tumors arising
from oligodendrocytes. They are usually found in the cerebral
hemispheres, especially the frontal lobes. On imaging studies
they appear as a heterogeneous, enhancing mass in the

12

cerebral hemispheres often with calcification (Osborn).

Ependymomas are derived from ependymal cells, which line the
ventricular system. Most of these tumors are found
infratentorially, arising in the fourth ventricle. The supratentorial
ependymomas are usually found outside the ventricular system
(Palma). Supratentorial ependymomas may resemble
astrocytomas on imaging. Fourth ventricular ependymomas are
isodense with some areas of hyperdensity representing
calcification on noncontrast CT and mildly heterogeneously
enhancing on MRI. They appear to "fill" the ventricles and may
extend up or down the fourth ventricle.
Metastases. Metastases represent one
quarter to one third of all adult brain tumors (Davis). Common
metastases to brain include lung, breast, melanoma, renal cell
carcinoma, GI, and GU tumors. Between 60 and 85% of all
metastases are multiple (Bindal). On non-contrast CT,
metastases are iso- or hyperdense lesions usually found at the
gray-white junction, but can occur anywhere in the brain. With
contrast administration, metastases will enhance either
homogeneously or peripherally. On MRI, most metastases are
hypointense on T1, hyperintense on T2 and enhance with
gadolinium in the same pattern as on CT (Osborn). Cerebral
metastasis should be considered first in the differential diagnosis
in any patient with multiple lesions since primary brain tumors
are usually solitary. Extensive vasogenic edema generally
surrounds the tumor. Although less common, cerebral abscess
may mimic metastases on imaging and should be ruled out
either clinically or by biopsy.
bb. Extra-axial tumors

The distinction between intra-axial and extra-axial tumors is important in

surgical planning and prognosis. Extra-axial tumors are generally benign
tumors, such as meningiomas, vestibular schwannomas, pituitary tumors,
dermoids and epidermoids. Malignant extra-axial tumors include
metastases, malignant meningioma, sarcoma, and chordoma.

Meningiomas. Meningiomas are the most

common primary, intracranial tumor of nonglial origin (Hardman,
Russell). They are derived from arachnoidal cap cells and can be
found on any surface carrying arachnoid tissue. The most
common locations are the sagittal sinus (parasagittal), cerebral
convexity, sphenoid ridge, olfactory groove, and posterior fossa
(Osborn).
Plain skull films may show hyperostosis adjacent to a
meningioma and dilated vascular channels leading to the tumor.
Angiography can demonstrate an extracranial blood supply as
well as parasitized pial vessels. There may be a persistent
vascular blush. CT will show a well-circumscribed, hyperdense
mass that abuts the dura. Some meningiomas show calcification
on CT and demonstrate peritumoral edema. They enhance
strongly with intravenous contrast. On MRI, meningiomas are
isointense to brain, enhance strongly and often have a dural tail
corresponding to migration of tumor cells (Osborn).

Vestibular Schwannoma. Vestibular

schwannoma or acoustic neuroma is a benign tumor arising from

13

vi.

vii.

the nerve sheath of the vestibular portion of cranial nerve VIII. As

such, they are seen only in the cerebellopontine angle, often with
extension into the internal acoustic canal (IAC). On CT they are
iso- or hypodense and enhance strongly. The bone windows may
show widening of the IAC. On MRI, vestibular schwannomas are
well circumscribed, enhancing masses that extend into the IAC
(Goldberg).

Pituitary Adenomas. Pituitary adenomas

represent approximately 10% of all primary intracranial tumors
(Russell). They are grouped into microadenomas (<= 10 mm)
and macroadenomas (> 10 mm). Most adenomas are slow
growing and 50% are endocrinologically active, with the most
common type being the prolactinoma (Russell). Microadenomas
appear hypodense on contrast enhanced CT and MRI because
the normal pituitary enhances more strongly. Macroadenomas
appear as isodense intra- and suprasellar masses that enhance
strongly on CT and MRI. Cysts, hemorrhage, or necrosis may
also be seen in the macroadenoma.
Hydrocephalus
Hydrocephalus is a condition in which the ventricles become enlarged and intracranial pressure becomes elevated.
It is commonly divided into two types: communicating, which is due to the inability of the arachnoid granulations to
adequately absorb CSF; non-communicating, which is caused by an obstruction of CSF flow within the ventricles.
Communicating hydrocephalus may occur after subarachnoid hemorrhage if the subarachnoid blood and its byproducts impair the function of the arachnoid villi. On noncontrast CT and MRI, these patients have symmetric
enlargement of all ventricles, which is particularly noticeable in the third ventricle and temporal horns of the lateral
ventricles. Non-communicating hydrocephalus may occur with intraventricular tumors or cysts. In these cases there
is enlargement of the ventricles proximal to the occlusion and normal sized ventricles distal.
Cerebral Abscess
Cerebral abscesses are foci of parenchymal bacterial infections that result from contiguous spread, direct
inoculation, or hematogenous spread. Hematogenous spread from an extracranial site such as the lung often
results in multiple lesions, whereas local spread from mastoid air cells or paranasal sinuses usually creates a
solitary lesion (Bellar).

f.

The appearance on CT or MRI depends on the age of the abscess. In the early cerebritis stage of cerebral abscess
(3 to 5 days), CT or MRI may show a small, ill-defined area of mild enhancement at the gray-white interface. From
5 days to 2 weeks, the late cerebritis stage develops, and CT and MRI show a thin ring of enhancement around a
necrotic center. During the early and late capsule stages (weeks to months), a thicker, more defined ring of
enhancement forms with a surrounding area of edema (Zimmerman).
Imaging in Spinal Disease
i.
Spinal Trauma
Prompt recognition of spinal injuries and neurologic deficits are essential in the successful treatment of the multiply
injured patient (Bohlman). As described previously, plain x-ray is a fast and easy first test to evaluate spinal injuries.
Most fractures and subluxations can be seen on plain x-ray, but patients with point tenderness or neurological
deficit should undergo further evaluation. CT of the spine with sagittal reconstruction is very useful for identifying
the anatomical detail of the fractures seen on x-ray or to find fractures not seen on plain x-ray. MRI is useful in
identifying spinal cord compression or contusion and disc/ligamentous injury.
The four types of spinal injury based on direction of forces applied to the vertebral column are: 1) hyperflexion, 2)
hyperextension, 3) axial loading, 4) rotational injury (Brant-Zwadzski). Hyperflexion injuries usually result in anterior
wedging and compression fractures of the vertebral bodies. If sufficient flexion forces are applied to the spine,
these fractures may be associated with posterior ligamentous injury or facet subluxation ("locked facets").
Hyperextension injuries are common in the cervical spine, and often result in fractures of the posterior elements
such as the lamina, lateral masses, and facets. Frequently there is rupture of the anterior longitudinal ligament and
disruption of the disc space. In patients with congenitally narrow spinal canals or degenerative disc disease,
hyperextension can cause central cord syndrome.
Axial loading injuries are common in the cervical and thoracolumbar segments of the spine. A direct vertical force is

14

applied to the spine as in diving or jumping injuries resulting in compression or "burst" fractures of the vertebral
bodies.

ii.

Rotational injuries are usually associated with other forces such as lateral bending, flexion, or extension. These
forces can result in unilateral facet dislocations and in the cervical spine.
Degenerative Spinal Disease
Degenerative spinal disease is the most commonly seen disease in most neurosurgical practices. Advances in CT
and MRI have been very important in the diagnosis of spinal stenosis and disc herniation.
aa. Spondylosis and spinal stenosis.
As the intervertebral discs age, they become desiccated, the annular
fibers become weak, and the discs bulge or herniate. When patients
develop spondylosis, they form osteophytes along the discovertebral
junction that can compromise the spinal canal (Resnick). There are two
forms of spinal stenosis, congenital or acquired. The acquired form is
usually the result of spinal degeneration caused by bulging discs,
osteophytes, and ligamentum hypertrophy, which are best visualized on
CT bone windows. CT myelography (CT performed after intrathecal
injection of contrast) and MRI will show compression of the thecal sac.
Patients with severe prolonged stenosis may show signal abnormality
with the spinal cord.
bb. Disc bulge and herniation.
Bulging of the intervertebral disc is a common phenomenon in people
over age 20 and may be asymptomatic (Boden). As the annulus fibrosis
ages, it becomes weaker, thus allowing the nucleus pulposus to bulge
beyond the vertebral body margins. While CT or CT myelography can
accurately diagnose herniated discs, MRI is the preferred test because of
its ability to define the relationship of disc material to CSF, bone, and soft
tissue in both axial and sagittal planes. MRI is also less invasive than CT
myelography.

iii.

Spinal Tumors
Spinal tumors can be divided into three groups, 1) extradural, 2) intradural extramedullary, and 3) intramedullary
masses.
aa. Extradural spinal masses.
The most common extradural masses are metastases to the bone. Other
extradural masses include benign and malignant tumors of bone and
multiple myeloma. Metastases often present with pain, pathologic
fractures, and neurologic deficit consistent with location. On plain x-ray,
metastases may appear as multifocal, lytic lesions or vertebral fractures
(Olcott). CT is good for delineating the bony anatomy of osteolytic areas,
and, with the injection of intrathecal contrast, can show neurological
element compression. Contrast MRI allows better examination of the
spinal cord and soft tissues surrounding the spine. Metastatic lesions are
usually multiple and strongly enhancing.
bb. Intradural extramedullary spinal masses.
Most intradural, extramedullary spinal masses are either nerve sheath
tumors or meningiomas. Schwannomas and neurofibromas both originate
from the Schwann cell but differ grossly and histologically. Schwannomas
are well-circumscribed round or dumbbell-shape tumors that may be solid
or cystic (Burger). Neurofibromas are poorly circumscribed masses, which
often have nerve fibers coursing through them and are more frequently
solid. The imaging characteristics of these two tumors are similar. On CT,

15

there is often widening of the neural foramen. On T1 weighted MRI, the

lesions are iso- or hyperintense and enhance strongly with contrast. On
T2 weighted images, they are usually hyperintense. They will displace but
not invade the spinal cord or nerve roots within the thecal sac.
The histology of spinal meningiomas is identical to intracranial
meningiomas. On MRI, they are isointense on T1 and T2 weighted
images and enhance well. A dural tail may be observed.
cc. Intramedullary masses.
Intramedullary masses are found within the spinal cord parenchyma. Most
are gliomas, either ependymoma or astrocytoma. Ependymomas arise
from ependymal cells in the central canal. They are found in all segments
of the spinal cord and may have a better cleavage plane between tumor
and spinal cord than the astrocytoma. A subtype that is frequently found in
the conus medullaris, the myxopapillary ependymoma, is histologically
benign and has a good prognosis. Any ependymoma may undergo cystic
degeneration and hemorrhage (McCormick). Their appearance is
isointense to spinal cord on T1 weighted images, hyperintense on T2, and
enhance homogeneously (Osborn).
Astrocytomas of the spinal cord usually involve multiple segments and
diffusely widen the cord. They occur more frequently in the cervical cord.
CT may show thinning of the pedicles or widening of the interpedicular
distance. On MRI, they are iso- to hypointense on T1, hyperintense on T2,
and enhance strongly.
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Bone Joint Surg 72:403-408, 1990.
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A.3. Intracranial hypertension

a.

Clinical Manifestation of Acute and Chronic Intracranial Hypertension

i.

Cerebrospinal Fluid (CSF) Physiology

CSF is produced by choroid plexus in the lateral, third, and fourth ventricle through an ultrafiltration process.
Normally CSF is produced at the rate of 0.3cc/minute (or 400-500 cc a day). CSF circulates through the ventricular
system, then down to the spinal subarachnoid space, before it returns to the intracranial cavity. CSF is generally
absorbed by the arachnoid granulation over the cerebral convexity and returned to the vascular system. Some
minor absorption occurs near the cranial nerve root sheaths. Under extremely high pressure, CSF may be resorbed
over the ventricular ependyma in proportion to the pressure gradient. The total volume of CSF in an adult is about
150cc, meaning that the CSF volume is turned over more than 3 times a day. Only about 40cc of the total body
CSF volume is found in the ventricular system.

ii.

Definition: Normal intracranial pressure (ICP) is generally considered to be less than 20mm Hg. The Monro-Kellie
doctrine states that the total intracranial volume, consisting of brain, blood, cerebrospinal fluid (CSF), and other
pathological entity, remains fixed. The cerebral perfusion pressure (CPP) is defined as Mean Arterial Pressure
(MAP) minus the Intracranial pressure (ICP). [CPP=MAP-ICP] In management of increased intracranial pressure,
an effort is made to maintain the ICP below 20 mmHg and the CPP above 70 mmHg.
Etiology: Elevated intracranial pressure is the final common pathway of a variety of intracranial pathology.
The most common cause of elevated ICP is traumatic brain injury. Trauma can lead to hematoma
(epidural, subdural, and intraparenchymal) formation with resultant mass effect on the involved
hemisphere or lobe. Other parenchymal lesions such as cerebral contusion can also exert significant
pressure on the surrounding brain tissue. Edema and hyperemia (probably due to loss of cerebral
autoregulation) can cause further increase ICP, leading to secondary injury after the initial insult.
Both primary and metastatic neoplasm of the brain can cause breakdown of blood brain barrier, edema,
mass effect, and increased ICP. Generally, benign neoplasms do not cause ICP elevation unless their size
becomes substantial, or if they block CSF flow. Malignant neoplasms, because of the breakdown of blood
brain barrier, readily cause edema, mass effect, and ICP elevation. Obstruction of the ventricular system
by a mass lesion can lead to obstructive hydrocephalus and intracranial hypertension.
Hydrocephalus can occur in two forms, communicating or obstructive. Communicating hydrocephalus
generally occurs as a result of prior infection, hemorrhage, or arachnoid irritation. Normal pressure
hydrocephalus (NPH) in the elderly population may occur without the above predisposing factors. Rarely,
a cerebrospinal fluid (CSF) producing neoplasm such a choroid plexus papilloma can cause CSF
overproduction and result in communicating hydrocephalus. Obstructive hydrocephalus is the result of
CSF obstruction at strategic points of the CSF pathway. Common causes include intraventricular or

17

b.

periventricular tumors, brain herniation causing trapping of the lateral ventricle, intraventricular
hemorrhage, or aqueductal stenosis. A combination of these two types of hydrocephalus can occur.
Venous sinus thrombosis can occur as the result of systemic hypercoagulable states or trauma. Risk
factors include the use of oral contraceptive and certain connective tissue disorders, including lupus
erythematosis. CSF absorption depends on flow across the arachnoid granulations. Because of the
reduced venous drainage, increased intracranial blood volume leads to decreased CSF resorption,
resulting in intracranial hypertension.
Idiopathic intracranial hypertension is a disease most commonly found in adult female patients. Obesity is
a predisposing factor. Headache and progressive loss of visual acuity are the primary symptoms. Chronic
elevation of ICP causes papilloedema, which could be detected on fundoscopic examination.
Systemic hypertension can cause breakdown of the blood brain barrier and causes hypertensive
encephalopathy. This can increase ICP because of higher MAP and cerebral blood volume.
Clinical Presentation of Intracranial Hypertension

c.

Acute intracranial hypertension most frequently presents with a constellation of symptoms including headache, altered
mental status (level of consciousness), nausea/vomiting, and occasionally sudden death. In addition to the above
symptoms, chronic intracranial hypertension can present with cranial nerve palsies (third or sixth cranial nerve), ataxia,
memory disturbance, personality changes, or urinary incontinence. Both acute and chronic intracranial hypertension can
cause seizures.
Emergency Management of Intracranial Hypertension

Head position: Simple head elevation can promote venous return from the head and reduce ICP. It should be noted
that head elevated does lower the mean arterial pressure supplying the brain, possibly negating the beneficial
effects of increased venous drainage.
Hyperventilation: Though very effective in reducing ICP through its cerebral vasoconstrictive effect, hyperventilation
causes reduced cerebral blood flow (CBF). This can lead to secondary hypoxic injury. Only mild to moderate
degree of hyperventilation is recommended. (pCO2 > 30mmHg) The patient need to be mechanically ventilated to
have desired controlled ventilatory effects. The effect of hyperventilation is generally transient. (48-72 hours)
Diuretics/Hyperosmolar agents: Mannitol can be used to draw water out of the brain tissue by osmotic forces. This
reduces brain tissue volume and ICP. Other agents that can be used include furosemide and urea. These agents
should generally be "weaned off" to prevent rebound cerebral edema.
Sedation and paralytic agents. Agitation and muscle tremors/spasms can artificially elevate ICP. Benzodiazepines,
narcotics, and if necessary, chemical paralytic agents should be employed.
CSF drainage with an external ventricular drain, or much more rarely with a CSF shunt, can lower the ICP.
Barbiturate coma can be induced with pentobarbital to reduce cerebral blood flow (CBF) and cerebral metabolic
requirement of O2 (CMRO2). Close arterial pressure monitoring is mandatory because of its cardio-depressive
effect. Most clinicians advocate continuous electroencephalographic (EEG) monitoring to assess the end point of
burst suppression of EEG.
Avoid hypotension in order to maintain CPP.
Evacuate the causative intracranial mass lesion, including hematoma or neoplasm. A depressed skull fracture can
also cause elevated ICP. The underlying injury may not be reversible.

.1. Diagnosis and Management of Head Trauma

a.

Understand and Assign the Glasgow Coma Scale.

The Glasgow Coma Scale was developed in order to standardize the neurologic assessment of patients with head injury. It
was specifically designed to be easily performed based upon clinical data, and to have a low rate of interobserver variability.
In addition, the Glasgow Coma Scale score is correlated with outcome in that patients with a higher Glasgow Coma Scale
score have a statistically better outcome than patients with a lower Glasgow Coma Scale score.
Glasgow Coma Scale:
Points

Best Eye Opening

Best Verbal Response

Best Motor Response

Obeys

Oriented

Localizes pain

18

Spontaneous

Confused

Withdraws to pain

To speech

Inappropriate

Flexor (decorticate)

To pain

Incomprehensible

Extensor (decerebrate)

None

None

None

b.

c.

The Glasgow Coma Scale score is determined by adding the values for eye opening, verbal response, and motor response.
Possible values range from 3 to 15. Note that this scale rates the best response only. In patients who are intubated, in whom
assessment of best verbal response cannot be performed, notation of this is made in the Glasgow Coma Scale score by
adding a "t" to the end of the score. In patients who are intubated, the best possible score would therefore be 11t. Certain
numerical values of the Glasgow Coma Scale have particular clinical significance. Patients with a Glasgow Coma Scale of 7
or less are considered to be comatose. Patients with a Glasgow Coma Scale score of 8 or less are considered to have
suffered a severe head injury.
Recognize the Presentation of Brain Herniation Syndromes in the Setting of Trauma:
Distortion of the midline brain structures secondary to brain trauma may lead to specific combinations of signs and
symptoms which are collectively referred to as herniation syndromes. In general, these symptoms result from the distortion
of midline brain structures secondary to brain swelling, hydrocephalus, or intracranial mass lesions. While numerous
herniation symptoms have been described, the two most commonly seen syndromes in the setting of trauma are uncal
herniation and tonsillar herniation.
i.
Uncal herniation:
Most often results from a laterally placed mass displacing the brain stem contralaterally and pushing the uncus of
the temporal lobe medially over the tentorial edge.
Early Sign:
Ipsilateral pupillary dilation
Late Signs:

Complete ipsilateral third nerve palsy

Loss of consciousness
Contralateral hemiplegia (secondary to mass)
Ipsilateral hemiplegia (secondary to compression of contralateral cerebral peduncle against edge of
tentorium [Kernohans notch])
Flaccid paralysis
Tonsillar herniation:

ii.

Results from downward displacement of the cerebellar tonsils through the foramen magnum, causing compression
of the cervicomedullary junction. Frequently secondary to posterior fossa mass. May be precipitated by lumbar
puncture in the presence of such a mass.
Signs:
Head tilt/neck pain
Respiratory arrest
Loss of consciousness
Flaccid paralysis
Initiate Management of Elevated Intracranial Pressure in Head Trauma:

d.

Brain injury after acute head trauma can be divided into two categories. The first category is primary injury, which is suffered
at the time of impact. The second category is secondary injury, which may occur at any time from that point forward. One of
the most important causes of secondary brain injury in head trauma is felt to be elevated intracranial pressure (ICP).
In treatment of the patient with head trauma, the possibility of elevated intracranial pressure should always be considered.
Management of the traumatized patient begins with the primary survey and resuscitation.

19

Steps involved include:

Airway patency with cervical spine control: It is important to establish the presence of a patent airway. If such an
airway is not present, one should be established. This may include the use of chin lift or jaw thrust, clearance of
foreign bodies, endotracheal intubation, or creation of a surgical airway. It is important to consider that the cervical
spine may be injured and that it should be maintained in a neutral position during any of the above maneuvers.
Breathing control. The chest should be examined and the rate and depth respirations determined. Inadequacy may
indicate the need for mechanical ventilation. High concentrations of oxygen should be administered. Pneumothorax
should be treated.
Circulatory and hemorrhage control. The quality, rate, and regularity of the pulse should be determined. Sights of
major hemorrhage should be identified and treated.
Disability. A brief neurologic examination should be performed. The Glasgow Coma Scale should be determined.
Pupils should be assessed for size, equality, and reaction.

During the secondary survey, a more complete neurologic examination should be performed including evaluation of the
patients strength, sensation, reflexes, and remaining cranial nerves.
Following the secondary survey, appropriate imaging studies should be obtained. In a patient with obvious craniofacial
trauma, mechanism of injury sufficient to produce brain injury, or a disturbed level of consciousness, a CT scan of the head
without contrast should be performed. The presence of fractures, foreign bodies, space occupying lesions, or hemorrhage
should be noted, as well as the ventricular size.
Intracranial pressure monitoring should be considered in the following situations:
Patients with an abnormal admission CT scan and Glasgow Coma Scale score of 3 to 8 after cardiopulmonary resuscitation.
Or
Patients with a normal head CT with a Glasgow Coma Scale score of 3 to 8 and the presence of two or more of the following
features: Age over 40 years, unilateral or bilateral motor posturing, systolic blood pressure less than 90 mmHg.
The current preferred modality for monitoring of intracranial pressure is the placement of a ventricular catheter
(ventriculostomy). Use of fiberoptic or strain gauge pressure monitors can be considered.
Elevated intracranial pressure has been shown to have definite prognostic implications in a patient with severe brain injury.
In addition, it is generally held that treatment of elevated intracranial pressure may improve outcome in the patient with
severe brain injury. The currently recommended threshold for treatment of elevated intracranial pressure is 20 to 25 mmHg.
Interpretation and treatment of intracranial pressure based on this threshold value should be corroborated by frequent
clinical examination and assessment of the cerebral perfusion pressure. Current recommendations suggest that CPP should
be a minimum of 60 to 70 mm of mercury. It is important to consider that, while MAP is an important determining factor in the
CPP, it has also been shown that low MAP is an independent predictor of poor outcome.
After elevated intracranial pressure has been identified, treatment should be initiated. In general, treatment should proceed
in a stepwise fashion, beginning with the least onerous treatment modalities. Escalation of treatment should proceed only
after failure of less onerous modalities. The suggested hierarchy of treatment includes the following therapeutic modalities:

Body positioning. The head should be elevated 30 degrees. The neck should be maintained in a neutral position.
Compression of the jugular veins should be avoided.

Maintenance of homeostasis. Euvolemia should be established. Arterial blood gases should be measured with the
goal of maintaining the PO2 in the 90 to 100-mm mercury range and the PCO2 in the 35 to 40-mm mercury range.
Mild sedation. This is most frequently carried out using a combination of benzodiazepines and/or narcotics.
External ventricular drainage. At this stage, placement of a ventriculostomy, if not done previously for ICP
measurement, should be considered. The reservoir is generally placed 5 to 10 cm above ear level or, alternatively,
placed at ear level and opened at regular intervals.
Use of osmotic diuretics. Mannitol is the most commonly used osmotic diuretic. It is most frequently given in a dose
of 0.25 to 1.0 grams/kg I.V. over 15 minutes. If the effect of treatment with Mannitol is transient, the dose may be

20

repeated, so long as the serum osmolality remains less than or equal to 320 mOsm/L and the patient remains
euvolemic.
Moderate hyperventilation. At this stage, moderate hyperventilation to a PCO2 of 30 to 35 mm of mercury can be
considered.
Second tier therapies including barbiturate therapy. At this stage, barbiturate therapy can be considered.
Pentobarbital is the most commonly used barbiturate for the treatment of refractory intracranial hypertension.
Recommended loading dose is 10 mg/kg over 30 minutes followed by a maintenance dose of 1 mg/kg per hour as
a continuous infusion. The dose is then titrated to achieve serum Pentobarbital levels in the range of 3 to 4 mg/dl or
an electroencephalographic pattern of burst suppression. Potential complications of this modality of treatment are
numerous, with hypotension being the primarily dose-limiting toxicity. Barbiturate treatment of refractory intracranial
hypertension has been shown to decrease mortality but has not been shown to improve neurologic outcome.

The above treatment algorithm assumes that all significant cranial space-occupying lesions have been appropriately
surgically treated. During implementation of the above algorithm, it is important to consider the possibility that a new
intracranial lesion has developed. Because of this, a repeat CT scan of the head should be considered prior to escalation of
therapy.
Note that the above treatment algorithm does not include the use of corticosteroids. The use of glucocorticoids is not
recommended for improving outcome or reducing intracranial pressure in patients with severe head injury. The routine use
of prophylactic anticonvulsant medication is not recommended for the prevention of posttraumatic seizures in the patient
without a premorbid seizure disorder. The use of anticonvulsants may be considered, in the first 7 days after injury, to
prevent early posttraumatic seizures in patients who are at high risk. Phenytoin and carbamazepine are the most commonly
used agents. Finally, nutritional support of brain injured patients should be instituted within 7 days of injury.

bb

Recognize and Initiate Management of Concussion Brain Contusion and Diffuse Axonal Injury.
i.

Concussion.
Cerebral concussion is a diffuse brain injury thought to be caused by acceleration-deceleration injury to the brain.
Cerebral concussion is a spectrum of injuries, ranging from mild to severe.
Mild concussion is defined as no loss of consciousness with transient neurologic disturbance.
Moderate concussion is defined as loss of consciousness with complete recovery occurring in less than 5
minutes.
Severe cerebral concussion is defined as unconsciousness lasting greater than 5 minutes.
Evaluation and treatment of patients with cerebral concussion remains controversial. Workup includes complete
history and physical examination, with neurological examination. Other tests include cervical spine x-rays and other
radiographs as indicated, blood alcohol level and urine drug screen, and CT scan of the head in all patients except
those who are completely asymptomatic and neurologically normal.
Treatment of patients with cerebral concussion who have a Glasgow Coma Scale score of 14 or 15 is usually
expectant. Most patients should undergo hospital admission with frequent neurological examinations. These
include all patients with an abnormal CT scan, history of loss of consciousness, decreased or decreasing level of
consciousness, severe headache, under the influence of alcohol or drugs, have physical examination evidence of
CSF rhinorrhea or otorrhea, significant associated injuries, no reliable companion at home, unable to return
promptly, or are amnestic for the injury. Only those patients who do not manifest any of the prior criteria should be
considered for discharge from the hospital. If hospital discharge is considered, any of the previously listed signs or
symptoms should prompt a return to the hospital. A written head injury "warning sheet" should be issued.
Neurosurgical follow-up should be scheduled.
In addition to frequent neurologic examinations, patients admitted after cerebral concussion may be treated with
Tylenol or very mild doses of narcotic pain medication for headache. Nausea and vomiting, which are frequently
present after mild or severe concussion, should be treated with non-phenothiazine antiemetic medication.
Discharge may be considered when the patient is neurologically normal, nausea and vomiting has ceased, and
headache has ceased or is adequately controlled.
All patients who have suffered a cerebral concussion should be counseled regarding the possible occurrence of
"post concussive syndrome". Prominent symptoms include headache, mild impairment of memory, dysequilibrium,
and alteration of mood. These symptoms usually regress spontaneously but may persist for weeks to months.

21

ii.

Diffuse axonal injury:

Diffuse axonal injury is the most severe form of diffuse brain injury. It is felt to be the most common cause of
prolonged posttraumatic coma that is not due to mass lesion or ischemia. Diffuse axonal injury is characterized by
focal hemorrhagic lesions involving the corpus callosum, rostral mid brain, superior cerebellar peduncles combined
with microscopic evidence of widespread axonal damage. Patients with diffuse axonal injury frequently manifest
decorticate or decerebrate posture and autonomic dysfunction in addition to their prolonged coma. Elevated
intracranial pressure is frequently absent. Care is primarily supportive. In patients with prolonged coma, the
prognosis is generally poor, with a 50% mortality and with an approximately 25% incidence of favorable outcome.

iii.

Cerebral Contusion.
A cerebral contusion is a focal brain injury caused primarily by impact of the brain surface and the bony ridges of
the calvarium. Cerebral contusions are frequently found in the region of the frontal poles, anterior skull base,
adjacent the sphenoid ridge, and at the temporal poles. Other locations include the cerebellar hemispheres and the
occipital poles. A characteristic pattern of cerebral contusion called the "coup and contrecoup" injury is frequently
seen. The coup contusion occurs at the sight of impact and the contrecoup contusion occurs in the brain at the
point diametrically opposite the point of impact.
Treatment of cerebral contusion is guided by the neurologic examination. The patient should be admitted to the
hospital for observation and frequent neurologic examinations. Intracranial pressure monitoring and treatment
should be instituted for the comatose patient. While surgical treatment, consisting of debridement of the contused
brain tissue, is not routinely recommended, it can be considered in patients with refractory intracranial
hypertension.

e.

Recognize and Initiate Management of Acute Subdural and Epidural Hematoma, Including Surgical Indications.
iv.

Subdural hematoma.
In the acute traumatic subdural hematoma, blood collects between the dura mater and surface of the brain. Most
commonly, the bleeding results from tearing of bridging veins located over the convexity of the brain surface.
Bleeding originating from a small cortical artery represents the second most common source. Associated
intracranial lesions, particularly cerebral contusions, are found in at least 50% of patients with acute traumatic
subdural hematoma.
Patients presenting with acute traumatic subdural hematoma may range from normal to deeply comatose. Unlike
the epidural hematoma, to be described later, the most common presentation of the patient with an acute traumatic
subdural hematoma is that of a patient rendered unconscious at the time of injury without regaining consciousness
prior to presentation.
Neurologic findings may be secondary to mass effect, elevated intracranial pressure, and/or associated brain
injuries. Elevated intracranial pressure is a common finding and evaluation and treatment of elevated intracranial
pressure, as outlined above, should be instituted immediately. The acute traumatic subdural hematoma is most
commonly treated surgically. Observation should only be considered in those patients with small (less than 10 mm
thick) subdural hematomas who are neurologically intact. Because the acute traumatic subdural hematoma
consists of solid blood clot, burr hole drainage is inadequate for relief of mass effect. A craniotomy should be
performed and all easily assessable blood clots should be removed. Postoperatively, patients frequently manifest
intracranial hypertension, and this should be treated as outlined above.

v.

Outcome after treatment of acute traumatic subdural hematoma is related to a number of factors, particularly
preoperative neurologic status. Mortality ranges from greater than 75% in those patients who present with a GCS
of 3 to 5 to minimal in patients who present with GCS of 12 to 15. Time from injury to surgical decompression,
elevated intracranial pressure, and associated brain lesions also have a detrimental effect on outcome.
Epidural hematoma.
In the acute epidural hematoma, blood collects between the inner surface of the calvarium and the dura mater.
Most commonly, the acute epidural hematoma results from fracture of the skull, stripping the dura mater from the
inner table of the skull, and causing laceration of meningeal vessels or dural sinuses. Bleeding from the middle
meningeal artery is responsible for many supratentorial epidural hematomas. In contrast to the subdural
hematoma, the patient harboring an acute epidural hematoma may have an initial loss of consciousness, followed
by a brief "lucid" interval, followed by progressive neurologic decline. This presentation is seen in approximately 1/3

22

of patients having an acute epidural hematoma.

Treatment of acute epidural hematoma is generally surgical. Patients with small epidural hematomas not traversing
a meningeal artery vein or major sinus, who present greater than 6 hours after injury may be considered for a
nonoperative therapy. However, admission with frequent neurologic examination and a low threshold for repeat CT
scanning is mandatory. For all other patients, operative treatment is recommended. As with the acute subdural
hematoma, the acute epidural hematoma is comprised of a solid blood clot. Therefore, burr hole drainage is
inadequate for removing the intracranial mass. A craniotomy should be performed and the entire blood clot
evacuated. After evacuation, the dura mater should be opened to inspect the subdural space. Frequently, a
subdural hematoma may be encountered which was not obvious on the preoperative CT scan. Postoperatively, the
patient is monitored for signs of elevated intracranial pressure. Elevated intracranial pressure, if detected, is treated
as outlined above.
As in the acute traumatic subdural hematoma, outcome after treatment of acute epidural hematoma is related to a
variety of factors, the most important being preoperative neurologic status. Mortality ranges from less than 15% in
those patients who present with GCS of less than 8 to very low in patients who present with GCS 8 to 15.
f.

Recognize and Initiate Management of Penetrating Trauma Including Gunshot Wounds.

Penetrating injuries include all injuries where the scalp and skull are violated by foreign objects including knives, sticks,
pencils, arrows, and bullets. Low velocity injuries, such as those produced by a knife blade, produce brain injury along the
tract of the knife. High velocity injuries, such as those produced by a bullet, produce both local injury along the tract of the
bullet, as well as remote injury in the cavity produced in the wake of the bullet.
The size of the cavity produced by a bullet is related to its kinetic energy, as well as its shape. The kinetic energy is
proportional to the mass of the bullet as well as the square of its velocity. The shape of the bullet not only affects the velocity,
but also its ability to transfer its kinetic energy to the brain tissue. Bullets that tumble or deform on impact transfer a greater
proportion of their kinetic energy to brain tissue and thus produce a more severe injury.
After resuscitation and primary survey, an evaluation of the patient with a penetrating injury includes a thorough physical
examination and neurological examination. The location of entry and exit wounds should be well documented. Extent of
tissue loss should likewise be documented. All patients who have had penetrating brain injury should undergo a CT scan of
the head. Coronal CT scan of the head should be considered in patients with involvement of the anterior skull base.
Cerebral angiography should be considered in patients where vascular injury is suspected. Cerebral angiography should be
given particular consideration where the tract of the injury passes close to a major vascular structure or in patients with a
significant subarachnoid hemorrhage or delayed hematoma.
As in patients with severe non-penetrating brain injury, elevated intracranial pressure should be suspected. The algorithm for
this is described above. In addition, in patients with penetrating brain injury, intracranial pressure monitoring is indicated
when it is not possible to assess the neurological examination accurately or when the need to evacuate a mass lesion is
unclear.
As with the open depressed skull fracture, most penetrating brain injuries require surgical treatment.

Entrance and exit wounds. Treatment of small bullet entrance wounds where the scalp is not devitalized and the
patient has no significant intracranial pathology may be treated with local wound care and closure. More extensive
wounds with nonviable scalp, bone, or dura may require operative debridement before primary closure and dural
grafting. Watertight dural closure is recommended. This includes patients with significant fragmentation of the skull.
Intraparenchymal lesions. Intraparenchymal lesions resulting in significant mass effect may require debridement of
necrotic brain tissue and removal of easily accessible bone fragments. This includes patients with significant
intracranial hematomas.
Injuries resulting in communication between an open-air sinus and the intracranial space should be closed in a
watertight fashion.

Routine removal of bone fragments or missile fragments remote from the entry site is not recommended. Vascular injuries
detected on arteriography may require surgical or endovascular treatment as indicated. Cerebrospinal fluid leaks which do
not close spontaneously or which do not resolve after the primary surgery should be treated with temporary CSF diversion.
Those which do not respond to temporary CSF diversion may require surgical treatment. Patients with penetrating brain
injury should be administered broad-spectrum antibiotics prophylactically. The duration of this treatment is somewhat
controversial. Finally, the use of anti-seizure prophylaxis in the first week of penetrating brain injury is recommended to

23

prevent early posttraumatic seizures. As with severe non-penetrating brain injury, long-term prophylactic treatment with
anticonvulsants to prevent late posttraumatic seizures is not recommended.

bb

Recognize and Understand the Principles of Management of Open, Closed, and Basilar Skull Fractures Including
Cerebrospinal Fluid Leak in Chronic Subdural Hematoma in Children and Adults.
i.

Open skull fractures.

ii.

The treatment of skull fractures with overlying laceration is primarily based on whether the fracture is depressed or
non-depressed. Open non-depressed fractures may be treated with inspection, cleansing, and scalp suturing with
an acceptably low rate of infection. Open depressed skull fractures present a significant risk of infection. They are
generally treated with operative irrigation, debridement, and removal of the depressed fragments. Frequently, this
will necessitate further surgical procedures to correct the resulting cosmetic deformity.
Closed fractures.

iii.

Closed skull fractures require no specific treatment. As noted above, the patient harboring a skull fracture is at
increased risk of formation of epidural hematoma. Patients harboring this injury should be treated as for those
patients with severe concussion, unless symptoms of elevated intracranial pressure develop. Traditionally, closed
depressed skull fractures were treated surgically to elevate the depressed fragments. It has since been shown that
this practice does not result in a decreased incidence of posttraumatic epilepsy and has therefore been
abandoned. Treatment of patients with depressed, closed fractures, except where there is a significant cosmetic
deformity, should be treated as outlined above for closed, non-depressed fractures.
Basilar skull fractures.

iv.

A basilar skull fracture involves the cranial base. The most common sites are the floor of the anterior cranial fossa
and the temporal bone. Evidence that a patient may have suffered a basilar skull fracture includes "raccoon eyes"
and "battle sign" (bruising posterior to the ear, possibly including the mastoid process). Most basilar skull fractures
do not require treatment in the absence of associated brain injury. They are a sign that a significant blow has been
delivered to the cranium. Most patients with significant basilar skull fracture should be observed with frequent
neurological examinations for 24 hours after the injury. Occasionally, complications of basilar skull fracture may
result. These include posttraumatic cerebrospinal fluid leakage, optic nerve injury, and facial nerve injury. The
incidence of posttraumatic cerebrospinal fluid leak after a closed head injury is approximately 2%. The most
common sites of cerebrospinal fluid egress are the nose (CSF rhinorrhea) and ear (CSF otorrhea). Most traumatic
CSF leaks resolve with nonoperative treatment including head elevation. It is important to insure that the CSF
leakage does resolve, as persistent CSF rhinorrhea carries with it an approximately 25% risk of meningitis, as well
as a risk of tension pneumocephalus.
Posttraumatic CSF leak.

v.

Treatment of the posttraumatic CSF leak which does not resolve with head elevation includes repeated high
volume lumbar punctures, as well as continuous catheter CSF drainage. There is a finite risk of inducing tension
pneumocephalus using those modalities. For leaks which persist despite these measures, surgical treatment
should be considered.
Chronic subdural hematoma.
As described above, the chronic subdural hematoma is a collection of blood and blood products between the inner
surface of the dura mater and the outer surface of the brain. Unlike the acute traumatic subdural hematoma, onset
of symptoms and detection of the subdural hematoma occurs much later in the course. The hallmark of the chronic
subdural hematoma is blood products, visualized on the CT scan, which are isodense or hypointense with respect
to brain tissue. It is theorized that, like the acute traumatic subdural hematoma, the source of the blood products is
lacerated bridging veins resulting from acceleration-deceleration forces applied to the skull. While this is usually the
result of trauma, trauma may be mild, remote, and not remembered by the patient or family. Many patients who
present with a chromic subdural hematoma have one or more risk factors. These risk factors include:
Advanced age and cerebral atrophy.
Male sex.
Coagulopathy.
Intracranial hypotension secondary to CSF shunting procedures
Chronic alcoholism.

Chronic subdural hematomas tend to enlarge slowly over time. The most common mechanism of enlargement

24

appears to be multiple episodes of re-bleeding, however, other mechanisms may also be important.
Symptoms at presentation include symptoms of increased intracranial pressure (headache, papilledema,
decreased level of consciousness), as well as those of hemispheric mass effect (hemiparesis, dysphasia, tremors,
and dystonia. Seizures occur occasionally.
Treatment of chronic subdural hematoma is generally surgical. While treatment with prolonged bedrest, head
elevation, and osmotic diuretics has produced acceptable results, the risks of prolonged immobilization in these
generally debilitated patients frequently outweigh the benefits.
There are multiple surgical options for the treatment of chronic subdural hematoma including:

Twist drill hole and drainage.

Multiple burr holes and drainage.
Craniotomy and drainage with stripping of membranes.

Hematomas that are not fully liquefied may require craniotomy. In addition, recurrence rate after evacuation of
chronic subdural hematoma is substantial.
Postoperatively, elevated intracranial pressure is unusual. Patients are generally maintained supine with minimal
elevation of the head for at least 24 hours to allow some re-expansion of the brain. Because of the substantial rate
of recurrence, neurosurgical follow-up is essential.
vi.

Chronic subdural hematoma in children.

Like the adult chronic subdural hematoma, blood and blood products, in the childhood chronic subdural hematoma,
accumulate between the inner surface of the dura and the brain surface. In children, it is particularly important, and
sometimes difficult, to distinguish between the chronic subdural hematoma and the subdural hygroma, in which
cerebrospinal fluid collects between the dura and arachnoid membrane. Furthermore, low-density fluid may collect
between the arachnoid and pia mater secondary to communicating hydrocephalus.
While trauma is the most common cause of chronic subdural hematoma in children, the possibility of nonaccidental trauma (child abuse) must always be considered, particularly in children less than 2 years of age.
Coagulopathy remains a major risk factor.
In children, presenting symptoms are usually those of elevated intracranial pressure including vomiting, lethargy,
irritability, or increase in the head size. Seizures are also seen.
The treatment of chronic subdural hematoma in children is different than that of adults. Unlike adult patients, very
young patients may have an open fontanel, facilitating percutaneous aspiration of the subdural hematoma. Failure
of this treatment modality generally requires placement of a subdural to peritoneal shunt.

References/Suggested Reading:
1.
2.
3.
4.
5.
6.

Bullock R, Chesnut RM et al. Guidelines for the management of severe head injury. Journal of Neurotrauma 13: 639-734,
1996
Bullock R, Chesnut RM et al. Guidelines for the management of severe traumatic brain injury. Journal of Neurotrauma 17:
451-627, 2000
Committee on Trauma of the American College of Surgeons: Advanced Trauma Life Support Student Manual. Chicago, IL,
American College of Surgeons, 1990
Eisenberg HM, Aldrich EF (eds). Management of Head Injury. Neurosurgery Clinics of North America 2: 251-501, 1991
El-Kadi H, Kaufman HH (eds). Chronic Subdural Hematoma. Neurosurgery Clinics of North America 11: 395-567, 2000
Florin RE. Management and prognosis of penetrating brain injury. Journal of Neurotrauma (in press)

B.2. Diagnosis and Management of Brain Tumor and Abscess

a.

Neuroepithelial tumors

25

i.

Astrocytoma
Astrocytomas, which arise from astrocytes, are the most common primary brain tumor. Astrocytomas are
histologically graded based on cellularity, anaplasia, mitotic figures, endothelial proliferation and necrosis. Welldifferentiated astrocytomas (WDA) have mild hypercellularity and minimal nuclear pleomorphism. They typically
occur in children and young adults who present with a seizure or headaches. On CT or MRI scan they usually
appear as a non-enhancing mass lesion. Anaplastic Astrocytomas (AA) have moderate cellularity and nuclear
pleomorphism with mitotic activity. Moderate endothelial proliferation can be present but no necrosis. These lesions
are typically found in mid-life as enhancing lesions with mass effect. They often present with seizures, headaches
and/or focal neurological findings. Glioblastoma multiforme (GBM) is characterized by hypercellularity, dramatic
nuclear pleomorphism, endothelial proliferation, mitotic figures and necrosis. These patients are usually older
adults who present with headaches, seizures or focal neurologic findings. On CT or MRI scan a GBM typically
appears as an irregular ring-enhancing lesion with significant mass effect and edema.
Survival correlates with grade: WDA 5-10 years, AA 2-3 years and GBM 1-1.5 year. Astrocytomas can initially
present as a low-grade tumor and subsequently convert to a higher grade. De novo AA and GBM tumors occur as
well.
Treatment is based on grade. Controversy exists for the optimal treatment of WDA. Most agree that a WDA in noneloquent brain with mass effect should be resected. A stereotactic brain biopsy for diagnosis is an option for
symptomatic lesions in areas of the brain where open surgery carries an increased risk of causing a deficit.
Radiation is controversial in stable tumors but is often used if growth is demonstrated. AA and GBM are typically
treated by surgical resection followed by radiation and BCNU chemotherapy. Surgery is indicated for diagnosis, to
relieve mass effect, and, possibly, to decrease the "tumor burden". AA or GBM in the motor strip, language areas or
other eloquent brain regions are often biopsied rather than resected because of the high risk of surgery in these
areas. It has been difficult to prove that the extent of tumor resection has an effect on patient survival. As a rule,
surgery is never curative. BCNU impregnated wafers implanted in the tumor bed after resection of a recurrent AA or
GBM have proven to be efficacious. Recurrence within a centimeter or two of the resection site is typical regardless
of the treatment given after surgical resection. Surgery for recurrent AA and GBM is controversial. Most surgeons
agree that reoperation is indicated for the relief of headaches or neurological deficits due to mass effect.
Pathologically, several types of astrocytomas exist, such as fibrillary, protoplasmic and gemistocytic astrocytomas.
Glioblastomas also have giant cell and gliosarcoma variants.
A separate group of astrocytoma is the juvenile pilocytic astrocytoma (JPA). This group is either not graded or
considered Grade 0. JPA are distinct because they behave in a more benign fashion and when completely resected
can be cured by surgery alone. The are typically discrete cystic lesions with an enhancing mural nodule.
Histologically, JPA are composed of loose and dense regions of stellate astrocytes. These have Rosenthal fibers
that indicate slow growth. JPA are typically found in children and young adults. They tend to occur in the cerebellar
hemisphere, optic nerve, hypothalamus and brainstem. Cerebellar JPA often present with signs of increased
intracranial pressure (headache, nausea, vomiting) due to hydrocephalus. JPA also can present with cerebellar
dysfunction such as gait ataxia or ipsilateral extremity dysmetria. Rarely, JPA can undergo malignant degeneration.
Subarachnoid seeding does occur rarely with JPA and probably carries a poorer prognosis. This has been seen
with hypothalamic tumors. Optic nerve JPA and WDA tumors are associated with neurofibromatosis type II.
Pleomorphic xanthoastrocytomas are astrocytic neoplasms found in young adults with a long history of seizures.
They are usually superficial in the cerebral cortex and may consist of a mural nodule associated with a cyst. They
are typically slow growing but malignant transformation does occur. Subependymal giant cell astrocytomas are
typically found at the foramen of Monro in patients with tuberous sclerosis.

ii.

Gliomatosis cerebri is a condition where there is diffuse infiltration of the entire brain with an astrocytic tumor.
Oligodendroglial tumors
The majority of oligodendrogliomas present in young adulthood with the onset of seizures. Radiographically,
calcifications are typical. The classic histologic appearance is of homogeneously appearing cells with a "fried egg"
appearance and "chicken wire" vessel pattern. Similar to WDA, patients can have long term survival. Malignant
transformation does occur. These tumors are called anaplastic oligodendrogliomas. In general, surgical resection is
recommended when possible for diagnosis, to relieve mass effect, and resect as much tumor as safely as possible.
Radiation therapy is controversial, but is probably beneficial. PCV (procarbazine, CNU, & vincristine) chemotherapy
has been shown to be beneficial in the treatment of oligodendrogliomas.

26

iii.

Ependymal tumors
Ependymomas typically arise from the lining of the ventricular system and usually occur in children and young
adults. The floor of the fourth ventricle is a common location. Ependymomas typically present with hydrocephalus
and increased ICP. Presenting symptoms include nausea, vomiting, headache, gait ataxia, diplopia and vertigo.
Ependymomas have a significant potential for CSF seeding and thus "drop metastasis". Complete surgical
resection has been shown to improve survival and should be attempted if there is minimal brainstem invasion.
Postoperative radiation and chemotherapy is usually administered. No clear consensus exists for grading
ependymomas but the term anaplastic ependymoma is sometimes used for more malignant appearing tumors.
Paradoxically, intramedullary spinal cord ependymomas, which are histologically identical, may be cured by surgery
alone.

iv.

Myxopapillary ependymomas are a variant of ependymomas. This tumor occurs in the conus or filum terminale of
the spinal cord. Complete resection is probably curative. Subependymomas occur in anterior lateral ventricles or
posterior fourth ventricle. They are benign slow growing tumors that are typically found incidentally at autopsy.
However subependymomas can cause hydrocephalus from obstruction of cerebrospinal fluid pathways.
Symptomatic or enlarging tumors should be removed. In elderly patients insertion of a VP shunt is a viable option if
obstruction of CSF pathways is present.
Mixed gliomas

v.

Mixed gliomas occur and appear histologically as a combination of neoplastic oligodendrocytes and astrocytes.
These tumors are referred to as oligo-astrocytomas or anaplastic oligo-astrocytomas. Often the name is shortened
to the dominant cell type only.
Choroid plexus tumors

vi.

Tumors of the choroid plexus are called choroid plexus papillomas (CPP). In children less than 2 years of age, they
usually are located in the lateral ventricle and present with hydrocephalus. In adults CPP usually occur in the fourth
ventricle, foramen of Luschka or cerebellopontine angle. The treatment is surgical resection, though elderly
patients with an asymptomatic cerebellopontine angle tumor may be followed with serial imaging studies.
Recurrence should be treated aggressively with reoperation, when possible, due to a favorable prognosis. One or
two percent of choroid plexus tumors are carcinomatous. Choroid plexus carcinoma carries a poor prognosis.
Neuronal and mixed Neuronal-glial tumors
Gangliocytoma is a tumor composed of large abnormal mature neurons. They are primarily supratentorial with the
most common location being the temporal lobe. The majority of patients are in their first two decades of life.
Surgical resection, when complete, is curative. Variable radiographic features occur, ranging from an enhancing
mural nodule to a ring enhancing mass with calcifications.
Dysplastic gangliocytoma of the cerebellum (Lhermite Duclos Disease) is a non-neoplastic mass of hypertrophic
granular cell neurons which expands the cerebellar folia. These tumors can cause mass effect and hydrocephalus
and typically occur in young adults. Resection (total or subtotal) and/or shunting are therapeutic options.
Dysembryoplastic neuroepithelial tumors (DNET) are a "hamartomatous", supratentorial, predominantly temporal
lobe lesion composed primarily of glial cells. They usually present with seizures. Radiographically these tumors
often lack edema and have a multinodular appearance. Inner table skull erosion or deformation may be present.

vii.

Gangliogliomas are tumors consisting of large mature neurons and a neoplastic glial component. This tumor affects
patients of all ages with the majority diagnosed in young adults who often have a long history of seizures. Surgical
resection even if subtotal can be curative. Surgery is recommended for diagnosis and, on occasion, for control of
seizures.
Pineal tumors
Pineal tumors are tumors arising from pineocytes. The well-differentiated pineocytoma occurs in mid-life as a
discrete contrast enhancing mass in the posterior third ventricle/pineal region. The poorly differentiated
pineoblastoma has a similar location and enhances with contrast but shows signs of local invasion and is prone to
CSF dissemination. The complex of pineoblastoma and bilateral retinoblastoma is called trilateral tumor. In general,
pineocytomas have a good prognosis while pineoblastomas with subarachnoid spread are aggressive and carry a
poor prognosis. Pineal tumors often present with hydrocephalus due to obstruction of the aqueduct of Sylvius.
Compression of the dorsal midbrain by a pineal tumor can result in Parinauds syndrome of pupillary mydriasis,
paralysis of upgaze, and convergence retractorius.

27

viii.

Embryonal tumors
Neuroblastoma is a small cell neoplasm with neuroblastic differentiation arising in the deep cerebral hemispheres
of young children (<5 yrs. of age). A variant is the ganglioneuroblastoma that has a preponderance of ganglion
appearing cells.
The olfactory neuroblastoma (esthesioneuroblastoma) is a neuroblastic tumor arising from the nasal epithelium
with cribiform plate involvement. There is a bimodal age distribution in adolescents and older adults. Patients
present with nasal obstruction and or epistaxis. Complete surgical resection with combined cranio-facial resection
is the treatment of choice, with a generally favorable prognosis. Adjuvant radiation therapy is generally
recommended.
Ependymoblastoma is a rare small cell embryonal neoplasm with prominent ependymoblastic rosettes. It typically
occurs in the cerebrum of children less than five years of age. Its propensity for craniospinal dissemination often
leads to death within a year.
Retinoblastoma is a retinal neoplasm that occurs in children < 3 years of age. Ophthalmoscopic exam is diagnostic,
giving the characteristic white reflex. These tumors have both hereditary (earlier onset and bilateral) and sporadic
forms. Surgical resection with or without radiation can be curative.

b.

Medulloblastoma or primitive neuroectodermal tumor (PNET) of the cerebellum is a small cell neoplasm believed to
arise from the external granular layer of the cerebellum. This tumor arises in the vermis of children and young
adults although cases in older patients have been reported. Radiographically, these are homogeneously enhancing
masses of the cerebellar vermis. CSF tumor seeding can produce drop metastases, even at the time of diagnosis.
Surgical resection, combined with radiation and chemotherapy, may lead to significant long-term survivals. Rarely,
metastasis to bone, lymph nodes and lung have been reported. Variants include desmoplastic medulloblastoma,
medullomyoblastoma, and melanocytic medulloblastoma. Medullomyoblastoma occurs in children, with a
propensity for boys. Cerebral (supratentorial) and spinal PNETs occur, but with much less frequency.
Tumors of cranial and spinal nerves
i.
Schwannomas

ii.

iii.

c.

Schwannomas (neurinoma, neurilemmoma) are tumors composed of Schwann cells that arise along cranial or
spinal nerves. The vestibular schwannoma (acoustic neuroma) is probably the most common schwannoma and
arises typically from the superior vestibular nerve. Vestibular schwannomas typically present with tinnitus and
sensori-neural hearing loss. Facial numbness follows when the tumor reaches approximately 2.5 cm. Ipsilateral
coordination difficulties and mild facial nerve weakness typically do not occur until the tumor diameter is greater
than 3 cm. Radiographically, these tumors enhance with contrast and extend into the internal auditory canal.
Complete surgical resection is curative. Hearing and facial nerve preservation is dependent on tumor size and
preoperative level of nerve function. In older patients or poor surgical candidates, stereotactic radiosurgery is an
effective treatment for tumor size < 2.5cm. Some proponents of radiosurgery feel that it should be used as the
primary treatment for all but the largest tumors. Bilateral vestibular schwannomas occur with neurofibromatosis
type II.
Neurofibromas
Neurofibromas are a nerve sheath tumor composed of Schwann cells, fibroblasts and perineural cells. This tumor
can occur in isolation or in associated with neurofibromatosis. This tumor may be solitary, plexiform or occur as a
mixed neurofibroma/schwannoma. These tumors arise from nerves in the subcutaneous tissue or in the
neuroforamena. Classic spinal tumors have a dumbbell appearance when they extend across the neuroforamena.
Surgical resection is indicated in symptomatic lesions. Malignant transformation in neurofibromas is rare but should
be suspected with increasing size or pain.
Malignant peripheral nerve sheath tumors

Malignant peripheral nerve sheath tumors: neurogenic sarcoma, anaplastic neurofibroma, and malignant
schwannoma are rare malignant tumors arising from the non-neural elements of nerves. These have a poor
prognosis with death resulting within the year. Complete resection is usually not feasible.
Tumors of the meninges
Meningioma (subtypes: meningothelial, transitional, fibrous, psammomatous, angiomatous, microcystic, secretory, clear cell,
choroid, lymphoplasmacyte-rich, metaplastic variants, atypical, anaplastic).

28

d.

Meningiomas are typically solitary, benign, slow growing, extra-axial tumors arising from arachnoid cap cells in the cranium
and spine. The most common locations are parasagittal, convexity, tuberculum sella and sphenoid ridge. A small percentage
may be intraventricular. Meningiomas rarely occur in children but when they do there is a predilection for the posterior fossa
and ventricle. Less than five percent of meningiomas are malignant, characterized by brain invasion and increased mitotic
activity. Meningiomas are most common in middle age and elderly women. Common symptoms include headache, seizures,
weakness, and mental status changes. Focal neurologic deficits on presentation depend on the site of origin of the tumor.
Radiographically these tumors are well circumscribed, homogeneously enhancing lesions. There may be hyperostosis of the
underlying skull. There is often a tail of dural enhancement at the edge of the tumor after contrast administration on imaging
studies. The primary treatment of symptomatic surgically accessible tumors is surgical resection. Surgery if complete is
often curative. Large lesions may require embolization of intra-operatively inaccessible vascular supply prior to surgical
resection to decrease intraoperative bleeding. Small or asymptomatic meningiomas in older individuals can be followed and
treatment recommended if growth is demonstrated. Radiation therapy and/or radiosurgery are treatment alternatives for
recurrent tumors or if surgery carries an increased risk of complications. There is an association between meningiomas and
neurofibromatosis type 2 and an abnormality in the long arm of chromosome 22.
Cysts and tumor-like lesions

e.

Numerous cysts and tumor-like lesions can occur in the brain including Rathkes cleft cyst, epidermoid cyst, dermoid cyst,
colloid cyst of the third ventricle, enterogenous cyst (neuroenteric cyst), neuroglial cyst, other cysts, lipoma, granular cell
tumor (choristoma, pituicytoma), hypothalamic neuronal hamartoma, nasal glial heterotopias.
Tumors of the anterior pituitary
Pituitary adenomas are slow growing, benign tumors that arise in the anterior pituitary gland. Pituitary tumors are divided
into hormone secreting tumors and non-secretors. Tumors less than 1 cm in diameter are referred to as microadenomas
while tumors larger than 1 cm are called macroadenomas. Prolactinomas are pituitary adenomas that secrete the hormone
prolactin. Prolactinomas often present with amenorrhea in women and loss of libido in men. The primary treatment for
prolactinomas is medication, usually bromocryptine analogues. Bromocryptine often results in a decrease in tumor size but
does not kill tumor cells. It usually must be continued for life or tumor recurrence is the rule. Adenomas that secrete growth
hormone produce gigantism if present before puberty and acromegaly if present after puberty. ACTH secreting adenomas
result in Cushings disease. Hypercortisolism is characterized clinically by centripetal obesity, moon facies, buffalo hump,
glucose intolerance, hypertension and impaired wound healing. Non-secreting macroadenomas with suprasellar extension
can compress the optic chiasm. Compression of the optic chiasm from below often produces a bitemporal hemianopsia.
Spontaneous hemorrhage or infarction of a pituitary adenoma is referred to as pituitary apoplexy and can result in sudden
visual loss or hypocortisolism. Emergent surgery is sometimes necessary for pituitary apoplexy.

f.

The primary treatment for patients with secreting pituitary adenomas producing Cushings disease, acromegaly, or
symptomatic non-secreting tumors is surgical resection. Surgery is typically performed through the transsphenoidal route.
Cavernous sinus invasion by adenoma cells often makes a surgical cure difficult. Recurrent tumors or those with cavernous
sinus invasion can be treated with re-operation or radiation therapy.
Metastasis

g.

Metastases are the most common brain tumor. Tumors that frequently spread to the brain, in order of decreasing incidence
are: lung, breast, skin, colon, and kidney. Commonly, lung, thyroid, renal cell and melanoma metastases can become
hemorrhagic. The treatment of solitary lesions is surgical resection followed by radiation therapy. For patients with multiple
asymptomatic lesions, surgery is reserved for diagnosis only. For patients with controlled systemic disease but multiple brain
metastases, a large symptomatic accessible lesion could be considered for resection. Whole brain radiation is generally
given for multiple brain metastases.
Brain Abscess
Brain abscesses arise by several mechanisms including hematogenous spread, penetrating trauma, surgery, or local spread
from the paranasal sinuses, mastoid air cells or emissary veins. The peak incidence is in young men due to the occurrence
of middle ear and paranasal sinus infections in addition to congenital heart disease. Other predisposing factors include
Osler-Weber-Rendu syndrome with pulmonary arteriovenous fistulae, endocarditis, congenital heart disease, dental work
and immunosuppression. Symptoms consist of headache, fever, seizures and/or neurological deficit. The majority of brain
abscesses are solitary.
Aerobic and anaerobic bacterial abscesses occur. Abscess cultures in one third of patients grow multiple organisms.
Common organisms based on the site of origin include Streptococcus species from the frontal/ethmoid sinus; Bacteroides
fragilis from chronic mastoiditis/otitis; Staph. aureus or enterobacteriacea following penetrating trauma or surgery; Strep.
viridans and Strep. pneumonia in cases of congenital heart disease; Staph aureus and Strep. pneumonia in cases of
endocarditis. In immunosuppressed patients, toxoplasma gondii, nocardia, mycobacteria, yeast and fungal abscesses occur.
Outside the US, tuberculomas, cysticercosis, echinococcus, schistosomiasis and strongoloidiasis are more common.

29

In the first stage of brain infection, there is inflammation of the brain, termed early cerebritis. This stage occurs in the first 3-5
days after inoculation. The CT scan appearance of cerebritis is that of an ill-defined hypodense contrast enhancing area.
This coalesces to a late cerebritis stage during days 4-13 with irregular rim enhancement. This is followed, at approximately
day 14, by a collagen reticulum encapsulation with a necrotic center (early capsule stage) On CT scan or MRI scan this
appears as a ring enhancing mass often with the abscess wall facing the ventricle appearing the thinnest. The final stage is
the late capsule stage in which there is a three-layer capsule: an outer gliotic layer, a middle collagenous layer and an inner
granulation layer. These can persist for months on imaging studies before ultimate resolution.
Antibiotics are the mainstay of treatment in all cases. Empiric treatment of a presumed bacterial abscess requires coverage
for both aerobes and anaerobes. Surgery is usually indicated to confirm the diagnosis of an abscess and for culture and
sensitivity of specific organisms. Stereotactic aspiration is the treatment of choice. Aspiration may need to be repeated
before resolution occurs. Often two to three weeks of antibiotic treatment are needed before a size decrease is seen on
imaging studies. In general 4 6 weeks of intravenous antibiotics are often used, followed by a period of oral antibiotics.
Patients with nocardia abscesses, or patients in whom treatment has failed after the third aspiration, should consider
surgical resection when accessible. Often, aspiration alone can treat significant mass effect and prevent rupture of the
abscess into the ventricular system. Ventricular rupture of a bacterial brain abscess is often fatal.
B.3. Diagnosis and Management of Headaches
a.

Major Causes of Intracranial Hemorrhage.

i.

Know the major causes of intracranial hemorrhage: Vasculopathy in the aged (hypertension and
amyloidosis), aneurysm, vascular malformation, tumor, and coagulopathy.
aa. Hypertension.
Spontaneous intracerebral hemorrhage may occur with both acute and
chronic hypertension. Acute hypertension sufficient to produce
spontaneous intracerebral hemorrhage is sometimes seen with eclampsia
and drug intoxication with sympathomimetic drugs. Chronic hypertension
may lead to vascular changes of the basal perforating arteries including
formation of Charcot-Bouchard aneurysms. The most common locations
for these hemorrhages are (in decreasing order of frequency):

Basal ganglia

Thalamus

Pons

Cerebellum

Cerebral white matter

Brainstem

bb. Amyloid angiopathy.

This is characterized by a deposition of beta amyloid protein within the
meningeal and cortical vessels. The clinical course is characterized by
multiple lobar intracerebral hemorrhages. Risk factors include advanced
age and Downs syndrome. Definitive diagnosis requires pathologic
examination.
cc. Aneurysm.
Cerebral aneurysms result from weakening of the wall of major
intracranial arteries. These aneurysms are generally saccular, though
fusiform aneurysms also occur. Saccular aneurysms usually occur at
branch points along the arterial tree. When a saccular aneurysm ruptures,
it most commonly produces subarachnoid hemorrhage. Intracerebral,
intraventricular, and subdural hemorrhage are also seen. Common sites
of aneurysm rupture include (in decreasing order of frequency)

30

Anterior communicating artery

Posterior communicating artery
Middle cerebral artery
Basilar artery
Vertebral artery

Multiple aneurysms are seen in approximately one-fourth of patients. Risk

factors include hypertension, smoking, family history, and collagen
vascular disease.
dd. Vascular malformation.
Vascular malformations include the high flow arteriovenous malformation,
cavernous angioma, venous angioma, and capillary telangiectasias.

The high flow arteriovenous malformation

results from a direct communication between the arterial blood
supply and draining veins without normal interposed capillaries.
These are usually visible on arteriography. Saccular aneurysm
are frequently seen on feeding vessels. Intraparenchymal
hemorrhage is most commonly seen, though subarachnoid and
subdural hemorrhage may occur.

Cavernous angiomas consist of irregularly

formed vascular channels, without intervening brain
parenchyma. Flow is usually low and these lesions, therefore, do
not routinely manifest themselves on arteriography. Hemorrhage,
when it occurs, is typically intraparenchymal. Family history is a
risk factor.
Venous angiomas result from a cluster of
normal medullary veins draining into a central enlarged venous
channel. The arterial system is uninvolved. Hemorrhage is
infrequent and usually intraparenchymal. Many are associated
with cavernous angiomas.
Capillary telangiectasias are clusters of dilated
capillaries with low flow. They are not visible on arteriography.
They are sometimes associated with Osler-Weber-Rendu
syndrome, Louis-Barr syndrome, Myburn-Mason syndrome, and
Sturge-Weber syndrome.

ee. Tumor.
Both primary and metastatic tumors may hemorrhage. Of the malignant
primary brain tumors, glioblastoma multiforme most commonly presents
with hemorrhage. While lung carcinoma is the most common hemorrhagic
cerebral metastasis, melanoma, choriocarcinoma, and renal cell
carcinoma are the most likely metastases to present with cerebral
hemorrhage. Most hemorrhages are intraparenchymal.
ff. Coagulopathy.
Even in the absence of underlying cerebral pathology, coagulopathy,
either secondary to underlying medical disease or iatrogenically induced,
increases the risk for intracranial hemorrhage. Particularly in elderly
patients, subdural hematoma is the most common manifestation.
Intraparenchymal hemorrhage may also occur, particularly in the setting
of anticoagulation after cerebral infarction.
b.

Recognize the Symptoms and Signs of Subarachnoid, Cerebral, and Cerebellar Hemorrhage.

31

i.

Subarachnoid hemorrhage.
The most common symptom of subarachnoid hemorrhage is explosive onset of severe headache. The headache is
described by the patient as "the worst headache of my life". The patient may complain of neck pain and stiffness,
as well as photophobia. Low back pain may also be present. Nausea and vomiting may be present. There may be
a history of transient loss of consciousness.

ii.

Signs of subarachnoid hemorrhage include nuchal rigidity, positive Kernigs sign, and/or positive Brudzinskis sign.
A depressed level of consciousness may also be present. Ocular manifestations include hemorrhages and
papilledema (secondary to elevated intracranial pressure). Focal neurologic deficit, particularly third nerve palsy
ipsilateral to the site of aneurysm rupture, may be present and in some instances may help to localize the
aneurysm.
Intracerebral hemorrhage.
Headache may be present after intracerebral hemorrhage. Patients with nondominant hemorrhages may complain
of weakness or numbness on their nondominant side.
Signs of intracerebral hemorrhage are typically those of hemispheric neurologic deficit. Patients with dominantsided lesions are frequently found to have hemiparesis and hemianesthesia. Dysphasia is usually present, though
the patient may present with frank aphasia. Patients with nondominant lesions typically have contralateral
hemiparesis and hemianesthesia. While speech is frequently normal, neglect may be profound.

iii.

If the hemorrhage is large, the patient may present with signs of elevated intracranial pressure including depressed
level of consciousness, decorticate or decerebrate posturing, or flaccid areflexia.
Cerebellar Hemorrhage.
The awake patient with cerebellar hemorrhage may complain of severe headache, which may be localized to the
suboccipital or upper cervical region.

c.

Symptoms include those of cerebellar and lower brainstem dysfunction, as well as hydrocephalus, which is
frequently present. Signs of cerebellar dysfunction include ipsilateral dysmetria, nystagmus when looking to the
side of the lesion, and difficulties with speech. Lower brainstem dysfunction typically manifests as difficulty with
swallowing. In the presence of hydrocephalus, signs of elevated intracranial pressure may ensue. These include a
decreased level of consciousness, decorticate or decerebrate posturing, and flaccid areflexia.
Apply Diagnostic Tools in Evaluation of Acute Headache (CT, MRI, and Lumbar Puncture).
In the patient who presents with the "worst headache of my life" a subarachnoid hemorrhage should always be suspected.
After initial resuscitation and complete neurologic examination, head CT without contrast is mandatory. In addition to
verifying the presence or absence of subarachnoid blood, the CT scan should be carefully scrutinized for other intracranial
pathology, including hydrocephalus.
If the CT scan is positive for subarachnoid hemorrhage, the patient should undergo cerebral arteriography. The arteriogram
should be scrutinized for the location of the aneurysm, as well as for any associated lesions including additional unruptured
aneurysms, arteriovenous malformations, and the presence or absence of vasospasm.
If the initial head CT is negative for subarachnoid hemorrhage, and the patient has a history that is highly suspicious for
subarachnoid hemorrhage, a lumbar puncture should be performed. A small needle should be utilized and only a small
volume of fluid should be withdrawn. Non-clotting blood, xanthochromia, or a red blood cell count greater than 100,000
which does not drop significantly from the first to the last tube are all highly suggestive of subarachnoid hemorrhage. If the
lumbar puncture meets any of these criteria, the patient should undergo arteriography as described above.

d.

If the cerebral arteriogram is negative, in the face of a CT scan indicative of subarachnoid hemorrhage or a positive lumbar
puncture, the patient should be admitted to the hospital for observation. At this point, an MRI scan of the brain with and
without contrast should be considered. MRI arteriography, if available, should be included. The purpose of this study is to
rule out other structural causes of subarachnoid hemorrhage including angiographically occult vascular malformations and
tumor. If no bleeding source is identified with the MRI scan, most clinicians recommend repeating the arteriogram in 7 to 14
days. Occasionally, an intracranial aneurysm that was obscured by mass effect, thrombosis, or vasospasm, may be
detected.
Understand the Natural History and Broad Treatment Strategies of Intracranial Aneurysms and Vascular
Malformations.
i.
Intracranial aneurysms - natural history.

32

aa. Ruptured intracranial aneurysms.

In the patient who survives their initial aneurysm rupture, the most serious
complication is rebleeding. The rate of rebleeding is approximately 4% in
the first 24 hours and decreases to 1 to 2% per day for the first two
weeks. After the first two weeks, the rate of rebleeding drops to
approximately 3% per year. A second potential serious complication of
aneurysmal subarachnoid hemorrhage is a vasospasm. Vasospasm is
defined as delayed narrowing of large and medium size arteries at the
base of the brain. If severe, this may lead to cerebral infarction. The risk
of symptomatic cerebral vasospasm is maximum between 4 and 11 days
of subarachnoid hemorrhage. The incidence of vasospasm, detected by
angiography, is approximately 70%. The incidence of symptomatic
vasospasm (resulting in neurologic deficit) is approximately 25 to 30%.
The major risk factor for the development of vasospasm is the amount
and location of subarachnoid blood visualized on the CT scan. This is
graded using the Fisher system.
Group Blood on CT Scan
1

No blood detected

Diffuse or vertical layers < 1mm thick

Localized clot and/or vertical layer >= 1mm thick

Intracerebral or intraventricular clot with diffuse or

no SAH

bb. Unruptured intracranial aneurysm - natural history.

Occasionally aneurysms are discovered prior to rupture. Again, the
primary risk is rupture. The available information suggests that the annual
rate of rupture for an unruptured intracranial aneurysm is between 1%
and 3% per year. The risk of rupture is affected by several factors. Larger
aneurysms, particularly those greater than or equal to 10 mm in diameter,
have a higher rate of rupture. Aneurysms located in the posterior
communicating artery, vertebrobasilar/posterior cerebral artery, and in the
basilar tip are more likely to rupture.
cc. Treatment strategies for intracranial aneurysms.
The goal of intracranial aneurysm treatment is to prevent bleeding or
rebleeding. Additionally, in the patient with a ruptured intracranial
aneurysm, treatment is also instituted to lessen the risk of symptomatic
vasospasm.
In patients with subarachnoid hemorrhage due to aneurysm rupture,
outcome is related to the modified Hunt-Hess grade. Higher-grade
patients have a statistically poorer outcome.
Grade Description
0

Unruptured aneurysm

Asymptomatic, or mild headache and slight

nuchal rigidity

1a

No acute meningeal/brain reaction, but with

fixed neurologic deficit

Cranial nerve palsy (e.g. III, IV), moderate to

severe headache, nuchal rigidity

33

Mild focal deficit, lethargy, or confusion

Stupor, moderate to severe hemiparesis, early

decerebrate rigidity

Deep coma, decerebrate rigidity, moribund

appearance

*Add one grade for serious systemic disease (e.g. HTN,

DM, severe atherosclerosis, COPD), or severe
vasospasm on arteriography
The patient with aneurysmal subarachnoid hemorrhage should be
admitted to the neurological intensive care unit. Adequate oxygenation
and respiration should be assured. The patient should be maintained
normotensive. Hypertension should not be treated unless the systolic
blood pressure rises above 160 mm of mercury. Hydrocephalus, which is
present in at least 15% of patients with subarachnoid hemorrhage, should
be treated with cautious external ventricular drainage if symptomatic.
Routine use of external ventricular drainage is controversial. Cerebral
selective calcium channel blockers lessen the risk of symptomatic
vasospasm and are started at this time. Use of anti-fibrinolytic agents and
corticosteroids may be considered.
Treatment of the ruptured intracranial aneurysm should at this point be
entertained. Direct surgical exclusion of the aneurysm from the circulation
(craniotomy and clipping) remains the "gold standard". Endovascular
obliteration of the aneurysm through the use of detachable coils may be
considered in certain situations (difficult or "unclippable" aneurysms,
medically unstable patient). The long-term results of this modality of
treatment are unknown and currently under study.
Treatment of unruptured aneurysms takes into account both the risk of
rupture, as well as age of the patient, severity and progression of
symptoms, and treatment alternatives. Older patients have a statistically
shorter life span and therefore lower cumulative risk of rupture. Recent
recommendations suggest that symptomatic intradural aneurysms of all
sizes should be considered for treatment. Unruptured aneurysms of all
sizes in patients with subarachnoid hemorrhage due to another treated
aneurysm should be considered for treatment. Treatment for unruptured
small (less than 10 mm) aneurysms is not generally recommended except
in special circumstances. These include aneurysms near the 10 mm size,
those with daughter sac formation, and in patients with family history of
aneurysm or aneurysmal subarachnoid hemorrhage. Asymptomatic
aneurysms greater than or equal to 10 mm in diameter warrant strong
consideration for treatment.

ii.

Arteriovenous Malformations - Natural History

aa. Risks.
The primary risk of arteriovenous malformations is rupture. The rate of
rupture has been estimated to be approximately 4% per year for
symptomatic arteriovenous malformations and 2% per year for
asymptomatic arteriovenous malformations. The rate of arteriovenous
malformation rupture appears to be higher in small malformations than in
large malformations. The second risk of arteriovenous malformation is
seizures. Nearly 60% of patients with an arteriovenous malformation have
a history of seizures. Larger malformations are associated with a greater

34

risk of preoperative seizures.

bb. Treatment strategies for arteriovenous malformations.
Treatment for arteriovenous malformations is recommended when the risk
of subsequent hemorrhage is greater than the risk of treatment. The risk
of treatment may be estimated using the Spetzler-Martin classification:
Points
Assigned

Graded Feature
Size of arteriovenous malformation
Small (<3cm)

Medium (3-6cm)

Large (>6cm)

Eloquence of adjacent brain

Noneloquent

Eloquent

Pattern of venous drainage

Superficial only

Deep

Grade = [size] + [eloquence] + [venous drainage]

There are several treatment options for arteriovenous malformations.
These include craniotomy and direct surgical excision, embolization using
interventional radiologic techniques, and Gamma Knife radiosurgery.
Because the risk of treatment rises with increasing Spetzler-Martin grade,
the following recommendations have been made:
Grade I or II arteriovenous malformations
should be treated.

Grade III lesions should be treated if

symptomatic.

Grade IV or V arteriovenous malformations

should be treated only when significant or repetitive intracerebral
hemorrhage has occurred or the patient is experiencing
progressive neurologic disability.

The treatment modality of choice remains

controversial. Surgical removal remains the "gold standard". With
all treatment modalities, the goal remains complete removal or
obliteration of the arteriovenous malformation. This goal should
be pursued until it has been achieved.
Differentiate the Symptomatology of Migraine, Cluster, and Tension Headache, and Sinusitis Headache.
i.
Migraine headache. Features of classical migraine headache include:
Onset in childhood, adolescence or early adulthood.
Positive family history.
More common in females.
Usually unilateral, throbbing pain.
Nausea and vomiting common.
Scintillating scotomata.
Duration: Several hours.

e.

35

ii.

Cluster headache.

iii.

Tension headache.

iv.

Much more common in men.

Pain severe and retro-orbital or temporal, usually unilateral.
Pain at night.
Accompanied by lacrimation, rhinorrhea, periorbital edema.
Duration of pain 20 to 60 minutes and may recur several times within a 24-hour period.
Pain-free period of months to years is typical.

Onset in adolescence or young adulthood.

Non-familial.
Pain is typically bilateral, bifrontal, bitemporal, or suboccipital.
Pain described as throbbing or a sensation of "pressure".
Nausea and vomiting rare, no lacrimation or rhinorrhea.
More frequent in afternoon or evening.
Duration hours to days.
Precipitated by stress, depression, or anxiety.

Sinusitis.

History of allergic background or frequent prior sinusitis.

Unilateral or bilateral.
Localized pain and tenderness over affected sinuses common.
Associated conditions include nasal obstruction, fevers, and chills. No history of scintillating scotomata,
nausea, or vomiting.
Headache persists until sinusitis is cleared.

References
1. Bederson JB, Awad IA, Wiebers DO, Et all. Recommendations for the management of patients with unruptured intracranial
aneurysms. Stroke 31:2742, 2000.
2. Fisher CM, Kistler JP, Davis JM. Relation of cerebral vasospasm to subarachnoid hemorrhage visualized by CT scanning.
Neurosurgery 6:1-9, 1980.
3. Hamilton MG, Spetzler RF. The prospective application of a grading system for arteriovenous malformations. Neurosurgery
34:2-7, 1994.
4. Hunt WE, Kosnik EJ. Timing and perioperative care in intracranial aneurysm surgery. Clin Neurosurg 21:79-89, 1974.
5. Ondra SL, Troupp H, George ED, Schwabb K. The natural history of symptomatic arteriovenous malformations of the brain:
A 24-year follow-up assessment. J Neurosurg 73:387-391, 1990.
6. Piepgras DG, Sundt TM, Ragoowansi AT, Stephens L. Seizure outcome in patients with surgically treated cerebral
arteriovenous malformations. J Neurosurg 78:5-11, 1983.

B.4. Diagnosis and Management of Ischemic Cerebrovascular Disease

36

Knowledge of the symptoms and signs of occlusive cerebrovascular disease is necessary for effective diagnosis and management of
these patients. Symptoms are often transient, may be subtle, and can be unappreciated by both patient and physician. As most
occlusive cerebrovascular is secondary to atherosclerosis of the internal carotid artery, the following brief review will focus on patients
with this particular problem.

a.

Transient Ischemic Attack

Transient ischemic attack (TIA), a transient neurological deficit secondary to dysfunction of a part of the cerebral
hemisphere because of a lack of blood flow, is the most common symptom in patients with atherosclerotic carotid artery
disease. TIA has been traditionally defined as lasting less than 24 hours.
Vague symptoms such as dizziness, confusion and blurred vision are not TIAs. Unilateral weakness, clumsiness and
numbness are the most common symptoms but dysphasia and dysarthria may also occur. The diagnosis is based on a
careful history, as most patients will not have discernible neurological deficits when being examined. Many conditions,
including seizures, intracranial tumors and hematomas, cardiac dysrhythmias, hypoglycemia and migraine may produce
symptoms similar to TIAs. These other etiologies must be considered in a patient suspected of having TIAs.

b.

c.

Amaurosis Fugax
Amaurosis fugax (AF), a transient loss of vision involving one eye, is the second most common presenting symptom in
patients with atherosclerotic carotid artery disease. This can be confused with a transient visual problem involving both eyes
in one visual field and careful questioning will be necessary to differentiate between the two. Many patients will not have
tested each eye separately during an episode of visual loss, in which case differentiation may be impossible.
Evaluation of Suspected atherosclerotic Carotid Artery Disease
i.
General

ii.

iii.

d.

As indicated above, patients with suspected carotid artery stenosis need a careful history to identify symptoms
suggestive of AF or TIA and to assess their risk factors for generalized atherosclerotic vascular disease. Such risk
factors include advanced age, smoking, diabetes, hypertension, hyperlipidemia, peripheral vascular occlusive
disease, coronary artery disease and a positive family history of stroke or myocardial infarction. General physical
and neurological examinations looking for evidence of peripheral and cerebrovascular arterial stenosis or
neurological deficit is done following a careful medical history. The presence of a cervical bruit on auscultation of
the neck may indicate carotid artery stenosis producing turbulent blood flow. This is the most common physical
finding in patients with clinically significant atherosclerotic carotid artery disease but its absence does not rule out
the diagnosis. Diminished peripheral pulses may indicate generalized atherosclerotic vascular disease.
Fundoscopic Examination
Cholesterol emboli may occasionally be seen in retinal vessels of patients with atherosclerotic carotid artery
disease with or without AF.
Laboratory Evaluation

Patients with TIAs should have a study such as a cranial CT or MRI scan to rule out intracranial lesions. Imaging of
the carotid arterial system with carotid duplex ultrasonography, magnetic resonance angiography, CT angiography
or digital subtraction angiography should also be done if atherosclerotic carotid artery disease is suspected from
the history and/or examination. Echocardiography may also be indicated to look for a cardiac source of embolism.
Screening laboratory evaluation to look for evidence of coagulopathy (PT, PTT, INR), vasculitis (ESR) or
hyperlipidemia should also be considered.
Indications for Carotid Endarterectomy
Patients with symptomatic atherosclerotic carotid artery disease producing a 70% or greater diameter stenosis of the
internal carotid artery disease benefit from surgery if the perioperative morbidity and mortality is less than 7%. Patients with
asymptomatic disease and greater than 60% stenosis benefit from surgery if perioperative complications are less than 3%. If
in doubt as to the potential benefit of carotid endarterectomy patients should be referred to skilled cerebrovascular surgeon
for evaluation.

C.1. Diagnosis and Management of Spinal Cord Injury

a.

The emergency room diagnosis and interpretation of radiologic studies in spinal trauma

37

i.

Diagnosis:
aa. Presentation:
There are approximately 11,000 new spinal cord injuries per year.
Mechanism of Injury:

Motor vehicle crashes: 45%

Falls: 22%
Sports: 14%
Violence: 14%
Other: 5%

Most patients (75%) are males. Sixty percent are between the ages of 16
and 30. Slightly more than half of all patients with cervical spine trauma
will present with signs of neurologic injury. Up to 10% of patients with
cervical spine trauma will develop signs of neurologic injury after
presentation.
bb. Association with other injuries
Five to ten percent of unconscious patients who have been involved in a
fall or motor vehicle accident will have a cervical spine injury. Conversely,
60% of patients with a cervical spine fracture will have at least one major
associated injury. Five to fifteen percent of patients with a cervical spine
fracture will have a second vertebral column fracture.
cc. History and physical examination clues to the presence of spinal
injury
Pain
-local pain, due to bone/soft tissue injury
-radicular pain, due to nerve root compression
Vitals
-bradycardia indicating loss of sympathetic input to the heart from cervical
or high thoracic lesion
-hypotension due to loss of sympathetic input tosystemic vasculature
("spinal shock")
External
-tenderness, bruising, or swelling due to local soft tissue damage
-palpable step-off due to malalignment
-paraspinous muscle spasm
-torticollis due to muscle spasm or unreducedvertebral dislocation
Weakness
-partial vs. complete
-level: motor level is defined as the most caudal level with grade III
(antigravity) strength, assuming that more cranial levels have grade IV
strength
Sensation
-decreased vs. absent vs. hyperesthesia
-level : sensory level is defined as the most caudal dermatome with

38

normal sensation
-saddle region needs to be tested - may be the only region of preserved
sensation ("sacral sparing")
Reflexes
-normal vs. absent vs. increased
-absent may indicate the presence of spinal shock
-increased may indicate the presence of an older complete or incomplete
injury
Sphincter
-decreased or absent tone
-loss of voluntary contraction
-loss of bulbocavernosis reflex
dd. In the comatose or intoxicated patient
The comatose or intoxicated may be unable to transmit information
regarding pain or cooperate with strength or sensory testing. Intracranial
injury may co-exist with spinal injury. Therefore one must assume that all
comatose or intoxicated trauma patients have a spinal injury until proven
otherwise.
Clues to the presence of a spinal injury in such a patient include:
-flaccid areflexia
-diaphragmatic breathing
-loss of grimace to painful stimuli below specific level
-loss of withdrawal response below a specific level
-loss of spontaneous movement below a specific level
-loss of sphincter tone
-priapism
Frankel grading system
Frankel Grade Designation

Definition

Complete
neurological
injury

No motor or sensory function

detected below level of lesion

Preserved
sensation only

No motor function detected

below level of lesion, some
sensory function below level of
lesion preserved

Preserved
motor,
nonfunctional

Some voluntary motor function

preserved below level of lesion
but too weak to serve any useful
purpose, sensation may or may
not be preserved

Preserved
motor, functional

Functionally useful voluntary

motor function below level of
injury is preserved

39

Normal motor
function
ii.
iii.

Normal motor and sensory

function below level of lesion,
abnormal reflexes may persist

Radiologic studies
aa. Plain x-rays
Static:
-Need to see down to the C7-T1 disc space on the lateral plain x-rayof the
cervical spine
-Anterior-Posterior and Lateral (Cervical/Thoracic/Lumbar)
Evaluate for the presence of:
-Prevertebral soft tissue swelling
-Malalignment
-Vertebral body fractures or loss of vertebral body height
-Posterior element fractures (spinous process, transverse process,
lamina, facet)
-Widening of interpedicular distance
-Fracture of dens
-Open Mouth Odontoid (Cervical)
-Evaluate for the presence of:
-Fracture of dens
-Widening of lateral masses of C1
Dynamic (Flexion/Extension):
-Performed only if static x-ray are normal but there is a high suspicion of
ligamentous injury not be visible on static x-rays
-Can only be performed in the conscious cooperative patient
-Evaluate for the presence of:
-Vertebral subluxation
-Widening of distance between dens and arch of C1
-Abnormal kyphosis or opening up of posterior elements in flexion
bb. Computed axial tomography
-Has largely replaced conventional polytomography
-Most useful for assessing bone detail; poor visualization of neural
elements
-Usually performed whenever the plain x-rays are abnormal in order to:
-More completely characterize a known fracture
-Rule out spinal canal compromise due to indriven bone fragments with a
known fracture
-Rule out occult fracture in the presence of known ligamentous injury

40

-Occasionally used to image the lower cervical spine when this cannot be
done using plain x-rays
-May be combined with intrathecal contrast (myelography) in order to
visualize neural elements when MRI is contraindicated or not available
cc. Magnetic resonance imaging (MRI)
-Most useful for visualizing soft tissue structures
-Resolution of bone inferior to computed axial tomography
-May be performed when plain x-rays are abnormal in order to rule out:
-Spinal cord injury/hematomyelia
-Traumatic intervertebral disc herniation
-Hematoma
-Ligamentous injury
b.

Initiate acute management of spinal cord injury including immobilization, steroids and systemic measures
i.

Immobilization
aa. Cervical
Traumatic cervical spine injury should be presumed to be present until it is
ruled out by physical examination and appropriate radiographic studies. In
order to prevent new or additional neurologic injury, immobilization of the
cervical spine should be instituted as soon as possible. Patients with a
known or suspected cervical spine injury, or those who are comatose or
intoxicated at the scene of injury, should ideally be placed in a cervical
orthosis at the scene. Alternatively, if an appropriate cervical orthosis is
not available, the head and neck may be immobilized by placing
sandbags or towel rolls on either side of the head.
If a cervical spine injury is found radiographically, it is usually imperative
that cervical spine immobilization be continued until definitive
management is complete. In some patients this may consist of continued
treatment in a cervical orthosis, combined with bed rest.
In other patients, especially those with neurologic injury, cervical spinal
instability, or unreduced cervical spinal dislocation, in-line cervical traction
may be used. Cervical traction is frequently applied using Gardner-Wells
tongs secured to the skull. Five pounds of weight are initially applied.
More weight is occasionally added in an attempt to reduce a spinal
dislocation. While the patient is in cervical traction, neurologic
examinations should be performed frequently. Radiographic examinations
should be performed after the initial application of weight and after any
subsequent change to assess for changes in spinal alignment and the
presence of overdistraction (pulling apart of the spine). Weight should be
immediately reduced if a decline in the neurologic examination or
overdistraction occurs. Cervical traction is contraindicated in certain highly
unstable cervical injuries, as well as in occipital-cervical dislocation.
Finally, some patients may initially be placed in a halo vest. In addition to
effective immobilization of the upper and middle cervical spine, placement
in a halo vest may constitute definitive therapy for a variety of lesions.
bb. Thoracolumbar
As with the cervical spine, thoracolumbar spine fracture should be

41

presumed to be present until it is ruled out by physical examination and

appropriate radiographic studies. Present extrication and transport
techniques emphasize maintenance of a neutral position during the
prehospital phase. Most commonly, patients are transported to the
hospital on a backboard. They should not be allowed to sit or stand prior
to evaluation. During the physical examination, patients should be
carefully logrolled by multiple personnel for examination of the back.
Unless the patient has a highly unstable lesion, the backboard may be
removed while the patient is flat in bed. Whenever the patient is moved,
for example to the CT or MRI scanner, the patient should be carefully
placed back onto the backboard.
ii.

Steroids
aa. Spinal cord injury
Results of the NASCIS II study showed that patients with spinal cord
injury who were treated with methylprednisolone within eight hours of
injury had significantly greater improvement in their neurologic function
(motor, pinprick sensation, and touch sensation) than those given a
placebo. The dose administered in the study was 30mg/kg given as a
bolus over 15 minutes, followed by an infusion of 5.4 mg/kg/hr for 23
hours, begun 45 minutes after completion of the bolus.
Results of a follow-up study, NASCIS III, concluded that patients with
acute spinal cord injury who receive methylprednisolone within 3 hours of
injury should be maintained on the treatment regimen for 24 hours. When
methylprednisolone is initiated 3 to 8 hours after injury, patients should be
maintained on steroid therapy for 48 hours.
bb. Cauda equina injury
No data from randomized, controlled studies.
cc. Gunshot wounds to the spinal cord
No data from randomized, controlled studies. However, a recent
retrospective study of 254 patients reported no neurological benefit from
intravenously administered steroids after a gunshot wound to the spine
(C1-L1). Both infectious and noninfectious complications were more
frequent in the group receiving steroids. It was recommended that
patients with spinal cord injury secondary to a gunshot wound to the spine
not be treated with steroids.

iii.

Systemic measures
aa. Respiratory
Maintenance of an adequate airway and breathing remains the first
priority in the trauma patient. Spinal cord injured patients may suffer from
inadequate respiratory function due to paralysis of the intercostal muscles
or diaphragm. Concomitant injuries may also compromise respiratory
function. Maintenance of spinal alignment is critical during intubation. If
endotracheal intubation is needed, it is best performed in conjunction with
in-line cervical traction, nasotracheally (if there is no evidence of basilar
skull fracture) or fiberoptically.
bb. Cardiac
After airway and breathing abnormalities are treated, adequate perfusion
must be assured. Hypotension in the patient with a spinal injury may be
due to intravascular volume loss due to bleeding or spinal cord injury.
These two processes may co-exist. Identification and control of bleeding

42

is the first priority. Hypotension, due to loss of sympathetic vascular tone,

and bradycardia, due to loss of sympathetic innervation of the heart, are
the most important elements of spinal shock due to spinal cord injury.
Initial treatment of spinal shock consists of intravascular volume
expansion. In patients refractory to this therapy, sympathomimetic drugs
may be needed
cc. Genitourinary
Placement of an indwelling urinary catheter is mandatory in the severely
injured patient for monitoring of urine output volume. In the patient with a
spinal injury, urinary catheterization during the period between
identification of the injury and definitive treatment facilitates nursing care
and avoids unnecessary patient movement. In the neurologically injured
patient, the bladder may not function normally. Continuous urinary
drainage prevents the complication of bladder rupture due to
overdistension.
c.

Understand the definition and subsequent management principles of the unstable spine
i.
Unstable spine: Definition
Spinal instability has been defined as "the loss of the ability of the spine under physiologic loads to maintain
relationships between vertebrae in such a way that there is neither damage nor subsequent irritation to the spinal
cord or nerve roots and , in addition, there is no development of incapacitating deformity or pain due to structural
changes." Notice that this is a clinical definition.

ii.

Numerous sets of radiographic criteria have been developed in an attempt to predict which patients are or will
become unstable after a spinal injury. The most commonly used is the three-column model of Denis. In this model,
the spine is divided into a posterior, middle, and anterior column. The posterior column includes all of the posterior
bony and ligamentous elements while the middle column includes the posterior longitudinal ligament and all of the
elements comprising the posterior one third of the vertebral body and intervertebral disc. The anterior column is
comprised of the remaining portions of the vertebral body and intervertebral disc, as well as the anterior
longitudinal ligament. Injuries with incompetence of two or three columns are inferred to be unstable. The threecolumn theory applies to the thoracolumbar spine only.
Management principles
As described above, initial management of the unstable spine consists of immobilization of the injured vertebral
segment while the patient is being stabilized and other injuries are being ruled out. After this initial phase,
management decisions are generally based on three factors:
aa. Need for decompression of neural elements.
General indications for neural decompression will be discussed in the
next section. It is important to consider that, in some cases,
decompressive procedures may further destabilize the unstable spine or
render the stable spine potentially unstable.
bb. Need to mobilize the patient as soon as possible.
Some unstable spinal injuries may be potentially treatable with prolonged
bed rest. However, by avoiding prolonged periods of bed rest, early
surgical stabilization of the unstable spine may help to prevent
atelectasis, pneumonia, deep venous thrombosis, and decubiti. In
addition, early stabilization allows the patient to begin rehabilitation
earlier, potentially reducing the complications of joint contracture and
deconditioning, as well as potentially reducing time off work.
cc. Need to stabilize the spine that is not likely to heal without surgical
intervention.
In general, injuries that are not likely to heal include those with widely
displaced fractures, unreduced or unreducible dislocations, severe

43

deformity, or severe ligamentous injury. Also include in this group are

injuries which have been treated with prolonged bed rest or bracing which
have not healed correctly.
Surgical stabilization has two components. The first component is
arthrodesis, or fusion. The goal of fusion is to induce adjacent vertebrae
above and below the injury to heal together into a solid block of bone,
eliminating any potential movement between them. This usually involves
placement of bone graft between the vertebrae. Bone graft may be placed
anteriorly, between adjacent vertebral bodies, or posteriorly, between
adjacent laminae, facets, or transverse processes.
The second component of surgical stabilization involves internal fixation
(instrumentation). This provides immediate strength and maintains
anatomic alignment during the time it takes for fusion to occur. Internal
fixation usually involves the implantation of some combination of wire,
hooks, screws, and/or rods. Internal fixation is not a substitute for fusion.
A general principle is that all internal fixators will eventually fail if fusion
does not occur.
d.

Understand management principles in spinal cord injury including indications for decompressive surgery and
treatment of the medical complication associated with cord injury (skin, bladder, bowel movement, respiratory)
i.
Indications for surgery
The most compelling indication for decompressive surgery in spinal cord injury is the presence of an incomplete
neurologic injury with persistent neural compression at the site of injury. Compression may be due to indriven bone
fragments, traumatic disc herniation, epidural hematoma, or persistent vertebral malalignment. While there is
currently debate regarding the most appropriate timing of decompression in the presence of a stable neurologic
examination, most clinicians agree that it should be done emergently in the presence of a rapidly declining
neurologic examination.

ii.

The goal of decompressive surgery is restoration of a normal spinal canal without additional injury to the neural
elements. This, in theory, will facilitate neurologic recovery. A review of the most common causes of neural
compression reveals that most are ventral processes. Therefore, technically demanding ventral decompressive
procedures are frequently necessary. As has been previously stated, these procedures may further destabilize the
unstable spine or render the stable spine potentially unstable.
Treatment of medical complications associated with cord injury
aa. Skin:
Patients with spinal cord injury are at particular risk for skin breakdown
due to inability to sense pain and prolonged periods of bed rest. This may
be further aggravated by incontinence and the necessity of wearing an
orthosis. All insensate regions should be surveyed regularly. While in bed,
spinal cord injured patients should be turned every two hours. Bony
prominences (sacrum, heels) should be protected. Shearing forces, which
frequently occur during transfers, should be avoided. Bowel and bladder
dysfunction should be treated accordingly as described below. All
orthoses and wheel chairs should be fitted appropriately and adjusted to
accommodate weight loss or gain.
bb. Bladder:
Patients with spinal cord injury frequently suffer from some form of urinary
bladder dysfunction. This may manifest as a lower motor neuron bladder
with overflow incontinence and urinary retention. Alternatively, an upper
motor neuron bladder may be present with incontinence and reduced
bladder capacity. In either instance, the goals of treatment are to maintain
a functional lower urinary tract free of infection and to preserve renal
function. As has been described above, the initial treatment of urinary
bladder dysfunction is usually placement of an indwelling urinary catheter.

44

After the acute period, sterile, intermittent catheterization is preferable to

the long-term use of an indwelling urinary catheter. Intermittent
catheterization should initially be performed every 4 hours and urinary
volumes should not be permitted to exceed 450cc. If possible, patients
may be taught to perform self-catheterization. Renal function should be
closely monitored long-term.
cc. Bowel movement:
Patients with spinal cord injury also frequently suffer from some form of
bowel dysfunction. The goals of management are for the patient to be free
of incontinence and to develop a predictable bowel routine without fecal
impaction. Patients with lower motor neuron bowels frequently require
manual removal of feces. Those with upper motor neuron bowels may
require daily digital rectal stimulation or suppositories. Adequate daily
fluids and fiber intake facilitate development of a bowel routine. Diarrhea
may be due either to excessive laxative intake or fecal impaction.
dd. Respiratory:
All trauma patients are susceptible to atelectasis and pneumonia during
the acute phase. This is especially true in the patient with concomitant
traumatic brain injury. Incentive spirometry should be instituted early in
treatment. If the patient is not able to participate, consideration should be
given to the use of intermittent positive pressure breathing (IPPB).
Additional problems arise in the spinal cord injured patient. The phrenic
nerve is supplied by the C3, C4, and C5 roots. Patients with a C4 or
higher level of injury most often require placement of a tracheostomy and
the assistance of a ventilator. Patients with a C6 or higher level of injury
will require some assistance with cough and clearing of secretions.
Patients with a low cervical or thoracic level of injury remain at higher risk
for atelectasis, pneumonia, and respiratory failure, despite normal phrenic
nerve function, due to dysfunction of the intercostal musculature. All
should be started on incentive spirometry, as well as exercise programs to
increase strength in the remaining muscles of inspiration. Prophylactic
clearing of secretions should be encouraged.

References:
1.
2.
3.
4.
5.
6.
7.

Bracken MB, Shepard MJ, Collins WF et al. A randomized, controlled trial of methylprednisolone or naloxone in the
treatment of acute spinal-cord injury. NEJM 322:1405-1411, 1990
Bracken MB, Shepard MJ, Holford TR et al. Administration of methylprednisolone for 24 or 48 hours or tirilizad mesylate for
48 hours in the treatment of acute spinal cord injury. JAMA 277:1597-1604, 1997
Denis F. The three column spine and its significance in the classification of acute thoracolumbar injuries. Spine 8:817, 1983
DeVivo MJ, Rutt RD, Black KJ, et al. Trends in spinal cord injury demographics and treatment outcomes between 1973 and
1986. Arch Phys Med Rehabil 73:424, 1992
Frankel HL, Hancock DO, Hyslop G et al. Value of postural reduction in initial management of closed injuries of the spine
with paraplegia and tetraplegia: Part 1. Paraplegia 7:179, 1969-1970
Heary RF, Vaccaro AR, Mesa JJ et al. Steroids and gunshot wounds to the spine. Neurosurgery 41:576-584, 1997
White AA, Panjabi MM. Clinical Biomechanics of the Spine. Philadelphia, Lippincott, 1978

45

C.2. Diagnosis and Management of Non-Traumatic Neck and Back Problems

Introduction
Degenerative diseases of the spine provide the typical neurosurgeon with the bulk of his or her practice. Low back pain is the most
common specific complaint generating a physician visit, and is the most often cited reason for missed work. It is estimated that 75%
of people will visit a health care professional at some time in their lives for back pain. Estimates of prevalence suggest that at any
given time 1 in 5 adults in the US will complain of low back discomfort if asked.
The direct and indirect economic impact of low back complaints is enormous. Direct costs of treatment are estimated at $20-30 billion
a year. Some 200 million person-days of work are lost per year due to low back problems. Low back pain has recently ranked 6th in
hospital-bed days per year. Clearly, efficient and effective management of degenerative conditions of the spine is crucial, both at the
level of the primary care provider, and at the level of the subspecialty spine surgeon.
The purpose of this chapter is to give the student an overview of how patients present with degenerative complaints of the lumbar
and cervical spine. The focus will be on identifying symptom complexes that point to specific pathological entities that are surgically
treated. Special attention will be paid to identifying signs and symptoms that are suggestive of serious pathology that may threaten
life or neurologic function and require urgent attention.

a.

Categories of Symptoms:
In order to have an orderly approach to the patient with spine related complaints, the physician needs to be familiar with the
broad categories of symptoms with which patients will present. The differential diagnosis of spine related pain is extensive.
Although the vast majority of patients have benign, non-surgical problems, very serious medical illness can present with the
chief complaint of back, neck, or extremity pain. With experience, common presenting syndromes emerge, and allow more
rational triage of patients into groups who need further evaluation, and perhaps surgical referral, and those who are best
managed without extensive testing and referrals.

i.

Axial Spine Pain

Pain in the spine with or without radiation into the extremities is the most common presenting compliant related to
spinal disease. Initial evaluation should center on obtaining a detailed history of the patients complaints and
previous medical history. A history of recent trauma should raise the suspicion of a fracture. Character, severity,
frequency, inciting and relieving events should be thoroughly explored. Inquiry about associated symptoms, such
as fever and visceral pain can suggest diagnoses and avenues of investigation. The timing of pain can also lead to
appropriate investigation. Morning stiffness and pain that relents as the day progresses suggests an inflammatory
disorder, where nocturnal pain associated with recumbency is a much more ominous symptom, being seen with
malignant, destructive lesions.
Activity-related back pain without neurologic symptoms or findings is quite common. In the absence of other
worrisome symptoms or findings, most patients can be successfully managed with a brief reduction in activity level
and analgesics. The optimum duration and content of non-operative treatment is controversial. Some practitioners
advocate strict bed rest, while others avoid it. Reduction of general activity level while avoiding any specific inciting
events, coupled with non-steroidal agents, is the mainstay of most clinicians first line therapy.

ii.

Evaluation for pure axial spine pain that does not resolve with conservative measures can include plain
radiographs, magnetic resonance imaging, computed tomography, myelography, electrophysiologic studies, and
discography. A set of plain films can exclude fracture, and identify serious bony pathology such as a destructive
tumor. However, for soft tissue imaging the MRI is the study of choice. CT can give detailed information about bony
anatomy. Myelography is sensitive to canal and foraminal pathology, and when paired with a post-myelogram CT
scan, can be very useful in the patient in whom an MRI is not an option. Discography is controversial, and will not
be discussed at length.
Radiculopathy
Referred pain into the upper or lower extremities often accompanies back or neck pain. Referred pain can be the
initial symptom of a compressed nerve root by a ruptured disc or neural foraminal stenosis from osteophytes.
Radicular pain is usually described as sharp or even shock-like, and may be associated with certain activities or
positions. The distribution of the pain may not always be classic, and often doesnt respect dermatomal
distributions.

46

Sensory changes are also often seen, with complaints of tingling and numbness being very common. On
examination decreased sensation to pinprick and light touch are found in a dermatomal distribution in many
patients. It is interesting that areas of referred pain and sensory loss often are different. Making determinations of
level of nerve root compression solely from pain or sensory distribution is often difficult.
Motor weakness is also seen in nerve root compression syndromes. Muscle innervation is more constant and has
less overlap than sensory innervation and is better at predicting level of pathology. Motor deficits that are of a more
long-standing nature can have significant wasting. Hyporeflexia in the appropriate distribution is also seen.
aa. Cervical
Cervical radiculopathy can present acutely, as with a traumatic ruptured
disc, or can be of a more chronic and intermittent nature, as is seen in
foraminal narrowing from osteophytes. Typically, the inferior nerve root is
affected (e.g. C5-6 disc abnormalities affect the C6 nerve root). C5-6 and
C6-7 are the most commonly affected segments.
A C5 radiculopathy typically presents with pain in the shoulder and the
upper part of the lateral arm. Paresthesias are often seen in the more
distal part of the affected dermatome. Deltoid weakness is seen
commonly with a C5 radiculopathy. Biceps or brachioradialis weakness
can be seen with a C6 radiculopathy along with the appropriate
hyporeflexia. Paresthesias and frank sensory loss are more distal, and
can extend into the hand. Root compression at C7 produces triceps
weakness and a decreased triceps reflex. Pain extending into the distal
forearm or hand is common. Sensory loss is commonly seen in the hand.
bb. Lumbar
Sciatica is a classic syndrome of lower lumbar nerve root compression.
Low back pain, that may or may not have been associated with some sort
of trauma, is commonly antecedent to the onset of leg pain by days to a
few weeks. Pain tends to be more proximal, and in a slightly different
distribution than sensory changes. Motor weakness is also seen, but can
be missed if dynamic testing is not done. All patients should be asked to
stand on their toes and heels, as confrontational testing will miss subtle
motor deficits in the lower extremities. As in the cervical spine, the
pathologic level usually affects the caudal nerve root (e.g. L5-Sl disc
produces an S1 radiculopathy). L5-S1 and L4-5 are overwhelmingly the
most common levels affected. The upper lumbar spine is affected less
frequently.
The classic S1 radiculopathy results in pain down the back of the leg and
into the heel or foot. Sensory loss is usually over the lateral aspect of the
foot. Plantar-flexion weakness is seen, but can be subtle. A loss of the
Achilles reflex is also fairly specific to S1. The L5 radiculopathy produces
similar pain, but the sensory symptoms tend to be over the dorsum of the
foot. Weakness in dorsiflexion of the foot (or more specifically extensor
hallicus longus) is the motor finding associated with L5. There is not a
reliably reproducible reflex associated with L5.

iii.

Cauda Equina Syndrome

The cauda equina syndrome is important to recognize, as prompt surgical attention may be necessary in cases of
acute pathology. Patients typically present with low back pain and diffuse lower extremity complaints. Minimal or
absent leg pain is seen, in contrast to the predominance of extremity pain seen in the radicular syndromes. Bowel,
and, more commonly, bladder dysfunction, are also seen. In many patients this goes unrecognized until bladder
distention leads to overflow incontinence. Because of the vague nature of the complaints, and the common lack of
severe pain associated with a large disc herniation producing a cauda syndrome, delay in diagnosis is not
uncommon. Patients with back pain and complaints of lower extremity weakness should be carefully examined to
rule out a cauda equina syndrome. Weakness can be diffuse, and vary from subtle to paraplegia. Sensory findings

47

are variable, but often found in the perineal area. Checking a post void residual can give a quick initial assessment
of bladder function, and should be done prior to placing a foley. Reflex changes are variable, but in general reveal
diffuse hyporeflexia. Patients in whom the diagnosis is suspected should undergo urgent imaging with MRI or a
myelogram.

iv.

Recovery from a severe cauda equina syndrome, even with prompt surgical management, can be very slow and
incomplete. Bladder function is often the slowest symptom to improve. This is another reason to keep a high index
of suspicion for this uncommon entity in patients presenting with back pain and complaints of lower extremity
weakness.
Myelopathy
Myelopathy is the clinical presentation of pathology affecting spinal cord function. The differential diagnosis for
causes of myelopathy is large and includes trauma, metabolic, degenerative, inflammatory, toxic, infectious, and
neoplastic etiologies. Degenerative conditions of the spine may produce the symptoms of myelopathy. In many
instances the onset of the myelopathy is insidious, and symptoms and signs subtle. Longstanding myelopathy,
unfortunately, is rarely reversible. Early identification of patients with progression of myelopathy is essential to
prevent permanent loss of neurologic function. Therefore, in patients who present with neck or thoracic spine pain,
the history and physical exam should be tailored to exclude myelopathy.
The most general signs of myelopathy are those of upper motor neuron dysfunction. Subtle symptoms include
difficulty with fine motor control of the hands and fingers, gait problems and instability, and numbness.
Hyperreflexia, increased tone, and weakness are the hallmarks of the clinical exam. Abnormal plantar response
and Hoffmans sign are frequent abnormal reflexes seen in patients with myelopathy. Urinary dysfunction, such has
hesitancy, frequency, and incontinence, is also seen, but tends not to be severe.
aa. Chronic
Progression of the myelopathy can be very slow and gradual, or stepwise.
It is not uncommon for the onset to be so insidious that patients are quite
disabled before they seek medical evaluation for their symptoms. Careful
history and examination can direct the level of suspicion. In general,
symptoms that affect the hands and upper extremities should prompt
cervical evaluation, while isolated lower extremity symptoms and a trunk
sensory level are more suspicious for a thoracic lesion. Often in chronic
myelopathy the distinction can be difficult. In patients with chronic
myelopathy an MRI is the study of choice to evaluate the spinal canal. In
patients who are unable to have an MRI, myelography and CT
myelography is adequate and gives good information about the spinal
canal.
bb. Acute
Degenerative disease may lead to acute onset of myelopathy. Acute disc
herniation can be seen in the setting of trauma, but also without
significant injury. Patients who present with the acute onset of
myelopathic symptoms deserve urgent evaluation with MRI or
myelography. If pathology such as an acutely ruptured disc causing spinal
cord compression is found, surgical evaluation should be sought.
Unfortunately, patients with complete spinal cord injuries only infrequently
make full recoveries.
Acute worsening of cervical myelopathy in the setting of cervical stenosis
can be seen in the face of fairly minor trauma. In patients with acute
myelopathy without obvious fracture, but significant degenerative disease,
cervical stenosis should be suspected.

b.

Specific Conditions
i.
The Herniated Disc
The central portion of the intervertebral disc is the nucleus pulposus. Under certain pathologic conditions it may
rupture through the annulus fibrosis and into spaces occupied by neurologic structures. Central, large disc rupture
causing compression of the spinal cord or cauda equina may be seen. It is, however, much more common to find a

48

posterior-lateral rupture producing nerve root compression and radiculopathy.

Mechanisms producing radicular pain are poorly understood. Direct compressive effects certainly play a role. The
dorsal root ganglion appears especially sensitive to compressive effects. Recent animal models have suggested a
role for biochemical factors leading to inflammation. Increasingly, experimental evidence suggests that the
mechanisms leading to pain generation are more complex than once thought.
aa. Lumbar
Lumbar radiculopathy is commonly known as sciatica. The classic
presentation is in younger patients who will present with a history of back
pain followed in a few days to weeks by intense leg discomfort,
paresthesias, and radicular weakness as described in the previous
section.
Soft disc herniations in the lumbar spine leading to radicular complaints
are seen most often in the 3rd through 6th decade of life. Estimates of
prevalence vary widely in the literature, from as low as 2% to high as
40%.
Non-surgical therapies for symptoms due to lumbar disc herniation are
plentiful. The natural history of radiculopathy is one of improvement in
many individuals. A variety of therapies are successful in helping patients
get through very painful periods. Oral or epidural steroids can be quite
successful in managing lumbar radiculopathy, although the results are
often temporary. Physical therapy, chiropractic manipulation, and a host of
other devices and regimens are used and promoted. Definitive evidence
on the superiority of any particular approach to non-surgical therapy is
lacking. All patients without severe neurologic deficit should undergo a
trial of non-surgical therapy. The duration of non-surgical therapy is not
set, and is often driven by the patients ability to continue to tolerate their
symptoms. Frequently 4 weeks, and, preferably 2-3 months of nonsurgical treatment are recommended., It is, however, common for patients
with severe pain or neurologic deficit to be operated upon more quickly.
The lumbar laminectomy for discectomy is one of the most widely
performed spinal procedures. Despite this, indications continue to be
debated. Except for patients with a cauda equina syndrome, nonoperative therapy is always an option. There is prospective data from
Weber that would suggest that patients that undergo discectomy improve
more quickly over the short term. This benefit appears to dissipate by 4
years. With those disclaimers, most spine surgeons would agree that
reasonable operative indications would include 1) large midline disc
herniation with resulting cauda equina syndrome; 2) nerve root
compression with pain and significant motor and sensory deficit; 3) nerve
root compression with or without neurologic deficit and incapacitating pain
that fails to improve with non-surgical measures; 4) recurrence of
incapacitating episodes of LBP and sciatica that prevent the patient from
leading a normal life.
In patients with clinical radiculopathy and concordant imaging findings a
successful surgical outcome can be expected in 80-95% of patients.
Recurrence rates are reported at 2-12%. The incidence of serious
complications is very low (<2%).
bb. Cervical
Cervical radiculopathy from nerve root compression can be caused by a
herniated disc or from foraminal narrowing from osteophytes. The root
compression syndromes produced by these conditions have been
described above. Neck pain associated with degenerative disc disease
and osteophytes will improve in the majority of people without invasive
treatment; although there is certainly a group that will go on to have

49

chronic symptoms.
The natural history of cervical radiculopathy is not as well characterized
as that of cervical myelopathy from degenerative disease. Radiculopathy
will improve with time in many patients. However, it is impossible to define
strict rules on the length of non-surgical therapy to undertake before
surgery should be considered. Cervical radiculitis from a soft disc
herniation may be less likely to improve spontaneously as that due to
osteophytes. Non-surgical therapy can include oral or epidural steroids,
cervical traction, physical therapy, bracing, and many others.
In carefully selected patients with radicular symptoms and evidence of
nerve root compression on their imaging studies, more than 90% can
expect a favorable outcome with careful surgical management. Serious
complications are rare (<1%).

ii.

Spinal Stenosis
Spinal stenosis is the narrowing of the cross-sectional diameter of the spinal canal to such an extent that
neurologic symptoms or signs are produced. The syndromes produced by lumbar and cervical stenosis are quite
distinct and will be discussed separately.
aa. Lumbar
Lumbar stenosis classically produces neurogenic claudication.
Neurogenic claudication is leg pain produced by walking or standing that
is typically relieved by a change of position such as squatting, leaning
over or sitting down. Leg pain can be in a variety of distributions, and
becomes quite debilitating. Patients often report associated paresthesias.
Neurologic examination may be normal at rest, though sensory deficits
and hyporeflexia are sometimes seen. When motor weakness is found it
can be associated with wasting, as stenosis is usually a slowing
progressive disease. Approximately 2/3 of patients with symptomatic
spinal stenosis will present with some variety of the classic picture of
neurogenic claudication.
Acquired spinal stenosis is caused by advanced degenerative disease of
the disc, facets and ligaments. The hypertrophy of the facets and
associated ligaments, such as the ligamentum flavum, combine with
bulging discs to produce both central and lateral narrowing. Most patients
with acquired lumbar stenosis are in their 6th to 7th decade or beyond.
Surgical treatment for lumbar stenosis involves decompressive
laminectomy and may require medial facetectomies for lateral recess
stenosis and foraminal stenosis. More recently, surgeons have been
exploring the role of lumbar fusion in the treatment of spinal stenosis in
the older population. The role of fusion and instrumented fusion in this
setting is yet to be fully determined. The reader is referred to the
suggested readings for more on this topic.
Early improvement after surgery for lumbar spinal stenosis is the rule
(>90%) in patients with a postural component to their pain. However, late
progression of symptoms is not uncommon. A large review by Turner et
al. suggests that good results are maintained in approximately 64% of
patients over time.
bb. Cervical
Cervical spondylotic myelopathy (CSM) is the clinical entity produced by
cervical stenosis. CSM usually progresses slowly, in a stepwise fashion.
This myelopathy can be quite subtle in the early stages, and some

50

patients will have significant disability before seeking appropriate medical

care. The most common presenting complaints include neck pain, gait
difficulties, and hand numbness and clumsiness. Loss of bowel and
bladder control is uncommon early in CSM. Occasionally patients will
present with acute and profound spinal cord injury after mild trauma
(usually a hyperextension injury). More common is a stepwise decline in
spinal cord function.
The typical patient with CSM is older than 50 and male. Men are seen
nearly twice as often as women. Myelopathic findings dominate the
physical examination of patients with CSM. Increased reflexes in both the
upper and lower extremities with lower extremity spasticity are common.
Pathologic reflexes such as Babinski and Hoffman are also often positive.
Lhermittes sign (electric, shock-like pain radiating down the spine on
neck flexion) is classically described, but occurs in a small minority of
patients. Complicating the clinical picture in CSM is the lower motor
neuron findings that can be seen secondary to nerve root compression.
Wasting, fasciculations, and hypoactive reflexes can be seen in the upper
extremities due to nerve root compression.
The differential diagnosis of CSM includes multiple sclerosis,
syringomyelia, spinal cord tumor, subacute combined degeneration, and
normal pressure hydrocephalus. Special care should be taken in patients
with both upper and lower motor neuron signs, as amyotrophic lateral
sclerosis and CSM can be difficult to distinguish.
Surgical decompression of the cervical spinal cord will be recommended
by most neurosurgeons in the setting of any signs of myelopathy and
significant cervical canal stenosis. Deficits acquired by patients with CSM
are rarely completely corrected by surgery, so most surgeons will tend to
offer decompression as early as possible. In patients with significant
cervical stenosis without signs or symptoms of myelopathy operative
indications are less clear. The role of fusion in the treatment of CSM is
debated, and is beyond the scope of this chapter.
Surgical results in the large series available suggest that in 75-90% of
cases the myelopathy can be stabilized or improved. The incidence of
worsening of myelopathy with surgery is low (<1%). Other complications
are approach- related and the reader is referred to the suggested
readings.

References/Suggested Reading:
1.
2.
3.
4.
5.
6.

Benzel EC. Cervical spondylotic myelopathy: Posterior surgical approaches, in Principles of Spinal Surgery, Menezes AH,
Sonntag VK (eds); McGraw-Hill, New York, 1996.
Hitchon PW. Management of lumbar herniated disks, in Principals of Spinal Surgery, Menezes AH, Sonntag VK (eds);
McGraw-Hill, New York, 1996.
Katz JN, Lipson SJ, Larson MG, et al. The outcome of decompressive laminectomy for degenerative lumbar stenosis. J
Bone Joint Surg 73A: 809-16, 1991.
Saunders RL. Corpectomy for cervical spondylotic myelopathy, in Principles of Spinal Surgery, Menezes AH, Sonntag VK
(eds); McGraw-Hill, New York, 1996.
Weber H. Lumbar disc herniation: a controlled, prospective study with ten years of observation. Spine 8: 131, 1983.
Wolcott WP, Malik JM, Shaffrey CI, Shaffrey ME, Jane JA. Differential diagnosis of surgical disorders of the spine, in Spine
Surgery, Benzel EC (ed); Churchill Livingstone, New York, 1999.

D.1. Diagnosis and Management of Peripheral Nerve Injury and Entrapment

51

This review is intended to present a set of general principles which can be applied clinically to both evaluate and treat a broad
spectrum of peripheral nerve problems which include traumatic injuries and associated entrapment neuropathies.
a.

Peripheral nerve injuries

i.

Clinical evaluation
Peripheral nerve injury or disease can cause symptoms of pain, dysesthesias, and either partial or complete loss of
sensory and motor function. A thorough clinical history, physical examination, electrodiagnostic evaluation, and
relevant radiographic studies should be performed to distinguish a peripheral nerve problem from one involving the
spinal cord or brain, bone, or soft tissues. In addition, early neurosurgical consultation should be obtained.
The strength of individual muscles or muscle groups is graded. A sensory exam is performed which includes testing
for light touch, pinprick, two-point discrimination, vibration, and proprioception accordingly. It is helpful to test
sensation in the autonomous zones of a nerve where there is minimal overlap from adjacent nerves. The presence
of Tinel's sign is useful to localize a nerve injury. The Tinel's sign refers to paresthesias elicited by tapping along the
course of a nerve. Progressive distal advancement of a Tinel's sign over time can be useful clinically to follow the
course of regenerating sensory axons. However, the presence of a Tinels sign does not guarantee motor recovery.
Return of sweating in an autonomous zone signifies sympathetic nerve fiber regeneration. Reflex changes are also
sensitive and early indicators of nerve damage.
Both electromyography (EMG) and nerve conduction studies (NCS) are useful to distinguish an upper from lower
motor neuron disorder as well as diagnose a primary muscle disease. For EMG studies, a needle electrode is
placed through the skin into a specific muscle and the activity at rest and electrical response to graded muscle
contractions are determined. The nerve conduction study involves stimulation and recording along the course of
peripheral nerves. This allows one to measure the velocity and amplitude of the propagating nerve action potential.
An understanding of the functional anatomy of the peripheral nervous system can often permit the clinician to
localize nerve injuries and lesions with a high degree of accuracy:
The brachial plexus typically originates from the fifth through eighth cervical spinal nerve roots as well as the first
thoracic root and innervates all of the muscles of the upper extremity. These nerve roots join to form trunks, which
further subdivide into divisions, then cords, then, more distally, individual peripheral nerve branches. Severe trauma
transmitted to the most proximal portions of the brachial plexus may produce a preganglionic injury with avulsion of
spinal nerve roots from the spinal cord. It is important to determine whether avulsion of the ventral or dorsal roots
has occurred, since direct repair of a peripheral nerve whose contributing spinal roots have been disconnected
from the spinal cord will not restore function. A Horner's syndrome, characterized by ptosis, miosis, and anhidrosis
indicates avulsion of the ipsilateral proximal C8 and/or T1 spinal nerve roots. Other physical signs of proximal
nerve root avulsion are elevation of the ipsilateral hemidiaphragm (phrenic nerve), scapular winging (long thoracic
nerve), and weakness of the rhomboid muscles (dorsal scapular nerve). All of these nerves originate proximally
along spinal nerves.

ii.

The lumbo-sacral plexus arises from the first lumbar through the fourth sacral spinal nerves. The femoral and
obturator nerves arise from the anterior divisions of L2-4. The sciatic nerve is the largest nerve in the body and
arises from the L4-S4 spinal nerves. This nerve passes through the sciatic notch and travels down the back of the
leg where it branches into peroneal and tibial nerves usually just above the popliteal fossa.
Imaging of peripheral nerve lesions
Imaging techniques such as X-rays, CT, and, most recently, MRI can be valuable diagnostic tools in evaluating
peripheral nerve lesions. Cervical spine fractures are frequently associated with brachial plexus injuries, as well as
injuries of the proximal spinal nerves and roots. Chest radiographs may show unilateral elevation of the diaphragm
as a signature of phrenic nerve paralysis (C3-5) from injury to the proximal upper cervical spinal nerves and roots.
Mid-humeral fractures are associated with radial nerve injuries while midforearm fractures of the ulna or radius are
associated with median or ulnar nerve injuries, respectively. Hip and proximal femur fractures are associated with
sciatic nerve injuries while more distal femur fractures are associated with peroneal or tibial nerve injuries.
Myelography in conjunction with a CT scan are useful to visualize meningeal diverticula and abnormalities of the
spinal nerve roots, findings of which also indicate a spinal nerve root avulsion injury.
CT is able to delineate soft tissue mass lesions such as tumors. MRI has proven to be much more effective in
resolving the fine anatomical detail of soft tissues. Using conventional and enhanced MRI techniques, it has been

52

iii.

possible to visualize both normal and abnormal peripheral nerve structures. New techniques such as MRI
"neurography" make it possible to image and reconstruct the complex peripheral nerve anatomy as well as pinpoint
regions of pathology. MRI can also be used to image signal changes in denervated muscle.
Grading of peripheral nerve injury
The severity or grade of a peripheral nerve injury is determined by the magnitude and duration of the applied forces
of injury. Seddon defined 3 grades of nerve injury (neurapraxia, axonotmesis, and neurotmesis ) based on the
extent of injury to the three structural components of the peripheral nerve described above.
Neurapraxia, the mildest grade of nerve injury, is characterized by a reduction or complete blockage of
conduction across a segment of nerve. Axonal continuity is maintained and nerve conduction is preserved
both proximal and distal to the lesion but not across the lesion. Neurapraxia can result from direct
mechanical compression, ischemia secondary to vascular compromise, metabolic derangements, or
diseases or toxins causing demyelination of the nerve. Conduction is restored once either the metabolic
derangement is corrected or remyelination occurs. Neurapraxic injuries are usually reversible and a full
recovery can occur within days to weeks.
Axonotmesis represents a more severe grade of nerve injury and is characterized by interruption of the
axons with preservation of the surrounding connective tissue "highway" which can support axonal
regeneration. Distal Wallerian degeneration (axon and myelin degenerate distal to site of injury) of the
axons occurs over a several day period after which direct electrical stimulation of the disconnected distal
nerve stump will not give rise to a nerve conduction or muscle response. Recovery can occur through
axonal regeneration due to the preservation of the connective tissue "highway" which consists of Schwann
cells and their basal lamina. The Schwann cells proliferate and form longitudinal conduits (i.e. the bands
of Bungner) through which axons regenerate. Axonotmetic injuries usually recover over a period of
months. The timing and degree of recovery depends on several factors which include the extent of
retrograde axonal loss, as well as the time to regenerate and reinnervate target muscles and/or sensory
end organs. As a general rule, peripheral nerve fibers regenerate at a rate of approximately 1 mm per day
or 1 inch per month. Therefore, more proximal injuries require longer time intervals for regenerating axons
to reinnervate their targets.
Neurotmesis is the severest grade of peripheral nerve injury. Neurotmetic injuries are characterized by
disruption of the axon, myelin, and connective tissue "highway" components of the nerve. Therefore,
recovery through regeneration cannot occur. This grade of injury encompasses nerve lesions where
external continuity of the nerve is preserved but intraneural fibrosis occurs and blocks axonal
regeneration. Neurotmetic injuries also include nerves whose continuity has been completely interrupted.
Since the necessary "highways" for axonal regeneration are absent, surgery is required to remove any
intervening roadblocks in the form of scar tissue as well as to re-establish continuity of the nerve.
On the basis of clinical symptoms and physical findings alone, it is often difficult to differentiate neurapraxic,
axonotmetic, and neurotmetic grades of nerve injury, especially in the acute setting. Nerve conduction studies, both
sensory and motor, are useful during and after the first week following an injury to distinguish neurapraxic from
axonotmetic and neurotmetic grades.

iv.

Treatment Strategies:
Trauma is the most frequent cause of peripheral nerve lesions. Nerve injuries are caused by traction, compression,
sharp laceration, and missile injury (gun shot wounds). Traction injury is often associated with a fracture or
dislocation.. An understanding of the mechanism of injury is extremely helpful in determining the severity of the
lesion and to guide clinical management.
Traumatic peripheral nerve injuries can be classified into open and closed injuries. Decision making for open
injuries is relatively straightforward. Immediate repair of acute sharp lacerating injuries (i.e. glass or knives) should
be undertaken with the goal of performing a primary end to end suture repair. However, not all transecting injuries
lead themselves to a primary repair. It the ends are ragged or contused, a delayed repair is preferable to
demarcate normal from abnormal neural tissue.
The decision making process in treating closed traumatic peripheral nerve injuries is more complex. The majority of
closed traumatic injuries are due to stretch and/or compressive forces. An associated expanding hematoma
producing a compartment syndrome may require emergent surgery to avoid irreversible nerve injury. Because
nerves are often contained in a neurovascular bundle, there is potential for combined vascular and neural trauma A
delayed onset of a neural deficit due to a traumatic pseudoaneurysm may also require urgent attention. An
angiogram is necessary when damage to vascular structures is suspected clinically. In the majority of closed

53

traumatic injuries, however, nerves are not actually transected. Instead, a "lesion in continuity" representing the
damaged segment of nerve may be produced which results in either a neurapraxic, axonotmetic, neurotmetic, or
combination of these grades of injury.
In the case of compression or stretching, it if often not possible to immediately determine the grade of the injury. A
partial nerve injury associated with muscle denervation usually indicates an axonotmetic grade of injury. Patients
with such injuries should be followed with serial clinical and electrodiagnostic examinations to document recovery
and confirm the diagnosis. These patients do not require immediate surgical intervention. An enlarging hematoma
can convert a partial nerve injury into a complete injury. Complete nerve injuries produce severe muscle
denervation and may represent either an axonotmetic or neurotmetic grade of injury. It is critical to distinguish
between these two grades of injury over time, since the latter requires a surgical repair for recovery to occur.
Patients are therefore followed closely over a several month period looking for clinical and electrodiagnostic
evidence of nerve regeneration and muscle reinnervation.
Muscles should be reinnervated within two years following a traumatic nerve injury if recovery of useful motor
function is to occur. Beyond this point, denervated muscles undergo irreversible atrophy and replacement with fat.
Therefore, it is necessary to time a surgical exploration so that a successful nerve repair results in muscle
reinnervation within two years of the injury. A useful rule of thumb is to follow a patient for 3 to 4 months to allow
any element of neurapraxia to resolve as well as permit axonal regeneration to occur beyond the point of injury. If
there is no clinical or electrodiagnostic evidence of muscle reinnervation, then a surgical exploration using
intraoperative electrophysiological monitoring should be performed.
Another approach in the management of these traumatic peripheral nerve injuries is to operate "early" (i.e. as soon
as medically feasible). The rationale for this approach is the following: 1) less scarring and thereby easier
dissection of peripheral nerve elements; and 2) intraoperative evaluation of anatomical and electrophysiological
continuity. However, it remains controversial whether an earlier surgical repair leads to a better recovery of
peripheral nerve function.

b.

Entrapment neuropathies
Peripheral nerve entrapment describes the mechanical irritation by which a specific peripheral nerve becomes locally injured
in a vulnerable anatomic site. Peripheral nerve entrapments produce focal disturbances of nerve function. Nerve entrapment
may occur at any site as a result of non-specific local lesions including fracture callus, hematomas, and benign or malignant
tumors. There are several anatomical sites where peripheral nerves run in relatively confined spaces and are therefore at
increased risk of compression. The differential diagnosis of entrapment neuropathies includes any disease process that
damages nerves in a focal manner: i.e. degenerative, hereditary, vascular, inflammatory, and metabolic. Predisposing
factors include repetitive activities involving the affected extremity, tenosynovitis, rheumatoid arthritis, acromegaly,
alcoholism, amyloidosis, mucopolysaccharidosis, gout, sarcoid, vitamin B6 deficiency, diabetes, trauma, and conditions
altering fluid balance including pregnancy, oral contraceptives, and hypothyroid myxedema.
i.
Carpal tunnel syndrome
Median nerve compression beneath the flexor retinaculum of the wrist is the most common entrapment
neuropathy. Women>men 2:1
aa. Symptoms

Intermittent numbness and paresthesias along

flexor aspects of thumb, index, and middle fingers, as well as
radial side of 4th finger with or without pain
Pain may radiate to the forearm and upper arm
Symptoms are worse with repetitive use of the
hand.
Pain awakens patients from sleep

bb. Exam

Phalens maneuver (flexion of wrist with elbow

extended for 60 seconds reproducing symptoms
Reverse Phalens maneuver (extension of
wrist for 60 seconds)
Tinels sign (localized pain or paresthesia in

54

the cutaneous distribution of the nerve when it is percussed)

Sensory loss in median nerve distribution
(altered light touch and later two-point discrimination)

Thenar muscle wasting (LOAF muscles:

lumbricals I,II, opponens pollicis, abductor pollicis brevis, flexor
pollicis brevis)
cc. Diagnostic studies

Nerve conduction studies show localized

slowing of nerve conduction velocity or decreased sensory
amplitude in the sensory fibers across the wrist

Signs of muscle denervation of thenar

musculature on EMG (electromyogram) with advanced disease
dd. Differential Diagnosis

C6 radiculopathy

Proximal median nerve compression

Anterior interosseus syndrome

Lateral cord of brachial plexus compression

Raynauds disease / vascular

Generalized peripheral neuropathy

Amyotrophic lateral sclerosis

ee. Treatment

ii.

Nonsurgical
Avoid precipitating activity
Volar wrist splint in neutral position
Short course of nonsteroidals or
prednisone
Local steroid injection into carpal
tunnel
Diuretic if premenstrual
Recommended for patients with mild,
intermittent, or acute symptoms
Surgical- Carpal Tunnel release
Indicated for thenar muscle weakness
or atrophy
Denervation by EMG (axonotmesis)
Failure of nonsurgical management

Ulnar Nerve Entrapment

Ulnar nerve entrapment in the region of the elbow is the second most frequently seen compression neuropathy. As
the ulnar nerve descends down the arm it becomes superficial behind the medial epicondyle at the elbow. At this
point it travels between the heads of flexor carpi ulnaris (cubital tunnel) and finally passes through the ulnar tunnel
(Guyons canal) to enter the hand. The anatomic cubital tunnel is a fibroosseous ring formed by the medial
epicondyle and the proximal part of the ulna. The ulnar nerve is vulnerable to compromise from compression, scar
fixation, or traction, as it winds around the medial epicondyle. Patients subjected to immobilization (e.g. anesthesia,
coma, restrained positions) are at risk for prolonged pressure on the ulnar nerve.
aa. Symptoms

Pain at the elbow

Paresthesias in ulnar side of 4th and 5th digits
(palm and dorsum)
Exacerbated with repetitive flexion

55

bb. Diagnosis

Weakness of pinching, grip, 4th and 5th flexors

Positive Froments sign (Inability to adduct the
thumb against the index finger without flexing the interphalangeal
joint)
Weakness of third palmar interosseous with
abduction of 5th digit (Wartenbergs sign)
Clawing posture of little and ring fingers
(benediction posture)
Point tenderness (Tinels sign) above elbow
(ligament of Struthers), at elbow (trauma), or below elbow
(cubital tunnel), with radiation into the 4th and 5th fingers.

Electrodiagnostics show motor nerve

conduction slowing across the elbow, reduced sensory action
potential, and denervation in ulnar innervated muscles (intrinsic
hand muscles)
cc. Differential Diagnosis

Ulnar neuropathy at Guyons canal in the hand

C8 radiculopathy

Thoracic outlet syndrome (medial cord of

brachial plexus C8-T1)

Raynauds disease

dd. Treatment

Nonsurgical
Avoid repetitive flexion and pressure
on the nerve

Splint elbow in extension

Elbow pad

Surgical

Ulnar nerve decompression and/or

anterior transposition (subcutaneous, intramuscular, or
submuscular) if progressive deficits or objective
weakness
Thoracic outlet syndrome

iii.

The brachial neurovascular bundle goes through the thoracic outlet to enter the arm. Thoracic outlet syndrome is
caused by bony, fascial, and muscular structures that interfere with the neurovascular bundle. A fibrous band within
the scalenius anterior muscle, a cervical rib, or its remnant may result in angulation or compression of the lower
trunk of the brachial plexus or C8/T1 roots and subclavian vessels.
aa. Diagnosis

Paresthesias in forearm and hand commonly

precede the development of pain
Atrophy of intrinsic hand muscles
Pain or paresthesias when arms held
overhead
Sensory loss in territories of ulnar and medial
cutaneous nerves

Adsons maneuver (obliteration of the pulse

with a full breath and head in extension or turned to side)
bb. Treatment

Nonsurgical

Corset to prevent elevation of the

56

arms or hands

iv.

Mild Symptoms may respond to

stretching physiotherapy
Surgical
Exploration for refractory symptoms

Meralgia Paresthetica
Entrapment of the lateral femoral cutaneous nerve is referred to as meralgia paresthetica (meros=thigh;
algo=pain). The lateral femoral cutaneous nerve is a branch of the L2 and L3 nerve roots and is purely sensory. It
exits the pelvis to enter the thigh at the upper lateral end of the inguinal ligament. The most frequent location of
entrapment is medial to its origin on the anterior iliac spine.
aa. Symptoms
Numbness, burning, or tingling of lateral thigh
Positive Tinels at the level of the inguinal

ligament

Worse standing or extending the leg

Better sitting

Associated with obesity and/or pregnancy

bb. Treatment

Nonsurgical
Weight loss
Remove constricting binders, corsets,
tight belts, tight jeans
Surgical
Steroid/local anesthetic test infiltration
around the nerve at the inguinal ligament
Lateral femoral cutaneous nerve
surgical decompression (high recurrence rate) or
proximal transection of nerve

E.1. Diagnosis and Management of Hydrocephalus and Spinal Dysraphism

a.

Hydrocephalus
i.

Definition of acute and chronic hydrocephalus:

ii.

Hydrocephalus is the imbalance between spinal fluid production and absorption leading to a build-up of
cerebrospinal fluid (CSF).
Normal physiology and pathophysiology of hydrocephalus:
Cerebrospinal fluid (CSF) is a liquid that normally surrounds the brain and spinal cord, serving to cushion the
nervous tissue as well as wash away metabolic byproducts. It probably serves other functions, which are not as
well understood. CSF is made within the brain by a specialized tissue known as choroid plexus. This specialized
modified cuboidal epithelium secretes CSF at a constant rate of .37 cc/minute, or roughly 500 ccs per day. The
CSF production rate is energy dependent and constant. The composition of this fluid is similar to an ultrafiltrate of
plasma. Choroid plexus in found within the 4 ventricles of the brain, the two lateral ventricles, the midline third
ventricle and the fourth ventricle of the posterior fossa. The CSF normally circulates through these ventricles by
way of communication pathways. The two lateral ventricles join the third ventricle by way of the Foramena of
Monro. The third ventricle joins the fourth ventricle by way of the Aqueduct of Sylvius, and CSF exits the fourth
ventricle by way of the midline foramen of Magendie and the lateral Foramina of Luschka. CSF then circulates up
over the convexity of the cerebral hemispheres where it is absorbed by another specialized tissue, the arachnoid
granulations. The arachnoid granulations are a single cell layer of cuboidal epithelium which allow CSF to cross

57

iii.

into the venous system in an energy passive process by forcing fluid across the membrane, utilizing the pressure
differential between the intracranial pressure (ICP) and the pressure within the venous sinuses. Normally, the
arachnoid granulations can absorb several times more than that which is produced; however, if the arachnoid
granulations become scarred because of trauma or infection, or if they become obstructed by blood products from
a hemorrhage, then CSF can no longer be absorbed well and begins to build up in the subarachnoid spaces,
putting pressure on the brain. If CSF flow is blocked at this level, this is termed "communicating hydrocephalus". If
the CSF pathways are blocked elsewhere, such as at the level of the aqueduct of Silvius, then this is termed
obstructive hydrocephalus. As the intracranial pressure begins to rise in response to a build up of CSF, the patient
becomes symptomatic.
Symptoms of hydrocephalus
aa. Acute:
If the blockage of CSF flow happens rapidly, such as following a sudden
hemorrhage, the brain has little time to compensate and the intracranial
pressure rises rapidly and to a sufficient degree to cause rapid
deterioration into coma. Patients will typically experience headache,
nausea and vomiting, followed in a period of a few hours by confusion,
agitation and then somnolence. If acute hydrocephalus is not treated
emergently, the patients intracranial pressure will reach the point at which
cerebral perfusion is compromised and the patient will deteriorate from
coma to death.
bb. Chronic:
If there is partial obstruction or partial blockage to CSF absorption such
that CSF pressure builds gradually, then the brain accommodates to this
change at first, and the onset of symptoms is more insidious. This is
typically seen in patients with slow growing tumors. They typically develop
headaches and nausea at night or upon awakening in the morning, with
improvement in their symptoms as they get up and walk around. In infants
with open sutures, chronic hydrocephalus manifests by increased head
growth, which crosses percentiles on the growth curve.

iv.

Signs of hydrocephalus
aa. Acute:
In the young child with an open fontanelle, an infant will develop a bulging
fontanelle and separation of the cranial sutures. Rapid head growth is
noted in premature and newborn infants. These young infants may also
have intermittent episodes of bradycardia and apnea. They may develop
crossing of the eyes in response to stretching of the abducens nerves or
may develop a conjugate downgaze, termed the "setting sun" sign. In the
young infant, irritability and somnolence is a late occurrence.
In older children and adults, the development of papilledema occurs over
hours to days and may not be seen in the acute state. Severe headache,
vomiting and alteration of mental status are followed by somnolence as
cortical perfusion becomes further compromised.
bb. Chronic:
Chronic hydrocephalus beyond infancy often manifests similarly to the
syndrome of Normal Pressure Hydrocephalus in which the patient
presents with an ataxic gait, urinary incontinence, and either dementia or
a decline in short-term memory. Patients may describe intermittent
headaches which are typically worse upon arising first thing in the
morning or which awaken them from sleep at night. They may have
chronic papilledema or optic atrophy with constriction of their visual fields.

v.

Radiographic diagnosis of hydrocephalus

aa. Skull x-ray:

58

Skull radiographs can demonstrate findings of raised intracranial

pressure, are inexpensive, and are readily available in most physicians
offices. Separation of the cranial sutures, demineralization of the sella
turcica, or a J-shaped sella may indicate chronically raised intracranial
pressure.
bb. Ultrasound:
In the newborn infant with an open fontanelle, sonography at the bedside
can demonstrate the ventricular size and large subdural collections. Small
subdurals can be missed. Insonation through the mastoid can image the
posterior fossa and rule out 4th ventricular masses.
cc. Computerized tomography:
This is the imaging modality of choice for screening for hydrocephalus. It
is relatively inexpensive and is of sufficient detail to rule out most tumors
which might obstruct the ventricular system.
dd. Magnetic resonance imaging:
MRI gives the highest resolution image of the brain. It has the advantage
of multiplanar imaging, which can be useful in determining subtleties such
as agenesis of the Foramen of Monro or aqueductal stenosis. With
resolution down to 0.5mm, a MRI is unlikely to miss even the smallest of
tumors.
vi.

Differential diagnosis of hydrocephalus

aa. Acute:
The differential diagnosis of acute hydrocephalus is age dependent. In the
premature infant, it will most commonly be secondary to a spontaneous
intraventricular hemorrhage. In the newborn, it may be secondary to a
congenital abnormality of the CSF pathways, may be secondary to
neonatal meningitis, or may be caused by a congenital brain tumor.
bb. Chronic:
Chronic hydrocephalus is caused by a slowly growing brain tumor until
proven otherwise. It may be secondary to a congenital abnormality such
as aqueductal stenosis, or possibly to one of the chronic meningitides, but
CT or MRI must rule out a mass lesion first.

vii.

b.

Treatment of hydrocephalus:

The treatment of hydrocephalus is dependent upon its cause. Acute hydrocephalus secondary to hemorrhage or
infection is often transient and can be managed by temporary CSF drainage, either by serial lumbar punctures
(LPs) or by placement of a temporary ventricular drain until the underlying pathology has been dealt with. Chronic
hydrocephalus has classically been treated by a shunt, which is a plastic tube and valve system which offers a
manmade plumbing system to replace the natural one which is no longer working. This most commonly involves
placement of a tube into the ventricle which exits the skull through a drilled hole (burr hole) and is connected to a
one way pressure regulating valve and then a distal tube which drains excess fluid into another body cavity where it
can be absorbed. Most often, this is the peritoneum.
Spina Bifida
i.
Definition of Spina Bifida:
Spina bifida is a developmental abnormality in which there is incomplete fusion of the dorsal elements composing
the roof of the spinal canal. If this birth defect is skin covered, it is termed "occulta". If the defect is open and the
neural elements are exposed, it is termed "cystica" or "aperta".

59

ii.

iii.

Normal development and abnormal development related to spina bifida:

In the course of normal development, the human embryo at day 18 of gestation is composed of 3 primordial layers
of tissue, the ectoderm, the mesoderm, and the endoderm. Shortly thereafter, the ectoderm begins to develop two
raised areas, one on either side of the primitive streak. These folds of tissue comprise the neural crest tissue,
which curves together and fuses across the midline. This fusion expands in both the rostral and caudal directions to
form the neural tube. The anterior neuropore closes at around day 24 of life and the posterior neuropore around
day 28. Failure of closure of the anterior neuropore will cause anencephaly, whereas failure of closure of the
posterior neuropore is associated with spina bifida cystica.
Signs of spina bifida
aa. Open:
Spina bifida cystica of aperta is being increasingly diagnosed by prenatal
ultrasound. When not diagnosed prenatally, it generally becomes readily
apparent at birth, with the fetus being born with a large head and a
myelomeningocele. This condition is usually associated with additional
abnormalities such as pes cavus deformity of the feet and neurogenic
bowel and bladder.
bb. Closed:
Spina bifida occulta can be a variant of normal, with 5% of the population
demonstrating incomplete fusion of the neural arches on spine x-ray. Most
of the time, this is not associated with neural abnormalities. At times, the
incomplete arch is accompanied by other midline lumbar ectodermal
abnormalities. These include an abnormal pit in the skin, representing a
rudimentary sinus tract, an abnormal lipomatous collection, a tuft of hair,
or an area of cutis aplasia (abnormal skin similar to a birth mark). When
found on screening physical exam, this should alert the physician to the
possibility of an underlying dysraphism. The filum terminale is the terminal
extension of the pia of the spinal cord. It forms a small linear structure,
which normally connects the end of the conus medullaris to the dura at
the end of the thecal sac. In the fetus, the spinal cord extends to the end
of the sacral spinal canal, but over time, there exists a differential rate of
growth between the vertebrae and the neural elements such that the end
of the spinal cord migrates rostrally within the spinal canal. At birth, the
end of the conus in normally around L3, and by six months of age, it is
normally between T12 and L2. In cases of abnormal development, a
thickened filum terminale, a spinal lipoma, or the bony spicule associated
with diastematomyelia may serve to tether the spinal cord and prevent the
normal rostral migration. This tethering will lead to progressive
dysfunction of the distal spinal cord, that which controls bowel and
bladder function, sexual function, and distal lower extremity function.

iv.

Symptoms of spina bifida

aa. Open:
Spina bifida aperta is generally too obvious to be symptomatic. The infant
has deformity of the lower extremities, an enlarged head circumference,
and a neural tube defect, which is obvious. In some instance, these
infants may develop symptoms of hindbrain compression secondary to a
Chiari malformation. This malformation is commonly found in patients with
spina bifida aperta and results in their brainstem structures being in their
cervical spinal canal. Chiari symptoms at this age may consist of drooling,
feeding difficulties, a hoarse or high pitched cry, vocal cord paralysis or
other signs of lower cranial nerve dysfunction.
bb. Closed:
The symptomatic spina bifida occulta typically becomes symptomatic
beyond infancy, typically following a growth spurt. Classically, the young
child who has become toilet trained begins to experience urinary

60

incontinence and urgency. This is often accompanied by back pain

exacerbated by exercise, similar to the syndrome of lumbar
pseudoclaudication seen in elderly adults. Numbness of the legs,
dysesthesias in the lower extremities and motor symptoms can also be
seen. Most of these children will have a history of chronic constipation.
v.

Radiographic diagnosis of spina bifida

aa. Plain radiographs:
Plain radiographs of the spine are generally diagnostic. Occasionally,
more subtle abnormalities will be found such as the midline upper lumbar
calcification of diastematomyelia or widening of the pedicles from a
chronic intraspinal mass such as a spinal arachnoid cyst.
bb. Ultrasound:
In the newborn period, the dorsal arches of the spine are cartilaginous
and have yet to ossify. In the first few weeks of life, ultrasound over the
distal spine can adequately image the spinal canal and cord to determine
the level of the conus medullaris, detect intraspinal lipomas and fluid
collections such as syringomyelia. At this age, a thickened filum terminale
can also be detected.
cc. CT and CT myelography:
The CT scan is an excellent mode of imaging abnormal bone anatomy
and is important in defining abnormal segmentation defects such as
butterfly vertebrae or diastematomyelia. In the MRI era, myelography is
only rarely performed in the child.
dd. MRI:
This is the imaging modality of choice for defining abnormalities of the
neural elements associated with spina bifida occulta or tethering of the
spinal cord. In the newborn period MRI may be difficult due to respiratory
and cardiac motion artifact. If the infant is clinically stable, most pediatric
neurosurgeons prefer to wait until the infant is 3-6 months old to perform
this study.

vi.

Treatment:
In the infant born with a myelomeningocele, repair is usually performed within the first 48 hours of life. The early
repair of a leaking myelomeningocele is believed to prevent the development of meningitis. Ninety percent of these
infants will also have hydrocephalus and will require placement of a ventriculo-peritoneal (VP) shunt. The repaired
myelomeningocele always scars into the walls of the spinal canal at the site of repair; therefore, all of these infants
have, by definition, a tethered spinal cord. Over time, at least a fourth of children with spina bifida will become
symptomatic from this tethering and will require further surgeries to untether the spinal cord.
In the asymptomatic infant noted to have spina bifida occulta detected by a midline lumbar cutaneous signature
mark, treatment continues to be controversial. Most pediatric neurosurgeons will untether the asymptomatic infant
due to reports of progressive neural dysfunction noted in many infants, which are followed over time without
treatment. Given that all neonates are incontinent, it is difficult to assess bowel and bladder function at this age,
and once lost, surgical intervention cannot reliably restore function, but can only halt the deterioration; therefore,
adequate evidence exists to support prophylactic untethering.

E.2. Diagnosis and Management of Surgically Treatable Pain Problems, Movement Disorders, and Epilepsy

61

a.

Recognizing the Features of Trigeminal and Glossopharyngeal Neuralgia, Causalgia and Cancer Pain, Indications
for Surgical Referral and Spectrum of Surgical Therapeutic Options.
i.

Trigeminal Neuralgia:
Trigeminal neuralgia, also known as tic douloureux, is a sudden lancinating pain usually lasting for a few seconds
that is confined to the distribution of one or more of the branches of the trigeminal nerve on one side of the face.
This pain is often triggered by sensory stimulus. There is no neurologic deficit associated with this pain. The patient
will oftentimes have a trigger point or position somewhere within the trigeminal nerve distribution that stimulates
this pain. Facial pain that does not fit into this classification is usually referred to as atypical facial pain. A condition
known as status trigeminus exists when the trigeminal neuralgia-like pain occurs as tic-like spasms in rapid
succession.
The disease is not uncommon, with an incidence of approximately 4 per 100,000 people. Occasionally, there is an
association with MS, with 18% of patients with bilateral trigeminal neuralgia having MS and 2% of patients with
multiple sclerosis showing evidence of trigeminal neuralgia.
The exact pathophysiologic cause of trigeminal neuralgia is not known, but it is probably likely due to irritation or
compression of the trigeminal nerve, particularly its large diameter myelinated a type fibers. This causes an
abnormal transmission of sensory stimuli through the poorly myelinated a delta and c type pain fibers. Common
causes include vascular compression of the trigeminal nerve at the root entry zone, posterior fossa tumor with
compression of the nerve, or a multiple sclerosis plaque within the brain stem. Nearly half of autopsy specimens
will show evidence of compression of the trigeminal nerve root entry zone in patients who have never had
symptoms of trigeminal neuralgia, whereas nearly all patients with trigeminal neuralgia will show this type of
compression. Most commonly the superior cerebellar artery is the culprit causing compression but other posterior
fossa vessels may also cause compression. Patients with tumors can also show compression either of the nerve or
the entry zone.
In order to definitively recognize trigeminal neuralgia, one rule out other causes of facial pain. Five other major
causes must be considered in the differential diagnosis of trigeminal neuralgia including dental disease, disease in
the orbit, temporal arteritis, tumor, and herpes zoster. In particular, herpes zoster pain tends to be continuous,
rather than sudden and lancinating, and is accompanied by other signs. These include characteristic vesicles and
crusting that usually follow the pain and pain noted in the distribution of the first division of the trigeminal nerve
(V1). To delineate these causes, one must perform a detailed history and physical examination. This includes an
accurate description of the distribution of the pain as well as the quality of the pain. The division or divisions of the
trigeminal nerve that are involved should be characterized. Trigeminal neuralgia pain is characteristically
paroxysmal (sudden) pain interrupted by pain free intervals. If pain is always present, then it is unlikely for the pain
to be trigeminal neuralgia. If the patient has been tried on medications, a full description of the dosages that have
been used, the duration of their use, and any side effects must be noted.
On physical exam the patient should have a normal neurologic exam unless the patient has undergone surgery in
the past which may have caused some neurologic deficit. If a neurologic deficit is present then one should look
instead
For a brain tumor or other brain lesion as a cause of the abnormality. A simple series of tests to look at trigeminal
nerve function include testing the corneal reflex, testing facial sensation in all three divisions of the trigeminal
nerve, and testing the patients bite (which assesses the motor function through the motor branch of the trigeminal
nerve). To rule out orbital disease, one should assess extraocular movement function.
It is important to differentiate true trigeminal neuralgia pain and pain that is induced by a tumor, which is usually
characterized by atypical facial pain. One of the atypical features of tumor-related pain is that the pain is usually
constant and neurologic abnormalities are often present, usually of sensory type. True trigeminal neuralgia patients
are typically older individuals with whereas tumor patients can be of significantly younger age.
Trigeminal neuralgia is usually treated by medications. In fact, the most common medication, Carbamazepine
(Tegretol), will give acceptable relief in 69% of patients. Patients on this medication should be checked for a relative
leukopenia. Baclofen and gabapentin can be used if these medications are not effective. Other medications can be
tried including phenytoin and clonazepam but if these medications show poor benefit for the patient surgical
therapy is considered.
Surgical referral is recommended for patients that are refractory to medical management or when the side effects
of the medications exceed the risks and the drawbacks that would be noted for surgery.

62

Surgical methods for the treatment of trigeminal pain are numerous and deal with different portions of the trigeminal
nerve.
aa. Extracranial Procedures

One can attempt to block the transmission of

the pain signal using a percutaneous trigeminal rhizotomy. The
object of this procedure is to attempt a selective destruction of
the a delta and c fibers which mediate nociception and to
preserve the a alpha and beta fibers which mediate touch and
other sensory parameters. The techniques most widely utilized
include radiofrequency thermocoagulation, glycerol injection into
Meckels cave, and percutaneous microcompression via inflation
of a Fogarty catheter balloon.

Rarely, peripheral nerve ablation or

neurectomymay be performed. These procedures are usually
reserved for elderly patients whose remaining life span may not
exceed the time to recurrence of the pain. This recurrence of
pain is secondary to nerve regeneration and occurs within 18-36
months, but it may be followed by a repeat of that procedure if
necessary.
bb. Intracranial Procedures

One common procedure is the microvascular

decompression (MVD), in which a microsurgical exploration of
the root entry zone is performed via a craniectomy of the
posterior fossa bone. The vessel that is impinging on the nerve is
displaced using a nonabsorbable insulator such as Teflon felt or
Ivalon sponge to absorb the pulsations of the vessel impinging
on the nerve.

A newer, less invasive means of treatment is

stereotactic radiosurgery. In this treatment, a small collimator
size (4 mm) is used to place a 70-90 Gy lesioning dose on the
portion of the trigeminal nerve as it enters the brain stem in the
root entry zone. All efforts are made to keep the 80% isodose
curve outside of the brain stem.

Infrequently, an extradural subtemporal

approach to section the trigeminal nerve or an open intradural
exposure of the nerve followed by sectioning may be performed.
Glossopharyngeal Neuralgia

ii.

Glossopharyngeal Neuralgia is a much less common form of pain compared to trigeminal neuralgia but is also a
severe lancinating type of pain. The pain is in the distribution of the glossopharyngeal and vagus nerves and
includes pain radiating to the throat and the base of the tongue, occasionally to the ear, as well as to the neck.
Sometimes this pain is associated with coughing or salivation. In rare instances the patient may have evidence of
cardiac arrest, convulsions, syncope, or hypertension. The pain may be triggered by talking, chewing, and
swallowing, all tasks that utilize the IXth and Xth cranial nerve reflex arcs.
One may try to treat initial pain using cocaine over the tonsillar pillars and fossa but usually patients with
glossopharyngeal neuralgia will require surgical treatment. Surgical treatment includes either microvascular
decompression or sectioning of the glossopharyngeal nerve via either an extra or intracranial approach. The
intracranial approach may provide better results. In the intracranial approach, the patient will undergo sectioning of
all of the preganglionic glossopharyngeal nerve fibers as well as the upper one-third or two fibers (whichever is
larger) of the vagus nerve. Occasionally patients may have problems either with their cardiovascular system or with
their swallowing and therefore require monitoring, particularly over the first 24 to 48 hours in order to treat any
vagus nerve complication. Unlike trigeminal neuralgia, medications do not appear to be effective and this particular
pain syndrome seems to require surgical management for long term benefit.

63

iii.

Causalgia
Causalgia is also known as reflex sympathetic dystrophy and, more recently, as complex regional pain syndrome
(CRPS). Patients with complex regional pain syndrome (CRPS) usually show some manifestation of a partial
peripheral nerve injury including autonomic dysfunction, severe burning or gnawing type of pain, and trophic
changes in the involved extremity. Previously the pain had been divided into minor and major causalgia forms, the
minor form usually occurring after nonpenetrating trauma and major causalgia occurring after high velocity missile
injuries in victims of war.
Theories regarding the pathogenesis of complex regional pain syndrome initially invoked ideas of electrical
transmission between sympathetic nerves and afferent pain fibers. More recently it has been postulated that
norephinephrine released at sympathetic terminals, together with hypersensitivity secondary to denervation or
sprouting, induces the complex regional pain syndrome.
Most commonly, patients present with burning pain in a single limb, most prominent in the distal extent of that
extremity. Most patients will show the onset within 24 hours or less of the actual time of injury though it may take
days or weeks to develop. Allodynia is often present and is defined as pain induced by a non-noxious stimulus. In
patients with CRPS, allodynia is a common finding.
On examination, patients may show either vasodilatory findings, with a warm and pink extremity, or vasoconstrictor
findings, with a cold, mottled, blue skin change, along with their other neurologic findings. The patient may show
trophic changes including stiff joints, tapering fingernails, long coarse hair or loss of hair, dry scaly skin, and
alterations in their sweating ranging from anhidrosis to hyperhidrosis.

iv.

There are no good tests at this time that will confirm or exclude the diagnosis of CRPS. In order to assess
improvement one can only assess the subjective impression of improvement by the patient. Medications have been
tried but are largely ineffective. These medications include the use of tricyclic antidepressants as well as pain
medications. One-fourth or less of patients will show satisfactory long lasting relief after a series of sympathetic
blocks. Surgical sympathectomy, usually involving an exposure (if the pain is in the upper extremity) to the upper
portion of the thoracic chain has, in some studies, been shown to significantly relieve pain. Spinal cord stimulation,
which is the most recent treatment being utilized, also seems to have some success in treating causalgia. Its
effectiveness is usually tested preoperatively by a spinal cord stimulator trial. A needle is utilized for percutaneous
placement of a lead electrode over the spinal cord. It is placed in the cervicothoracic area for the upper extremity
symptoms and in the thoracolumbar region (most commonly the lower thoracic area) lower extremity symptoms.
Cancer Pain
Cancer pain may be extremely difficult to treat. Many malignant tumors may cause pain, particularly if the cancer
itself is localized peripherally. Cancer within the CNS, particularly the brain and spinal cord, tends not to show pain
symptomatology except for headache, and can be treated with other means.

b.

Patients with cancer pain are usually referred when opioid (narcotic) medication management has been found to be
ineffective. These patients may undergo a trial of either an IV narcotic pain medication regimen or, more recently, a
morphine pump infusion. The intrathecal morphine pump utilizes a subcutaneously placed pump, usually in the
abdominal region, which slowly and continuously instills the narcotic within the CSF, usually into the lumbar cistern.
This has been found to be quite effective in many types of cancer pain and has become a widely used treatment
option. More aggressive and invasive surgical techniques may also be utilized but have found significantly greater
disfavor with the advent of the intrathecal morphine pump. These other procedures include cordotomy (either open
or percutaneous), dorsal root entry zone lesioning, and commissural myelotomy to treat bilateral pain. One may
also consider deep brain stimulation in the periaqueductal or periventricular gray matter for treatment of cancer
pain. Medial thalamotomy has been tried but is controversial. Stereotactic mesial encephalotomy has even been
utilized for head, neck, facial, and upper extremity pain. All of these more invasive techniques have found much
less favor since the introduction of intrathecal morphine pump treatment.
Recognize movement disorders amenable to surgical intervention, including Parkinsons disease, dystonia,
spasticity and hemifacial spasm, indications for surgical referral and the spectrum of surgical therapeutic options
i.
Parkinsons disease
Parkinsons disease is characterized by a triad of bradykinesia or akinesia, tremor, and cogwheel rigidity. Prior to
the early 1950s the only treatment that showed some benefit was ligation of the anterior choroidal artery. The
results were quite variable, however. It was soon discovered that most patients with favorable outcomes had
lesions that included the globus pallidus. Because of this, techniques for lesioning this site developed and this
lesioning provided benefit. Initially, anterior and dorsal pallidotomy became the accepted procedure. This mainly
improved rigidity but had minimal effect on tremor or bradykinesia. The thalamus then became a lesion target,

64

particularly the ventral lateral thalamus. This site of treatment provided marked improvement in tremor on the
contralateral side. It could not be performed bilaterally because of an unacceptably high incidence of complications
with the bilateral procedure, including dysarthria and gait disturbance, as well as an unacceptable level of memory
disturbance.
When L-dopa was introduced in the late 1960s, surgical treatment for Parkinsons disease diminished significantly.
However, in the early 1990s a resurgence of surgical treatment occurred because it was found that medications
only provided benefit for several years. Tissue transplantation developed as one method of treatment (e.g. with
adrenal medullary tissue or fetal tissue), but showed only modest benefits. Lesioning procedures, including the
posterior ventral lateral pallidotomy, were later re-explored. They showed significant benefit in end-stage patients
and became the accepted form of surgical treatment in the early and mid-1990s. Patients showed some
improvement, particularly in the dyskinesias or abnormal movements induced by their medication, as well as some
improvement in their rigidity and tremor. Much less improvement was seen in bradykinesia, akinesia, or gait
disturbance.
More recently, deep brain stimulation has been utilized as a treatment for Parkinsons disease. Criteria for referral
include Parkinsons disease symptoms that do not improve with maximal medical treatment or if unacceptable side
effects result when control is obtained. Usually patients will do well with medical treatment for the first several years
but require surgical treatment to provide improvement after that period. It is possible that patients should be
referred earlier for surgical treatment because of preliminary evidence that deep brain stimulation performed
bilaterally may actually reduce the severity, progression, and course of the disease.

ii.

Deep brain stimulation for Parkinsons disease is almost always now directed at the subthalamic nucleus. The
procedure may be performed either by staged unilateral procedures or occasionally as a bilateral procedure. The
staged unilateral procedure appears to have lower risk for the patient, particularly in postoperative recovery, but do
require a second operative setting. The bilateral procedure has the advantage of combining the surgical treatments
all in a single day but patients may show some difficulty postoperatively because of the micro-lesioning effect
obtained bilaterally at the time of implantation.
Dystonia

iii.

Treatment for dystonia has mainly evolved from treatment already developed for Parkinsons disease. Dystonia,
however, differs in its clinical presentation from Parkinsons disease. Very rigid muscle contraction or spasm in
multiple muscle groups creats a distorted posture. Many patients with dystonia will note severe pain secondary to
the dystonic muscle contractions. Preliminary evidence suggests that pallidotomy procedures may be of significant
benefit. Additionally, current work is underway exploring deep brain stimulation directed at both the globus pallidus
and the subthalamic nucleus. At present, however, there is no generally accepted surgical treatment for dystonia.
Spasticity
Spasticity is an uninhibited reflex arc between alpha motor neurons and Ia afferents for muscle spindles resulting in
a hypertonic state. Oftentimes patients will show clonus and may show involuntary movements as well. The lack of
inhibition of the reflex arc results from a lesion in the upper motor neuron pathways causing absence of inhibitor
influence on the motor neurons, including both the alpha motor neurons, as well as the gamma motor neurons
(intrafusal fibers). Many types of CNS injury may cause spasticity, but the most common are cerebral injury (e.g.
stroke), spinal cord injury (e.g. trauma to the spinal cord), congenital abnormalities (e.g. cerebral palsy or spinal
dysraphism), and multiple sclerosis.
Spasticity is noted on physical examination by hyperactive muscle stretch reflexes, increased resistance to passive
movement, and, occasionally, simultaneous activation of opposing antagonistic muscle groups. Patients oftentimes
have pain accompanying this rigidity. In addition to causing pain, spasticity may disrupt activities of daily living by
making the patient unable to sit in a wheelchair with or without special modification, or even lay confortably in bed.
Some patients will develop a spastic bladder with spontaneous emptying and low capacity. Ulcers may also
develop because of characteristic postures that develop including scissoring of the legs or hyperflexion of the
thighs. In cases of spasticity following spinal cord injury, the onset of the spasticity may be delayed anywhere from
days to months after the initial injury, particularly in cases where spinal shock or hypotonia and hyporeflexia exist in
the early period. Some level of spasticity may actually be helpful in these patients, usually to provide support when
sitting in the wheelchair and to help in bracing the patient, but in other respects the spasticity is very limiting for the
patient and requires medical and/or surgical treatment.
Spasticity is graded by a score known as the Ashworth score. This is a useful score graded on 1-5 with 1 showing
no increase in tone and 5 showing the effected muscle as being rigid in flexion or extension.
Medical treatment for spasticity includes stretching, to prevent muscle and joint contractures, removal of inciting

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stimuli, and use of various medications. The most common medications utilized include baclofen which activates
gaba-b receptors, diazepam, which activates gaba-a receptors, and dantrolene, which reduces calcium flux through
calcium channels within the sarcoplasmic reticulum. Often, however, these medications have significant
undesirable side effects culminating in the need for surgical treatment.
Patients are referred for surgical treatment when they are noted to be refractory to medical treatment or when the
side effects of the medication become intolerable. Orthopedic procedures, including tendon releases and
myotomies, are sometimes performed, particularly for fixed deformities. Neurosurgical interventions include
nonablative procedures, such as the intrathecal baclofen pump, and ablative procedures, including injections,
rhizotomies, neurolyses, and neurectomies. By far the most common procedures now utilized are the selective
dorsal rhizotomy and the intrathecal baclofen pump.
Selective dorsal rhizotomy utilizes intraoperative EMG and electrophysiologic stimulation to evaluate the reflex arc
involved in the spasticity. This procedure acts by interrupting the afferent component of the pathologic reflex arc.
Particularly in children with cerebral palsy, this operation has been noted to produce improvement, both in
ambulatory and nonambulatory patients. It usually does not provide much improvement in upper extremity, trunk, or
head and neck-related spasticity.

iv.

The intrathecal baclofen pump, on the other hand, provides improvement in all of these respects. The device
consists of an implated pump that delivers an intrathecal infusion of baclofen through a catheter in the lumbar
region. The dosage can be adjusted to maximize improvement in spasticity without reducing tone to such a degree
that the patient is handicapped by hypotonia. Prior to placement of a baclofen pump a baclofen trial is performed in
which incremental test doses of 50, 75, or 100 ugs of intrathecal baclofen are infused via lumbar puncture or a
temporary catheter. If a positive improvement in Ashworth score is noted, the patient then becomes a candidate for
placement of the pump itself. Intrathecal baclofen pump implantation is a minor procedure performed as an
outpatient, but does require continued follow-up for refills of the pump and adjustments of the rate of infusion to
maximize benefits.
Hemifacial spasm
Hemifacial Spasm is a condition in which spasmodic, involuntary, usually intermittent, usually unilateral, and
painless contractions of the facial muscles occur. In rare instances, the contractions may be limited to just one
portion of the facial nerve distribution. Patients may occasionally have excessive lacrimation. Usually the orbicularis
oculi start the contractions, but they usually progress to involve the entire half of the face. The condition usually
increases in frequency until the ability to see out of the affected eye is impaired. It is critical to distinguish this from
facial myokymia, a continuous facial spasm. One must also distinguish hemifacial spasm from blepharospasm, a
bilateral spasmodic closure of the orbicularis oculi muscles. It is interesting to note that hemifacial spasm and
palatal myoclonus are the only involuntary movement disorders that can still persist during sleep. In other types of
movement disorders, the symptoms dissipate with the induction of sleep. Hemifacial spasm shows a predilection
for the left side, usually appears sometime after the teenage years, and is more common in women. Alteration of
cranial nerve VIII function is noted frequently as well.
The pathogenesis of the disease appears to be compression of the facial nerve at the root entry zone by an artery
or other vascular structure. Most commonly this artery is the anterior inferior cerebellar artery but other veins or
arteries may cause compression as well. Typically the vessel that contacts the root entry zone causes other
symptoms as well (e.g. compression of the vestibular nerves causes vertigo, cochlear nerve compression causes
tinnitus or hearing loss). Occasionally, benign tumors, cysts, multiple sclerosis, or bony abnormalities may cause
hemifacial spasm due to irritation of the facial nerve. In hemifacial spasm the facial motor nucleus may be involved
secondarily as the result of the root entry zone compression via a phenomena similar to kindling.
Patients should undergo an MRI scan to rule out a posterior fossa mass such as a tumor or AVM. Occasionally the
offending vessel may be seen on these studies. Diagnostic work-up otherwise will be negative. Medical
management is usually limited to botulinum (Botox) injections. Botulinum toxin injections may be effective in
temporarily treating hemifacial spasm and particularly blepharospasm, but, in general, hemifacial spasm is a
surgically treated disease. Because of this, patients with this disease, with limitation of vision or significant limitation
of their activities of daily living, will present for surgical treatment.
There are a number of options for surgical treatment. Ablative procedures are effective but they will leave the
patient with facial paresis or paralysis. The current procedure of choice is microvascular decompression (MVD), in
which the offending vessel is physically moved off of the nerve and cushioning material similar to that utilized in the
treatment of trigeminal neuralgia is interposed. Though it is common for a patient to show mild hemifacial spasm
immediately after decompression, the symptoms usually have significantly improved by 2-3 days following the
procedure. Facial weakness, remarkably, is a rare postoperative complication but hearing decrease may occur in

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c.

as many as 10-33% of patients. Some patients will not resolve the hemifacial spasm even after microvascular
decompression. Approximately 10% of the patients will show recurrence of symptoms.
Understanding the general classification of seizure disorders, definition of intractable epilepsy, and the broad
categories of surgical intervention for epilepsy including invasive electrodes, resective and disconnective surgery.
i.
Classification of seizure disorders:
Seizures are defined as sudden cerebral neuronal discharges that result in alterations of motor function, behavior,
or consciousness. This discussion, of course, will be limited and cannot cover all aspects of seizure diagnosis and
treatment, but will give brief descriptions of each of the major types.
Seizures are usually divided into either generalized or partial seizures. Generalized seizures involve bilateral
symmetric synchronous activation of both cerebral hemispheres with evidence of a non-focal onset and loss of
consciousness at the very start of the seizure. Generalized seizures account for approximately 40% of seizures.
There are six major types of generalized seizures:
Clonic seizures, characterized by symmetric bilateral synchronous semirhythmic jerking of the extremities
Tonic seizures, which include sudden sustained increase in tone often accompanied by a characteristic
cry or grunt as air is forced through the adducted vocal cords
Generalized tonic clonic seizures, which usually involve a generalized seizure that evolves from tonic to
clonic motor activity
Absence seizures (previously called petit-mal seizures), in which there is impaired consciousness but only
mild or no motor movement
Myoclonic seizures, characterized by shock-like body jerks
Atonic seizures (sometimes called drop attacks), which involve sudden brief loss of tone that may cause
falls.

Partial seizures, on the other hand, involve only one hemisphere or one focal center in the brain at the onset. When
partial seizures exist, one should conduct a thorough evaluation for a potential structural lesion. Surgery is most
useful in the treatment of this type of seizure. Partial seizures are divided into simple partial, complex partial, and
partial seizures with secondary generalization.

Simple partial seizures have no impairment of consciousness and may involve either motor signs,
autonomic signs, or sensory symptoms
Complex partial seizures involve an alteration of consciousness, usually with a loss of consciousness and
automatisms (e.g. chewing, picking with the fingers, or lipsmacking) and, oftentimes, an autonomic aura,
e.g. gastric discomfort.
Partial seizures may generalize, with simple partial or complex partial seizures evolving to generalization,
or, in some instances, with a simple partial seizure evolving to a complex partial seizure that then evolves
to a generalized seizure.

Some patients may have seizures due to a known etiology, also sometimes known as symptomatic or secondary
seizures. Etiologies include stroke, tumor, and mesial temporal sclerosis. Other seizures, sometimes referred to as
primary seizures or idiopathic seizures, may occur without an underlying cause, e.g. juvenile myoclonic epilepsy.
ii.

Intractable epilepsy
Twenty percent of patients with epilepsy will continue to have seizures even with antiepileptic medication. Surgery
becomes a consideration for these patients under the classification of medically refractory seizures. Usually the
seizure disorder must be severe, with the seizures being medically refractory with several trials of tolerable
medication for at least one year, with significant disability to the patient. In functional terms, the patient has usually
been tried on two different monotherapy antiepileptic drugs at high doses and has had one attempt at polytherapy.

iii.

Surgical intervention for epilepsy:

aa. Invasive electrodes:
Invasive techniques are sometimes required to definitely identify a seizure

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focus. These techniques include subdural strip electrode placement as

well as depth electrode placement. Subdural strip electrode placement
involves creation of small burr holes through which electrodes are then
passed in a subdural location over the brain surface to determine, in an
invasive fashion, and with more precision, the actual seizure focus. Depth
electrodes may be placed, usually stereotactically, to localize the seizure
focus as well. In cases where the general region of a seizure focus is
known but the exact location is not, subdural grid electrodes may be
placed via craniotomy. These grids also allow mapping to be performed,
either in surgery, or in an epilepsy unit, in order to evaluate motor
function, language function, and other vital centers that may be near the
epileptogenic focus.
bb. Resective surgery:
By far the most common epilepsy operation is temporal lobectomy with
resection of the amygdala and hippocampus. Usually, if surgery is
performed on the dominant hemisphere, the temporal lobe is removed
from the temporal tip to a point approximately 4-5 cm posteriorly. If
resection is proceeded beyond this distance, speech centers may be
involved. Nondominant temporal lobe resections can go further back,
usually 6-7 cm. Intraoperative electrocorticography is used in some
centers to guide the resection. Patients undergoing such a temporal
lobectomy in an epilepsy center can usually expect a 60-70% incidence of
seizure control (defined as the absence of seizure activity) with or without
medications. Other patients may experince a reduction in seizure
frequency, even if not rendered totally seizure-free.
cc. Disconnective surgery:
Disconnection surgeries are usually utilized when eloquent brain is
involved, or to separate the electrical activity of the two cerebral
hemispheres. Disconnective surgery includes corpus callosotomy
(particularly useful when drop attacks are the most disabling seizure
type), hemispherectomy (utilized for unilateral seizures with wide spread
hemispheric lesions and profound contralateral neurologic deficit), and
multiple subpial transsections (MST). In MST, the cortex is transected at 5
mm intervals, interrupting the horizontal spread of seizures, while sparing
the vertically oriented functional fibers. Most recently, the vagus nerve
stimulator has been used to interrupt the spread of seizure activity. This
device consists of a pulse generator coupled to an electrode system
around the left vagus nerve. The exact method of inhibition of seizures is
unclear, but it has been noted to have significant benefit in both focal
seizures as well as generalized seizures.

References:
1.
2.
3.
4.
5.
6.

Ashworth B: Preliminary Trial of Carisoprodal in Multiple Sclerosis. Practitioner 192:540-542, 1964.

Lunsford LD, Apfelbaum RI: Choice of Surgical Therapeutic Modalities for Treatment of Trigeminal Neuralgia. Clin
Neurosurg 32:319-333, 1985.
Engel JJ: Surgery for Seizures. N Engl J Med 334:647-652, 1996.
Laitinen LV, Bergenhein AT, Hariz NI: Leksells Posterior Ventral Pallidotomy and the Treatment of Parkinsons Disease. J
Neurosurg 76:53-61, 1992.
National Institutes of Health Consensus Development Conference: Surgery for Epilepsy. JAMA 264:729-733, 1990.
Ocoa JL, Verdugo RJ: Reflex Sympathetic Dystrophy: A Common Clinical Avenue for Somatoform Expression. Neurol Clin
13:351-363, 1995.

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