Neuronotes Clinical Pocket Guide

Electrophysiologic Studies
Auditory-Evoked Potential (AER) uses sounds in the ear to determine the

response of the brainstem and auditory regions of the cortex to the
sound. AER is useful in diagnosing abnormalities of hearing, damage to
the acoustic nerve and acoustic neuromas.
Brainstem Auditory-Evoked Response (BAER) see Auditory-evoked
potential.
Electroencephalography (EEG) utilizes electrodes on the scalp to
measure the electrical activity of the brain. It is used to analyze general
brain function and for the diagnosis of different forms of epilepsy and
seizures and metabolic and degenerative disorders of the brain as well
as sleep disorders. EEG is age specific; in the normal aging process, the
person may demonstrate a modest degree of slowing in the temporal
regions.
Electronystagmography (ENG) is used evaluate some brain functions
and to assess the vestibular system including involuntary eye movement,
dizziness and balance disorders.
Electromyography (EMG) records the electrical discharges of the
muscle in response to nerve stimulation. EMGs can be performed with
surface electrodes measuring voltage on the skin or needle electrodes
inserted into the skin. EMG is used for testing muscle and nerve disor-
ders including amyotrophic lateral sclerosis, myasthenia gravis, the
muscular dystrophies, peripheral neuropathies, and Guillain Barr
syndrome. It is useful in differentiate between demyelinating and
axonal pathology.
Evoked Potentials examine the visual, auditory, and somatosensory sys-
tems to identify sub-clinical lesions. Evoked potentials are essential to
diagnose multiple sclerosis, stroke, visual acuity in children, optic neu-
ropathy, demyelinating diseases, leukodystrophies, and lipidoses.
Nerve Conduction Velocity (NCV) measures the speed of electrical
impulse conduction along sensory and motor nerves that are superficial
enough to be stimulated. NCV is used in nerve disorders such as carpal
tunnel syndrome, diabetic neuropathy, peripheral nerve lesions, and
Charcot-Marie-Tooth disease.
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INTRO
Copyright 2009. F.A. Davis Company
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Somatosensory-Evoked Potential (SEP) uses EEG electrodes to record
the response of the brain to a sensory stimulus. It is useful in determining
determine if there is a delay in conduction to the brain or nerve or spinal
cord damage or nerve degeneration from multiple sclerosis and other
degenerating diseases.
Visual-Evoked Potential (VEP) involves the use of EEG electrodes and
visual stimuli, such as flashing lights. VEP is use to determine the brains
response to the stimuli and is useful in diagnosing optic nerve damage.
Genetic Testing
Genetic tests involve molecular, biochemical and/or cytogenetic analysis
to determine if a condition is genetic or inherited. They can involve DNA,
RNA, chromosome, and protein analysis.
Imaging Studies
Angiography utilizes a radio-opaque liquid that is injected into an artery or
vein to detect blockages by aneurysm, occlusion or displacement of blood
vessels by tumors. It is also useful in detecting vascular malformation.
Computed Tomography (CT) is the preferred initial imaging procedure
for patients with stroke, other intracranial abnormalities, brain tumors
and damage from head injury. CT is usually performed first without, and
then with intravenous contrast enhancement (for example, iodine) to
show vascular abnormalities, hematoma, or enhancement of lesions. CT
of the face and sinuses are performed to evaluate patients with suspected
trauma or infection. In CT, an x-ray beam and a detector system move
around the patient in an arc of 360 degrees. The images are then recon-
structed by specialized software.
CT of the Spine
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Computed Tomographic Angiography is less sensitive than conventional
angiography but can provide important information for patients with
acute stroke. CTA circle of Willis
CTA of the Brain
Magnetic Resonance Imaging (MRI) is used to determine intracranial abnor-
malities, such as tumors and multiple sclerosis (because it is capable to
detect small changes in soft tissue). Tissue with more water (high hydrogen
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4
density) shows high MRI signals and the image appears to be white. MRI
angiography is usually performed alone or after intravenous contrast
enhancement, is to evaluate the carotid, vertebral, and cerebral arteries.
MRI of the Brain
Functional MRI (fMRI) is based on the increase of blood flow to specific
brain structures responsible for certain neural/functional activities. fMRI
allows us to examine not only brain structures but also to map brain func-
tions. fMRI is used primarily in research.
Magnetic Resonance Angiography is less sensitive than conventional
angiography. MR angiogram is useful to examine the carotid arteries and
proximal portions of the intracranial circulation to screen for stenosis,
occlusion, or large atheromatous lesions.
MRA of the Neck
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Positron Emission Tomography (PET) is used to evaluate dynamic brain
activities by evaluating positrons emitting by glucose isotopes. These
scans can detect tumors, measure cellular and/or tissue metabolism, and
show blood flow.
*** PET Normal and PET of Patient with Alzheimers
Disease
Source: From National Institute of Aging.
Radiography is used mostly to identify trauma to the skull and the spine
and metastatic diseases; for example: vertebral fracture caused by
metastatic breast cancer or prostate cancer.
Single Photon Emission Computed Tomography (SPECT) uses Gamma
rays to examine brain structures and is often used in fMRI.
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Transverse Slice of Brain of Person with Multiple
Concussions
Source: Accessed August 17, 2007, from, http://www.drrobertkohn.com/BrainSpect/TBI/nk.htm
8/17/07, with permission.
Ultrasonography (US) utilizes high-frequency sound waves to obtain
images and is used with infants to examine intracranial hemorrhage,
hydrocephalus, and other abnormalities. Neurosonography is used to
analyze blood flow in the brain and is useful in diagnosing stroke, brain
tumors and hydrocephalus. Transcranial Doppler ultrasound is used to
assess blood flow in arteries and blood vessels in the neck to determine
the risk of stroke.
Laboratory Based Exams
Cerebrospinal Fluid Examination is useful to determine CNS infection,
neoplastic invasion of the subarachnoid space, multiple sclerosis, menin-
gitis, acute inflammatory demyelinating polyneuropathy (Guillain-Barr
syndrome). CSF exam is performed through lumbar puncture to deter-
mine the CSF pressure and CSF contents (cell counts, biochemical and
immunologic studies, and microbiologic analysis)
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Glossary
Unless otherwise noted (*), definitions are from Tabers Dictionary;
Copyright 2005 by F. A. Davis Company.
Agnosia Inability to recognize or comprehend sights, sounds, words,
or other sensory information.
Auditory agnosia Inability to interpret sounds.
Tactile agnosia Inability to distinguish objects by sense of touch.
Visual agnosia Loss of the ability to visually recognize objects
presented, even though some degree of ability to see is intact.
Allodynia The condition in which an ordinarily painless stimulus, once
perceived, is experienced as being painful.
Amnesia A loss of memory. The term is often applied to episodes during
which patients forget recent events, although they may conduct them-
selves appropriately, and following which no memory of the period
persists. Such episodes often are caused by strokes, seizures, trauma,
senility, alcoholism, or intoxication.
Anterograde amnesia Amnesia for events that occurred after a
precipitating event or medication.
Post-traumatic amnesia (PTA) A state of agitation, confusion, and
memory loss that the patient with traumatic brain injury (TBI)
enters soon after the injury or on awakening from coma. Edema,
hemorrhage, contusions, shearing of axons, and metabolic distur-
bances impair the brains ability to process information accurately,
resulting in unusual behaviors that often are difficult to manage.
Post-traumatic amnesia can last for months but usually resolves
within a few weeks.
Retrograde amnesia Amnesia for events that occurred before the
precipitating trauma.
Anomia Inability to remember names of objects.
Anosognosia The apparent denial or unawareness of ones own neuro-
logical defect.
Aphasia Absence or impairment of the ability to communicate through
speech, writing, or signs because of brain dysfunction. It is considered
complete or total when both sensory and motor areas are involved.
Brocas or executive aphasia Aphasia in which patients know
what they want to say but cannot say it; inability to coordinate the
muscles controlling speech. It may be complete or partial.
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8
Wernickes aphasia An inability to comprehend the spoken or
written word. Visual and auditory pathways are unaffected; however,
patients are unable to differentiate between words or interpret their
meaning.
Global aphasia Total aphasia involving failure of all forms of
communication.
Apraxia Inability to perform purposive movements although there is
no sensory or motor impairment. Inability to use objects properly.
Constructional apraxia Inability to draw or construct two- or
three-dimensional forms or figures and impairment in the ability to
integrate perception into kinesthetic images.
Ideation apraxia Misuse of objects due to inability to perceive
their correct use. SYN: sensory apraxia.
Motor apraxia Inability to perform movements necessary to use
objects properly, although the names and purposes of the objects
are known and understood.
Verbal apraxia The inability to form words or speak, despite the
ability to use oral and facial muscles to make sounds.
Arteriovenous malformation Congenitally abnormal tangle of blood
vessels, which can produce seizures, neurological deficits and
headache.*
Astereognosis Inability to recognize the form of an object or forms
by touch.
Ataxia Defective muscular coordination, especially that manifested
when voluntary muscular movements are attempted.
Atherosclerosis The most common form of arteriosclerosis, marked by
cholesterol-lipid-calcium deposits in the walls of arteries.
Autonomic dysreflexia The state in which an individual with a spinal
cord injury at T7 or above, experiences a life-threatening uninhibited
sympathetic response of the nervous system to a noxious stimulus.
Symptoms include sudden hypertension, bradycardia, sweating,
severe headache, and gooseflesh.
Axonotmesis Nerve injury that damages the nerve tissue without actu-
ally severing the nerve.
Coma A state of unconsciousness from which one cannot be aroused.
Coma is the most severe of the alterations of consciousness. It differs
from sleep in that comatose patients will not awaken with stimulation; it
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differs from lethargy, drowsiness, or stupor (states in which patients are
slow to respond) in that comatose patients are completely unresponsive.
Confabulation A behavioral reaction to memory loss in which the
patient fills in memory gaps with inappropriate words or fabricated
ideas, often in great detail.
Diadochokinesia The ability to make antagonistic movements, such as
pronation and supination of the hands, in quick succession.
Dysesthesia Uncomfortable, abnormal sensations, such as pins and
needles, burning, or crawling feelings that can occur spontaneously or
from external stimuli.*
Dysarthria Impairments or clumsiness in the uttering of words due to
diseases that affect the oral, lingual, or pharyngeal muscles. The
patients speech may be difficult to understand, but there is no
evidence of aphasia.
Dyscalculia An inability to make calculations. It may be found in child-
hood as a learning disability or may result from a stroke.
Dysgraphia A persistent deficit in handwriting, usually the result of
developmental diseases (in children), and of brain injury, dementia, or
stroke (in adults).
Dysphagia Inability to swallow or difficulty in swallowing.
Dyspraxia A disturbance in the programming, control, and execution
of volitional movements. It cannot be explained by absence of com-
prehension, inadequate attention, or lack of cooperation; it is usually
associated with a stroke, head injury, or any condition affecting the
cerebral hemispheres.
Executive functions The cognitive processes involving logic, planning,
analysis, and reasoning. These capacities enable us to solve problems
encountered in daily life that require considerations of goals, contexts,
options, and previous experiences to select an appropriate strategy.
Hemianopia, hemianopsia Blindness in one-half of the visual field.
Intrathecal therapy Injection of medications into the cerebral spinal
fluid via a lumbar puncture.*
Memory The mental registration, retention, and recollection of past
experiences, sensations, or thoughts. This group of functions relies
on the coordinated activities of the association regions of the cerebral
cortex, specific sensory areas of the brain, subcortical centers, the
hypothalamus, the midbrain, and a wide array of neurochemicals and
neurotransmitters. Injury or damage to any of these regions of the
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10
brain (e.g., as a result of intoxication, stroke, atrophy, or infection)
impairs the ability to incorporate new memories or recall and use
prior ones.
Declarative The conscious recollection of learned informationa
memory function that is improved by the association of learning
with highly charged emotional experiences.
Long-term Recall of experiences, or of information gained, in the
distant past.
Procedural The memory capability that permits an individual to
perform activities. This type of memory is usually preserved when
other memory functions are lost.
Short-term, immediate Memory for events or information in the
immediate past. Brain damage that limits ones ability to store new
information may impair immediate memory but have no effect on
memories of the distant past.
Muscle tone The state of slight contraction usually present in muscles
that contributes to posture and coordination; the ability of a muscle to
resist a force for a considerable period without change in length.
Neurapraxia, neuropraxia A temporary impairment in nerve conduc-
tion, typically caused by an injury that does not produce permanent
structural damage to the nerve.
Neurotmesis Nerve injury with complete loss of function of the nerve
even though there is little apparent anatomic damage.
Oscillopsia The visual perception that stationary objects are moving.
This perception is an illusion, and is usually associated with vestibular
dysfunction.
Paresthesia An abnormal or unpleasant sensation that results from
injury to one or more nerves, often described by patients as numb-
ness or as a prickly, stinging, or burning feeling.
Perseveration Abnormal, compulsive, and inappropriate repetition of
words or behaviors, a symptom observed, for example, in patients with
schizophrenia or diseases of the frontal lobes of the brain. The repeti-
tion of rhythmic but meaningless actions, behaviors, or movements.
Phonophobia A morbid fear of sound or noise. A fear of speaking or
hearing ones own voice.
Photophobia Unusual intolerance of light, occurring in measles,
rubella, meningitis, and inflammation of the eyes.
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Reflex sympathetic dystrophy An abnormal response of the nerves
of the face or an extremity, marked by pain, autonomic dysfunction,
vasomotor instability, and tissue swelling. Although the precise
cause of the syndrome is unknown, it often follows trauma, stroke,
neuropathy, or radiculopathy. In about one third of all patients, the
onset is insidious. Affected patients often complain of burning pain
with any movement of an affected body part, excessive sensitivity to
light touch or minor stimulation, temperature changes (heat or cold)
in the affected limb, localized sweating, localized changes of skin
color, or atrophic changes in the skin, nails, or musculature.
Vertigo The sensation of moving around in space (subjective vertigo)
or of having objects move about the person (objective vertigo).
Vertigo is sometimes inaccurately used as a synonym for dizziness,
lightheadedness, or giddiness.
PT EXAM
12
Medical Yellow Flags
Precautions: Yellow Flags indicate a medical precaution that warrants a
referral to an appropriate medical professional.
Bilateral edema
Chronic or persistent cough
Development of pain, sensory loss, weakness, paralysis, or sphincter
or sexual dysfunction (may be due to spinal cord compression)
Fever of unknown etiology
Pain on weight-bearing
Seizure
Shortness of breath at rest or with minimal exertion
Wheezing
Physical Therapy Examination for Patients with
Neuromuscular Disorders
Anthropometric Characteristics
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PT EXAM
24
52
20
in cm
Birth
Birth to 36 months: Boys
Head circumference-for-age and
Weight-for-length percentiles
W
E
I
G
H
T
W
E
I
G
H
T
H
E
A
D
C
I
R
C
U
M
F
E
R
E
N
C
E
H
E
A
D
C
I
R
C
U
M
F
E
R
E
N
C
E
3 6 9 12 15 18
AGE (MONTHS)
21 24 27 30 33 36
cm in
19
18
17
20
97
90
75
50
25
10
3
97
90
75
50
25
10
3
19
18
17
16
15
14
13
12
50
48
46
44
42
52
50
48
46
44
42
22
50
48
46
44
42
40
38
36
34
32
30
28
26
24
22
20
18
16
14
12
21
20
19
18
17
16
15
14
13
12
11
10
9
8
7
6
5
Date Age Weight Length Head Circ. Comment
64
26
50 60 62 58 56 54 52 48 46
18 19 20 21 22 23 24
27 28 29 30 31 32 33 34 35 36 37 38 39 40 41
68 70 72 74 76 78 80 82 84 86 88 90 92 94 96 98100
40
38
36
34
32
30
22
20
18
16
14
12
10
8
6
4
2
lb kg
cm
cm
in
in
lb kg
LENGTH
11
10
9
8
7
6
5
4
3
2
1
NAME
RECORD #
66
Source: Available at: http://www.cdc.gov/nchs/data/nhanes/growthcharts/set1clinical/
cj41c017.pdf. Accessed October 1, 2007.
PT EXAM
14
W
E
I
G
H
T
W
E
I
G
H
T
S
T
A
T
U
R
E
S
T
A
T
U
R
E
in cm
in cm
2 to 20 years: Boys
Stature-for-age and Weight-for-age
percentiles
12
2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20
13 14 15 16 17 18 19 20
AGE (YEARS)
AGE (YEARS)
97 190
160
155
150
145
140
135
130
125
120
115
110
105
100
95
90
85
80
35
30
25
20
15
10
185
180
175
170
165
160
155
150
105
100
95
90
85
80
75
70
65
60
55
50
45
40
35
30
25
20
15
10
Date
*To Calaculate BMI: Weight (kg) Stature (cm) Stature (cm) x 10,000
or Weight (lb) Stature (in) Stature (in) x 703
Mothers Stature Fathers Stature
Age Weight Stature BMI*
lb lb kg kg
NAME
RECORD #
30 30
30
32
34
36
38
40
42
44
46
48
50
52
54
56
58
60
62
3 4 5 6 7 8 9 10 11
40
50
60
70
80
40
50
60
70
80
90
60
62
64
66
68
70
72
74
76
100
110
120
130
140
150
160
170
180
190
200
210
220
230
90
75
50
25
10
3
97
90
75
50
25
10
3
Source: Available at: http://www.cdc.gov/nchs/data/nhanes/growthcharts/set2clinical/
cj41c071.pdf. Accessed October 1, 2007.
Assistive and Adaptive Devices
Wheelchair (WC) Checklist
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PT EXAM
Source: Adapted from OSullivan SB, Schmitz TJ. (2007). Physical Rehabilitation. 5th ed.
Philadelphia: F.A. Davis; 2007, page 1300, Figure 33.16 Foundation components of a prescriptive
wheelchair, with permission.
PT EXAM
16
Fittings/
Category Components Recommendations Findings
Seat Width Provide 1 in. of space to each
side of widest portion of thigh
& hip
Depth Subtract 1 in. from distance
between posterior aspect of
buttock & popliteal fossa (for
adults)
Height Provide 2 in. of space separating
(measure footplates from floor
with cushion Allow patient to keep trunk
in place) erect & rest sound foot flat on
floor to facilitate propulsion
(in hemi-WC)
Cushion Allow pressure relief for ischial
tuberosities
Frame Angle Adjust according to patients
sitting tolerance
Set at 90 for patients with
paraplegia
Tilt backward for patients with
high level tetraplegia
Backrest Height Adjust according to patients
(measure trunk stabilization ability
with cushion Adjust top to below scapulas
in place) inferior angle for patients with
tetraplegia
Adjust lower for patients with
paraplegia
Straps Pelvic belts Install for safety reasons
Install additional trunk strap
for patients with limited trunk
stability
Leg rests Length Have patient keep hips & knees
at 90 with foot in neutral &
adjust until a 2 in. space
separates floor from footplates
Types Install swing-away leg rests to
ease transfer
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PT EXAM
Fittings/
Category Components Recommendations Findings
Heel loops Types Install heel loops & calf straps or
& calf straps pads for patients with severe
or pads spasticity
Armrests Height Have patient hold trunk in
(measure comfortable sitting position with
with cushion arms at sides & elbows at 90 &
in place) adjust accordingly
Types Install detachable or flip-up
armrests to ease transfer
Brakes Types Install brake extensions for
extremity with limited upper
extremity strength or motion
Wheels Alignment Install parallel wheels for standard
or lightweight adult WCs
Install angled wheels for sports WCs
Tires Pressure If WC has pneumatic tires, assess
pressure for leakage
Power WC Control Install & position control mecha-
mechanism nism for easy control & access
PT EXAM
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Muscle Performance (Including Strength, Power,
and Endurance)
Apgar Scores
Sign Score 0 Score 1 Score 2
Heart rate Absent Below 100 per Above 100 per minute
minute
Respiratory effort Absent Weak, irregular, Good, crying
or gasping
Muscle tone Flaccid Some flexion Well flexed or
of arms and active movements of
legs extremities
Reflex/irritability No response Grimace or Good cry
weak cry
Color Blue all over, Body pink, hands Pink all over
or pale and feet blue
Total Score
Scores are taken at 1 minute, 5 minutes and also may be taken at 10 and 20 minutes post birth
Pediatric Disorders
Cerebral Palsy (CP)
Description/Overview
The motor disorders of cerebral palsy are often accompanied by distur-
bances of sensation, cognition, communication, perception, and/or
behavior, and/or a seizure disorder. While considered non-
progressive, the clinical expression of CP can change with maturation of
the brain, growth of the body, and increasing demands for mobility. Early
symptoms include developmental delay, lack of integration of primitive
reflexes, hyperreflexia and abnormal muscle tone.
Spasticity involves velocity-dependent resistance to passive
movement that results in increased muscle tone; movements
occur synergistically
Athetosis involves fluctuating muscle tone, involuntary and uncon-
trolled movements, and postural instability; its onset is usually
preceded by a period of hypotonia in infants
Ataxia is an infrequent finding in CP in which there is diminished
control of coordination and balance
Hypotonia involves a decreased ability to generate sufficient muscle
force for normal movement patterns
Special Tests
Radiograph used for assessment of scoliosis, bony deformities,
subluxed/dislocated joints, especially of the hips
Ultrasound used to detect hemorrhage or hypoxic-ischemic injury in
the neonatal period
Three-dimensional gait analysis used for baseline measure and prior
to introduction of new medication, Botox injection, and surgery.
Magnetic resonance imaging (MRI) used to detect congenital
malformations, intracranial hemorrhages, and periventricular
leukomalacia
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PEDS
20
Tests and Measures
Work, Community, and Leisure
Assessment
Pediatric Evaluation of Disability Index (PEDI)
Functional Independence Measure For Children (WeeFIM)
Cognition
Individuals with CP have a higher incidence of cognitive impairment and
learning disabilities than the non-disabled population. However, many
individuals with CP often have age-appropriate intelligence that may be
masked by difficulties in communication and motor control.
Differential Diagnosis
Genetic, metabolic, muscular, or neuronal tumor disorders that result
in abnormal tone
Rett syndrome, found primarily in girls
Progressive cerebellar degenerative disorders
Lesch-Nyhan syndrome, found in males, features self-mutilation and
hyperuricemia
Infantile spinal muscular atrophy, associated with hypotonia and
hyporeflexia
Tuberous sclerosis
Surgery
Common surgical procedures associated with CP include:
Dorsal rhizotomy to minimize spasticity
Obturator neurectomy to decrease the spastic influence of the hip
abductors
Soft-tissue releases/transfers to decrease the influence of spastic
muscles and improve gait
Major reconstructive femoral and/or pelvic osteotomy for prevention
and correction of hip subluxation and dislocation
Proximal femoral varus-producing osteotomy in combination with
appropriate soft tissue releases for treatment of subluxing hips
Tibial derotation osteotomy
Achilles tendon, hip adductor, and hamstring lengthening
Prognosis
Maintaining independent sitting by the age of 2 years is a good prognos-
tic indicator of an eventual ability to ambulate. Individuals with CP gener-
ally have a life expectancy into adulthood. Issues limiting life span include
severe problems with sucking, swallowing, and feeding. Morbidity and
mortality are higher than for non-CP and are complicated by respiratory
and cardiac difficulties, cerebrovascular accident, and cancer.
Referral to Other Health-Care Providers
Speech-language pathologist
Audiologist for nerve deafness that may occurs in individuals with
athetoid CP
Ophthalmologist for visual problems such as strabismus, decreased
visual acuity secondary to retinopathy of prematurity, and visual field
abnormalities
Neurosurgery for consideration of an intrathecal baclofen pump,
stereotactic basal ganglia surgery to improve rigidity, choreoathetosis,
and tremor
Orthopedist for surgery for muscle and tendon release and transfer,
and correction of bony abnormalities
Nutritionist when feeding problems exist
Mental health workers for issues involved with disability and depression
Resources
Easter Seals www.easterseals.com
United Cerebral Palsy (UCP)/UCP Research & Educational Foundation
www.ucp.org
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PEDS
PEDS
22
Developmental Coordination Disorder
DCD is associated with speech delays, visual-perceptual, visual-spatial and
visual-motor impairments and can interfere with academic achievement and
activities of daily living. It is estimated that 6% of the U.S. children between
the ages of 5 and 11 have DCD.
Tests and Measures
Arousal, Attention, and Cognition
Potential findings
Individuals with DCD generally have age-appropriate intelligence
but poor academic achievement due to impairments that limit their
coordination
Autism
Attention-deficit hyperactivity disorder
Developmental motor delay
Mild cerebral palsy
Pervasive developmental disorder
Prognosis
DCD can continue into adulthood. Individuals can learn strategies to
develop skills they need for ADLs and other tasks. However, they often
continue to lack the abilities of age-matched peers.
Occupational therapist for fine motor and visual-perceptual issues
Speech-language pathologist
Ophthalmologist if visual problems detected or suspected
Mental health workers as DCD can significantly impact social and
emotional well-being and result in low self-esteem, diminished
self-image, and depression
Resources
Can Child Centre for Childhood Disability Research
Epilepsy
In epilepsy, the normal pattern of neuronal activity becomes disturbed,
causing strange sensations, emotions, and behavior or sometimes con-
vulsions, muscle spasms, and loss of consciousness.
23
PEDS
Other Seizure Activity Requiring Immediate Medical
Attention
Symptoms/conditions Management
The first occurrence of a seizure Seek immediate medical attention
Seizure occurring after an injury or Follow safety procedures for
illness seizures (see following)
During pregnancy
With a diagnosis of diabetes
For individuals with a seizure history:
Recovery from the seizure is slower
than usual
Change in frequency and severity
Seizure is preceded by a severe
headache or neurological
symptoms such as numbness,
weakness, speech disturbances, etc.
PEDS
24
Special Tests
Electroencephalogram (EEG) and 24-hour EEG monitoring to deter-
mine seizure pattern and areas in which the seizure originates
Magnetic resonance imaging (MRI) to determine the source of
seizures
Positive emission tomography (PET) used during epilepsy surgery to
determine how areas of the brain metabolize glucose
Single photon emission computed tomography (SPECT) to localize
the area of the brain responsible for seizures
Wada test (also known as Intracarotid Amobarbital Procedure) is
used before epilepsy surgery and determines which side of the brain
controls language and memory function
Surgery
Resective surgery involves removal of the section of the brain respon-
sible for the seizure.
Corpus callosotomy prevents a focal event to cross over and become
a generalized seizure and is most effective for generalized atonic
seizures
Hemispherectomy is used for patients with congenital syndromes
that result in severe uncontrollable symptoms.
Vagus nerve stimulation is used to limit the severity of seizures.
Prognosis
Life expectancy is minimally reduced for individuals with idiopathic
epilepsy. However, symptomatic epilepsy (having an identifiable cause)
has a negative impact on life expectancy which can be shortened by
several years.
Resources
Epilepsy Foundation http://www.epilepsyfoundation.org/
Hydrocephalus
Hydrocephalus is a pathologic accumulation of cerebral spinal fluid (CSF)
resulting from an imbalance between the formation of CSF and its
absorption. Common causes include excessive secretion of CSF; block-
age of CSF flow within the ventricles (non-communicating hydro-
cephalus); obstruction of CSF distal to the fourth ventricle foramina, in
the cisterns or the cerebral subarachnoid space itself (communicating
hydrocephalus); tumor; and traumatic brain injury. Hydrocephalus is a
common occurrence in Chiari and Dandy-Walker malformations and in
meningocele and myelomeningocele.
Special Tests
CT scan allows prenatal diagnosis, treated with intrauterine
shunting.
MRI, including fetal MRI
Ultrasonography
Lumbar puncture
Intracranial pressure monitoring
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Setting Sun Sign Hydrocephalus
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26
Tests and Measures
Communication Ability (Affect, Cognition, Language,
Learning)
Cocktail party syndrome is associated with hydrocephalus. It involves
being verbose and engaging in superficial social language that may lack
depth and not be appropriate to the situation.
Surgery
Hydrocephalus is treated by:
Extracranial shunts including ventriculoperitoneal (most common),
ventriculoatrial, and ventriculopleural types are used to divert the
CSF to a compartment of the body that can absorb the fluid
Surgical correction of CSF obstruction, such as a tumor or cyst,
ventricular bypass via shunt to an extracranial compartment
Prognosis
The prognosis varies and is dependent on many factors including the
timeliness of treatment/surgery and associated disorders that accompany
hydrocephalus, including head injury, infection, and SB. Because of
the likeliness of comorbidities, hydrocephalus is often associated with
physical, perceptual, and cognitive deficits.
Ophthalmologist as hydrocephalus may result in decreased visual
acuity due to pressure on the optic nerve
Speech-language pathologist if there are issues with sucking, feeding
and swallowing
Resources
Hydrocephalus Association www.hydroassoc.org
National Hydrocephalus Foundation http://nhfonline.org
Muscular Dystrophy (MD)
Duchenne MD is the most common form, affecting primarily boys and
results from an absence of dystrophin, whereas Becker MD is caused by
insufficient dystrophin.
Special Tests
Blood tests for the presence of creatine kinase (suggests muscle
disease)
Electromyography
Ultrasonography can detect certain muscle abnormalities
Muscle biopsy to detect markers associated with specific types of MD
and the presence of dystrophin
Genetic testing to detect gene mutations that produce dystrophin
Tests and Measures
Neuromotor Development and Sensory Integration
(Refer to Tab 2)
Potential findings
DMDdifficulty rising to stand from sitting or lying and with stair
climbing; frequent falls
Work, Community, and Leisure (See Tab 2)
Assessment
Pediatric Evaluation of Disability Index
Functional Independence Measure For Children (WeeFIM)
27
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28
Disease-Specific Tests and Measures
Surgery
Achilles tendon lengthening
Spinal stabilization for scoliosis and other spinal deformities
Must distinguish among the types of MD
Kugelberg-Welander spinal muscular atrophy
Werdnig-Hoffmann spinal muscular atrophy
Prognosis
Refer to earlier table for life expectancy for each variation of MD; death is
usually associated with respiratory or cardiac complications
Speech-language pathologist for assessment of language and dysphagia
Occupational therapy for ADLs and environmental adaptations
Mental health workers for issues involved with disability, decreased
function and independence, and depression
Medications
Indications Generic Name Brand Name Common Side Effects
Weight loss; oxandrolone Oxandrin Insomnia, depression,
side effects anxiety
caused by
corticosteroids
Source: Vignos PJ, Spencer, GE, Archibald, KC. Management of progressive muscular dystrophy.
JAMA. 2963;184:103112.
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Resources
Muscular Dystrophy Association mda@mdausa.org
Neonatal Neurological Conditions
Hypoxic Ischemic Encephalopathy (HIE)
Considered the most common cause of severe, non-progressive neuro-
logic defect caused by perinatal events, it can result from respiratory or
cardiac insufficiency. Hypoxic-ischemic events can also be caused by
emboli, thrombosis, or clot formation.
HIE is diagnosed based on umbilical arterial blood pH, the persistence
of an Apgar score of less than 3 at 5 minutes, neurological signs includ-
ing seizures and hypotonia and involvement of several organs, including
but not limited to, the lungs, heart, kidneys, and liver.
Severe HIE is characterized by the need for ventilatory support, flaccid-
ity, and absent reflexes. Respiratory and cardiac functions may be
depressed and can fail.
Low Birth Weight
Infants with low birth weights often develop periventricular leukomalacia,
intraventricular hemorrhage, and respiratory problems, including respiratory
distress syndrome. These complications worsen for those in the VLBW and
ELBW categories and they have a greater chance of developing hypother-
mia, perinatal asphyxia, hyperbilirubinemia, infection, and other medical
complications.
Prematurity
Infants born earlier than 37 weeks gestation are considered premature.
Special Tests
Electroencephalogram for diagnosing neonatal seizures
Somatosensory evoked potentials (SSEPs)
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30
Visual evoked potentials (VEPs)
MRI is the imaging modality of choice for HEI and PVL
Ultrasonography used to diagnosis IVH and PVL
Near-infrared spectroscopy for monitoring CNS ischemic events.
Physical Therapy Examination - Tests and Measures
Reflex Integrity
Reflex Integrity is often accomplished as part of assessment tools listed in
section Neuromotor Development and Sensory Integration.
Assessment
Deep tendon reflexes
Postural responses, including righting, equilibrium, and protective
reactions
Primitive reflexes
Muscle tone
Prognosis
Hypoxic Ischemic Encephalopathy is associated with significantly high
rates of mortality and morbidity. HIE often results in CP, especially when
abnormal muscle tone persists past the first week of life. Ongoing seizures
and cognitive impairment are common neurological sequelae. HIE can
also result in visual impairment, hearing impairment, and chronic lung
disease.
Intraventricular Hemorrhage: Outcome is dependent on the severity of
hemorrhage. IVH grades of I and II are associated with minimal risk of
sustaining long-term disability. However, grades III and IV represent a
worsening prognosis that is more significant when accompanied by other
medical complications. Grades III and IV are associated with a high risk of
developing mental retardation, hydrocephalus, seizures, and CP.
Low Birth Weight: Infants who have a very low birth weight or extremely
low birth weight are at high risk for infant mortality and morbidity.
Neurological consequences include CP, learning disorders, and cognitive
impairment. Other complications related to VLBW and ELBW include sen-
sorineural hearing loss, seizures, and attention-deficit disorder.
Periventricular Leukomalacia represents an increased risk for CP, cogni-
tive impairment, coordination difficulties, and vision and hearing impair-
ments. The prognosis is dependent on the severity of brain damage and
can range from very mild symptoms to significant disability.
Prematurity: The prognosis of premature infants varies and is linked to
accompanying medical complications. While some very premature
infants have no long-term complications others have neurological seque-
lae including CP, learning disabilities, attention-deficit hyperactivity disor-
der, sensorineural hearing loss, feeding difficulties, and retinopathy of
prematurity.
Speech language pathologist for language delays and feeding
difficulties
Early intervention/speech education professional for cognitive delays,
attention deficit/hyperactivity disorder and learning disability.
Ophthalmologist for suspected vision impairments
Audiologist for hearing difficulties
Resources
American Association of Premature Infants www.aapi-online.org
Obstetrical Brachial Plexus Palsy
Injury can result from neurapraxia, neurotmesis, and axonotmesis. It can
also be caused by hemorrhage and edema that compress the nerves in
the plexus, and pressure on the nerve from neuromas that develop
during the healing process.
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Special Tests
EMG and fluoroscopy to determine diaphragmatic function
High-resolution MRI
Radiographs to assess possible fractured clavicle and humerus
Tests and Measures
Assessment
Girth measurements
Limb length
Assistive and Adaptive Devices
Considerations
Assistive and positioning devices, splints, etc., should be appropriate
for childs age; some positioning can be done with stuffed animals,
infant pillows, etc.
Work, Community, and Leisure
Assessment (Refer to Tab 2)
Pediatric Evaluation of Disability Index
Functional Independence Measure For Children (WeeFIM )
Fractures of the clavicle and humerus
Torticollis
Facial nerve palsy
Surgery
In infancy:
Excision of neuromas or hematomas
Neurolysis to remove scar tissue that develops around the injured
nerve
Repair by nerve graft
In older child:
Tendon transfers for improved flexibility, function and cosmesis;
common procedures include transfer of:
Latissimus dorsi and/or teres major to the posterior aspect of the
upper humerus for improved external rotation and abduction
Triceps to biceps
Contralateral trapezium and/or rhomboids for stabilization of the
affected scapula
Soft tissue releases of contractures
Osteotomy for external rotation of the humerus
Prognosis
Prognosis is dependent on the site, nature, and severity of the injury. The
prognosis is best when significant recovery occurs in the first few months
of age. If there is a lack of recovery within the first few weeks, long-term
disability is likely to occur. Prognosis based on the type of injury is:
Avulsion injuries Permanent injury unless surgically repaired
Erbs and Klumpkes Spontaneous recovery in 70% to 80% of
palsy cases
Total plexus palsy Spontaneous recovery rate less than 50%.
Resources
Brachial Plexus Palsy Foundation www.brachialplexuspalsyfoundation.org
United Brachial Plexus Network www.ubpn.org
Rett Syndrome
Precautions
There is an increased risk of osteoporosis associated with RS.
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34
Special Tests
Genetic testingpositive MECP2 mutation gene analysis indicative
of RS
EEG to identify seizure activity
Swallowing studies to determine risk of aspiration
Respiratory studies of disorganized breathing patterns, clinical
cyanosis, sleep apnea
Tests and Measures
Communication Ability (Affect, Cognition, Language,
Learning)
Speech apraxia is present in RS but receptive speech may remain.
Patients may attempt to communicate with body language and
expression. Communication may be possible through the use of eye gaze
systems, picture boards, and voice output systems.
Angelmans syndromebut no loss of hand function
Spinocerebellar degenerationsbut no loss of hand function
Benign congenital hypotonia
Cerebral palsy
Autism
Surgery
Vagal nerve stimulation has been successful in increasing alertness
and social functioning
Gastrostomy is used if there are significant feeding problems or aspi-
ration pneumonia
Surgical correction of scoliosis should be considered when curves are
greater than 40 to 45 degrees
Prognosis
Patients often stabilize by the second decade when seizures may lessen
and an increase in hand function and social interaction may occur. Gait
may improve in some cases. Active PT and OT are important in preventing
contractures and maintaining strength and function. Patients with RS may
live into their fifth or sixth decades.
Speech-language pathologist for assessment of oral motor status and
communication
Psychologist for IQ and autism testing
Dietician as a high calorie diet is associated with improved weight
gain and seizure control
Assistive technology specialist for eye gaze system or voice output
communication
Resources
International Rett Syndrome Association www.rettsyndrome.org
Rett Syndrome Research Foundation www.rsrf.org
Shaken Baby Syndrome
Due to the weakness of an infants neck muscles and the size of the head,
shaking results in multiple forces of the fragile brain against the skull. This
causes direct trauma to the brain resulting in a classic triad of symptoms
that include brain swelling, subdural hematoma, and retinal hemorrhage.
Fractures of the long bones and ribs may also occur.
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36
Special Tests
CT scan is used if SBS suspected and can demonstrate subarachnoid
hemorrhage and diffuse hypoxic-ischemic changes
Magnetic resonance imaging is used to demonstrate changes in the
brain tissue
Radiographs are used for diagnosing skull fractures and other fractures
Surgery
Surgery may be indicated to stop bleeding in the brain or to relieve pres-
sure from bleeding.
SBS syndrome results in serious brain damage that is not caused by
an accidental fall, bouncing, baby swings, etc.
Initially SBS may appear to be related to the flu or meningitis
Prognosis
SBS is the most common cause of mortality from child abuse occurring in
infants. Resultant disability ranges from mild learning disability to pro-
found mental retardation and permanent vegetative state.
Referral
In all states, therapists are mandated reporters, who are required by law
to report suspected child abuse or maltreatment. Every state has a Child
Abuse Hotline. The National Child Abuse Hotline is 1-800-4-A-CHILD.
Resources
Think First Foundation National Injury Prevention Program
http://www.thinkfirst.org
National Center on Shaken Baby Syndrome E-mail: mail@dontshake.org
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Spina Bifida (SB)
Spina bifida occulta: often accompanied by neurocutaneous stigmata
such as a dimpling, small hole, a lipoma or tuft of hair on the back, it
is usually asymptomatic
Myelomeningocele: there may not be a clear delineation of involve-
ment associated with a particular spinal level; orthopedic deformities
are common due to bony malformations, residual paralysis, weak-
ness, and the uneven muscle activity around affected joints
Tethered Cord Syndrome: can result from spina bifida occulta or scar-
ring after surgical repair of SB; complications arise during periods of
growth when blood vessels stretch, resulting in decreased blood flow
to the spinal cord; the cord may stretch abnormally and resultant ten-
sion can cause permanent damage to the nerves; Hydrocephalus
occurs in more than 90% of those with lumbrosacral myelomeningo-
cele. Chiari malformation is a common occurrence in myelomeningo-
cele and involves structural defects in which medulla, pons, fourth
ventricle and lower portion of the cerebellum herniate into the upper
cervical canal. The flow of the CSF is blocked resulting in symptoms
that include dizziness, numbness, visual defects, headache and dimin-
ished balance and coordination. There may be complications with
respiration, swallowing, gagging and aspiration.
Dislocation of the Hips
Symptoms Possible Causes Management
Decreased passive range Muscle imbalance Referral to physician
of motion, typically limiting around the joint
hip adduction and internal Joint contractures
rotation
Leg length discrepancy
Pain
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38
Special Tests
Urinalysis, urine culture, and serum urea nitrogen creatinine test are
assessed at birth to evaluate renal function and for signs and symp-
toms of urinary tract infection
Voiding cystogram is used to assess the lower urinary tract
X-Rays are used for determination of the level of lesion; spinal
deformities, deformities of the foot, ankle, and other joints, and
subluxation/dislocation of hips
EMG and nerve conduction velocities may be done in the LEs of
infants with SB
Myelogram to detect a tethered cord or spinal cord abnormality
Neuro-imaging
CT scans for hydrocephalus
MRI of the brain and spine to determine the level and extent of the
SB and to detect any abnormalities of the spinal cord and column
Tests and Measures
Consideration
Obesity is a common problem in SB
Assessment
Limb length and circumference
Worsening of Neurological Signs
Symptoms Possible Causes Management
A decrease in muscle Tethered cord Referral to physician
function Malfunctioning
Decreasing bowel and shunt
bladder function
Arousal, Attention, and Cognition
Considerations
The majority of individuals with myelomeningocele have intellectual
scores within age-appropriate ranges; lower intellectual abilities
and cognitive impairments are associated with higher-level lesions
(thoracic and cervical), hydrocephalus, and CNS infections
Assessment Refer to Neuromotor Development and Sensory Integration
Bowel and Bladder Control
Potential findings
Anorectal sensation is usually absent
Kidney infections are common because of retrograde flow of urine
and difficulties emptying the bladder
Integumentary Integrity
Orthotics should be modified if their use results in redness that per-
sists for more than 20 minutes
Instruction in patient/family assessment and reporting is important
due to sensory deficits
Muscle Performance
Considerations
It is necessary to determine the motor lesion level as it is a useful
predictor of potential contractures and deformities as functional
abilities that may be attained; lesions may resemble complete cord
transection or may be incomplete, with mixed representation of
muscle use, spasticity, and paralysis in muscles below the lesion
Assessment
MMT should be performed if appropriate considering age and
cognition
Dynamometry can be used for muscle force and grip and pinch
strength
Neuromotor Development and Sensory Integration
Considerations
Testing should be done with and without adaptive/assistive equip-
ment and orthoses
39
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40
Orthotic, Protective, and Supportive Devices
Considerations
Braces/orthosis are often required to prevent contractures/deformities
and aid mobility
Bracing for spinal deformities is difficult because the decreased sen-
sation makes breakdown more likely
Range of Motion
Considerations
Clubfoot and rocker-bottom foot deformities may occur secondary to
the unopposed pull of intrinsic foot muscles or muscles at the ankle
joint and as the result of intrauterine positioning
Ventilation and Respiration
Coughdetermine strength of cough and ability to clear secretions
Language
Language is often impaired in those individuals who have hydrocephalus,
who often demonstrate a cocktail personality. They may be verbose
and use clichs and social conversation, which has little depth.
Surgery
In myelomeningocele, surgical closure and repair is ideally performed
within 12 to 48 hours to minimize the risk of infection and further
damage to the spinal cord; if there is CSF leakage, immediate surgery
is performed
Surgical correction of a Chiari malformation and tethered cord may
be required if neurological signs worsen
Other surgical management includes correction of:
Contractures of hip and knee musculature
Subluxation and dislocation (controversy exists about the need to
relocate hip dislocations in non-ambulatory patients)
Club feet and other ankle and foot deformities including
equinovalgus, pes cavus, calcaneovarus and calcaneovalgus.
Contractures resulting in functional deficits, including tenotomy of
knee flexors
Spinal stabilization is used for correction of kyphosis and scoliosis.
Prognosis
Prognosis is dependent on the level of the lesion and medical complica-
tions. Generally survival rate is lower, the higher the sensory level of
deficit. Adults with lower level lesions can be employed and self suffi-
cient. With aggressive attention to bowel and bladder management
survival well into adulthood can be expected.
Orthopedist for correction of contractures and deformities
Neurologist for changes in neurological signs and symptoms
Psychological or early childhood specialist for assessment of
cognitive status
Speech-language pathologist for feeding issues, language delays
Social work for psychosocial issues
Orthotist for bracing fabrication and adjustment
Resources
Spina Bifida Association of America http://www.sbaa.org
March of Dimes Birth Defects Foundation http://www.marchofdimes.com
41
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CNS-NP
42
Nonprogressive Disorders of the Central Nervous
System
Central Vestibular Dysfunction
Vertigo of Central Origin
Central vestibular dysfunction (CVD) can result from atherosclerosis of the
vertebral and basilar arteries; infarcts to the anterior-inferior cerebellar
artery, posterior-inferior cerebellar or vertebral artery; neoplasms; or
degenerative diseases.
Special Tests
Electronystagmography (ENG)
Caloric testing
Rotational testing
Neuroradiologic imaging for diagnosis of a central cause
Orthostatic hypotension
Cardiac arrhythmia
Psychological dysfunction
Peripheral vestibular disorders (Refer to Tab 6)
Surgery
Surgical incision may be used for any tumors affecting the central
vestibular system.
43
CNS-NP
Prognosis
Prognosis for recovery of CVD varies depending on the underlying cause.
Recovery is typically slower in response to treatment (versus PVD).
Neuro-otologic examination
Neurologist
Resources
Brown KE, Whitney SL, et al. Physical therapy for central vestibular dys-
function. Arch Phys Med Rehabil. 2006; 87(1):7681.
Vestibular Disorders Association http://www.vestibular.org
Cerebrovascular Accident
Cerebrovascular accident (CVA), or stroke, is an interruption of blood flow
to the brain resulting in transient or permanent neurological deficit.
Ischemic stroke results when a thrombus or embolus causes a block-
age of cerebral blood flow
Hemorrhagic stroke, caused by an intracerebral or subarachnoid hem-
orrhage, can result from an aneurysm, arteriovenous malformation,
or traumatic brain injury
Transient ischemic attack occurs from an interruption of arterial blood
flow in the brain that resolves spontaneously without tissue damage
Common syndromes related to disrupted blood flow include:
Ischemic stroke
Ideomotor apraxia (inability to pantomime the use of tools, e.g.. pre-
tending to brush hair)
CNS-NP
44
Right hemisphere infarction
Motor impersistence
Visual field neglect (attentional, representational, and intentional
neglect)
Visuospatial impairment
Contralateral neglect
Behavioral impairment including agitation and impulsiveness
Cognitive impairment including confusion, delusion, and
anosognosia
Basilar artery (lateralized deficits relate to branches of the basilar artery;
ischemia of the entire basilar artery results in bilateral deficits)
Nystagmus
Ipsilateral decrease in facial sensation
Precautions
Seizures often occur within first week after onset of ischemic stroke; later
onset with hemorrhagic stroke.
Stroke is often associated with vascular disease so physician clear-
ance should be obtained before initiating therapy
Symptoms Causes Management
Loss of consciousness,
followed by stiffening
and then jerking of
extremities; May bite
tongue, cheek or lip;
drool; and have bladder
and bowel incontinence
Post-stroke
hypoxia and
impaired blood
flow; metabolic
imbalance
Protect the patient
from injury
Contact a physician
for first occurrence
or change in seizure
pattern
Special Tests
Thyroid function to test for accelerated atherosclerosis secondary to
hypothyroidism
CT scan to diagnose hemorrhage and subdural hematomas
45
CNS-NP
MRI to diagnose ischemic stroke
Echocardiogram including transesophageal echocardiography (TEE)
to assess possible cardiogenic sources of stroke
Carotid duplex scanning to determine the cause of the stroke and
need for carotid endarterectomy
Transcranial and carotid Doppler ultrasonography
Tests and Measures
Assessment Assess
Girth measurements if edema is present
Motor Function
Assessment
Examine components of motor control (Refer to Tab 2)
Describe accuracy (or error) in reaching a target; ability to correct
movement in mid-course
Muscle Performance
Assessment Assess
Any loss of muscle bulk
Substitutions used in movement
Pain
Potential findings
Subthalamic lesions can result in spontaneous pain on the contralat-
eral side that may be triggered by light touch, pressure, or contact
with heat or cold
Posture
Assessmen Assess
Potential findings
Persistent posturing may be observed
Secondary to pusher syndrome, the patient may leans toward hemi-
plegic side and resists upright positioning
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46
Reflex Integrity
Assessment
Resting posture and position of limbs, and resistance to passive
movement
Response to perturbation
Sensory Integrity
Potential findings
Sensory deficits may be contralateral or ipsilateral, depending on
location of lesion
Transient ischemic attack
Encephalitis
Migraine headache
Hypernatremia
Meningitis
Neoplasms of the brain
Surgery
Hemorrhagic stroke usually requires surgical evacuation to decrease
intracranial pressure and/or repair defective blood vessels
Endovascular coil embolization involves inserting stents into the
defective blood vessel to prevent further damage
Carotid endarterectomy is used to remove atherosclerotic deposits in
the carotid artery
Arteriovenous malformations require surgery to repair abnormal
blood vessels
Prognosis
Prognosis varies depending on the type of stroke and duration and extent
of obstruction or hemorrhage. Disability can range from minimal loss of
sensory and motor function to loss of motor and sensory functions,
speech, understanding, and reasoning. Prognosis affected by age, pre-
morbid status and social supports.
47
CNS-NP
Postural Assessment Scale for Stroke (PASS)
Items and Criteria for Scoring
Maintaining Date/Pt
a Posture Score Score
1. Sitting without
support (sitting
on edge of a
50-cm-high
examination
table with
feet touching
the floor)
2. Standing with
support (feet
position free,
no other
constraints)
3. Standing without
support (feet
position free,
no other
constraints)
Cannot sit
Can sit with slight support, for
example, by 1 hand
Can sit for more than 10 sec
without support
Can sit for 5 min without support
Cannot stand, even with support
Can stand with strong support
of 2 people
Can stand with moderate sup-
port of 1 person
Can stand with support of only
1 hand
Cannot stand without support
Can stand without support for
10 sec or leans heavily on
1 leg
Can stand without support for 1
min or stands slightly
asymmetrically
Can stand without support for
more than 1 min and at the
same time perform arm
movements above the
shoulder level
0
1
2
3
0
1
2
3
0
1
2
3
Mental health workers as depression is a common occurrence
Speech language pathologist if language is involved
Orthotist if splints or orthotics needed
CNS-NP
48
Postural Assessment Scale for Stroke (PASS)Contd
Maintaining Date/Pt
4. Standing on
nonparetic leg
(no other
constraints)
5. Standing on
paretic leg
(no other
constraints)
Changing
Posture
6. Supine to
affected
side lateral
7. Supine to
nonaffected
side lateral
Cannot stand on nonparetic leg
Can stand on nonparetic leg for
a few seconds
more than 5 sec
more than 10 sec
Cannot stand on nonparetic leg
Can stand on nonparetic leg
for a few seconds
for more than 5 sec
for more than 10 sec
Scoring of items 612 is as fol-
lows: items 611 are to be per-
formed with a 50-cm-high
examination table; items 1012
are to be performed without any
support; no other constraints
Cannot perform the activity
Can perform the activity with
much help
Can perform the activity
with little help
without help
much help
little help
without help
0
1
2
3
0
1
2
3
0
1
2
3
0
1
2
3
49
CNS-NP
Postural Assessment Scale for Stroke (PASS)Contd
Maintaining Date/Pt
8. Supine to sitting
up on the edge
of the table
9. Sitting on the
edge of the table
to supine
10. Sitting to
standing up
11. Standing up to
sitting down
12. Standing,
picking up
a pencil
from the floor
with much help
with little help
without help
much help
with little help
without help
much help
with little help
without help
much help
little help
without help
with much help
with little help
without help
0
1
2
3
0
1
2
3
0
1
2
3
0
1
2
3
0
1
2
3
Source: Benaim C, et al. Validation of a standardized assessment of postural control in stroke
patients. The Postural Assessment Scale for Stroke Patients. Stroke. 1999;30:18621868,
with permission.
CNS-NP
50
Resources
American Stroke Association www.strokeassociation.org
National Stroke Association www.stroke.org
Traumatic Brain Injury (TBI)
It can result from a closed head injury (acceleration, deceleration, and
rotational forces resulting in compression and/or shearing of brain tissue)
or an open head injury, in which there is disruption to the skull.
Complex concussion also includes cases of repeated concussions,
including those that result from progressively less impact.
Mild traumatic brain injury or concussion can result in post concussive
syndrome in which the following symptoms can last more than 1 year:
Confusion Visual disturbances Disorientation
Dizziness Headache Balance disturbances
Memory deficits Fatigue Mood swings/irritability
Seizures Poor attention/concentration
Moderate to severe TBI can include the symptoms previously listed but
also may initially result in nausea and vomiting, a worsening headache,
seizures, lethargy, pupil dilation, loss of coordination, weakness of the
extremities and agitation.
Special Tests
Refer to Tab 1 Bonus Content imaging techniques related to skull frac-
tures, intracranial hemorrhage, and hematomas.
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CNS-NP
Tests and Measures
Considerations
Due to deficits in sensation and language, potential areas of break-
down should be monitored
Patient/family should be trained to recognize early stages of skin
breakdown
Motor Function
Assessment - Provide narrative description of
Ability to initiate, maintain, and terminate movement
Ability to shift weight
Medications
Generic Brand Common
Indications Name Name Side Effects
Spasticity diazepam Valium Dizziness, drowsiness,
overall ataxia, nausea, blurred
vision, slurred speech,
confusion, impaired
memory
Seizures phenytoin Dilantin, Slurred speech, dizziness,
Diphenylan nausea, incoordination,
diplopia, nystagmus
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52
Surgery
Surgery may be needed to evacuate hematomas and decompress the
injured brain.
Prognosis
The prognosis is dependent on the:
Severity and location of the injury
Duration and severity of the coma
Duration of post-traumatic amnesia
Age and general health of patient
Referral To Other Health-Care Providers
Neuropsychologistbehavioral/cognitive testing, depression, and
anxiety
Speech-language pathologist for language and dysphagia
Mental health worker to work with multiple needs that occur with
recovery
Resources
Brain Injury Association of America www.biausa.org
Brain Trauma Foundation www.braintrauma.org
53
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Progressive Disorders of the Central Nervous System
(CNS-P)
Alzheimers Disease
Dementia is an acquired, generalized, & usually progressive impairment
of cognitive function without changes in consciousness. Dementia is not
a part of normal aging. Many diseases may cause dementia, with
Alzheimers disease as the leading one.
According to the National Institute of Health (NIH), Alzheimers disease
(AD) initially affects cognitive function & can be divided into 4 stages: pre-
clinical, mild, moderate, & severe. In the preclinical stage, patients often
demonstrate minimal cognitive impairment with memory loss usually
being the first visible sign. AD diagnosis usually occurs during the mild
stage in which symptoms include memory loss, confusion (feeling lost
despite being in a familiar place), taking more time to accomplish daily
tasks, problems with money-handling & bill-paying, poor judgment lead-
ing to bad decisions, loss of spontaneity & initiative, mood & personality
changes, & increased anxiety. In ADs moderate stage, patients may
demonstrate more severe cognitive, perceptual and/or motor problems
such as hallucination, delusion or paranoia, making repetitive statements
or movements, lack of impulse control, difficulty rising from a chair, etc.
Finally, patients with severe AD may no longer recognize family members
or loved ones & often are completely ADL-dependent.
Special Tests
No definitive laboratory & imaging tests to diagnose AD
MRI, blood test, CSF exam to rule out other diseases
Dementia associated with mini stroke or Lewy bodies
Brain tumors (mass lesions)
Delirium
CNS-P
54
Frontotemporal dementia (Picks disease)
Prion diseases (Creutzfeldt-Jacobs disease)
Depression
Hydrocephalus
B
12
deficiency
Hypothyroidism
Demyelinating diseases
Infectious diseases (meningitis, syphilis, HIV/AIDS, etc.)
Lead, mercury, arsenic, or copper poisoning
Head trauma
Wilsons disease
Prognosis
According to NIH, patients with AD generally live between 5 & 20 years
post diagnosis with the average life expectancy post diagnosis being
8 years.
Dieticians
Neurologists
Psychiatrists
Social workers
Amyotrophic Lateral Sclerosis
(ALS or Lou Gehrigs Disease)
Amyotrophic lateral sclerosis (ALS) is characterized by a progressive
degeneration of motor neurons in the spinal cord & cranial nerves. ALS
generally does not involve sensory or autonomic nervous systems.
Patients with ALS may demonstrate both lower motor neuron & upper
motor neuron symptoms, & recent research shows that a small percent-
age of patients with ALS may experience cognitive problems including
memory loss, decision-making difficulty, & even dementia.
For 70% to 80% of the ALS population, initial symptoms involve the
limbs while the remaining population experiences initial symptoms
associated with bulbar signs (difficulty swallowing, slurred speech,
hoarseness, or low speech volume).
Special Tests
Neurological exam should only show muscular or motor involve-
ment; sensory tests should be normal
NCV & EMG (with fasciculation potentials) to verify ALS diagnosis;
sensory nerve conduction velocity (NCV) may appear normal but the
motor nerve is affected
Blood test to detect creatine kinase presence & to confirm familial ALS
Respiratory system test to determine if respiratory muscles are
affected
MRI & CSF exam to rule out other neurological diseases
Other noninfectious disorders of anterior horn cells (motor neurons)
Brain stem tumors
Cervical spondylosis
Dermatomyositis or polymyositis
HIV-associated progressive neuropathy & myopathy
Lyme disease
Multifocal motor neuropathy (diabetic neuropathy)
Myasthenia gravis
Spinal muscular atrophy
Prognosis
Most patients with ALS die from respiratory failure 3 to 5 years following
the onset of symptoms. Only about 10% of patients with ALS survive for
10 years.
55
CNS-P
CNS-P
56
Dietician (patients with advanced illness may have difficulty swallow-
ing & may need tube feeding)
Neurologist
Occupational therapist
Orthotist
Pulmonary specialist
Speech & language pathologist (for swallowing & feeding & assistive
technology for communication)
Social worker
Brain Tumor
According to NIH, brain tumors (BT) primarily affect two groups of
patients: children 0 to 15 years of age & adults 40 to 60 years of age. The
most prevalent BT types are anaplastic astrocytoma & glioblastoma
(38%) & meningiomas & other mesenchymal tumors (27%). BT can be
malignant or benign & can produce symptoms primarily by pressing
against or destroying functioning brain tissue. Symptoms directly corre-
late to BT location (refer to CVA for specific cerebral lesion symptoms,
CNS-NP tab).
Special Tests
MRI (more sensitive than CT) to confirm tumor existence
Biopsy to determine brain tumor cell type
EEG to monitor brain activity & detect seizures
Endocrine assessment to determine if tumor is located in either
pituitary gland or hypothalamus
CSF exam & blood & urine tests to rule out other diseases
Brain abscess
CNS infections
Stroke
Surgery
Perform tumor removal surgery if possible
Prognosis
Prognosis of primary BT depends on type of tumor. From 19972002,
the 5-year survival rate for brain & other nervous system cancer was
approximately 33%.
Neurologists
Neurosurgeons
Occupational therapists
Oncologists
Respiratory therapists
Wheelchair/Assistive Technology specialists
Cerebellar Degeneration
Unilateral damage to a cerebellar hemisphere (vascular occlusion, tumor,
or white matter demyelination in one or more cerebellar peduncles)
results in symptoms affecting the same side of the body as the damaged
hemisphere. Patients with middle cerebellum lesions or patients with
multiple sclerosis (demyelination throughout the brain & spinal cord)
show bilateral symptoms such as ataxic gait.
Types of cerebellar degeneration (CD) include: alcoholic cerebellar
degeneration (occurs among chronic alcoholics & may be caused by
57
CNS-P
CNS-P
58
nutritional deficiency); phenytoin-induced cerebellar degeneration
(caused by high dose of phenytoin therapy); paraneoplastic cerebellar
degeneration (caused by lung cancer, Hodgkins disease, or breast cancer);
autosomal dominant spinocerebellar ataxias (an inherited, mutated gene);
& Friedreich ataxia (an inherited, mutated gene that first shows signs in
childhood).
Special Tests
Genetic testing to identify & verify mutated gene(s)
CT & MRI to rule out posterior fossa tumor, lung & breast cancer &
Hodgkins disease
Blood test & CSF exam to rule out infection
Ataxia - Telangiectasia
Wilsons disease
Prion disease (Creutzfeldt-Jakobs disease)
Posterior fossa tumor
Posterior fossa malformation
Prognosis
Prognosis for patients with CD varies depending on underlying cause.
Patients with Friedreich ataxia usually become wheel-dependent 15 to
20 years after the appearance of symptoms.
HIV Infection
The U.S. Center for Disease Control & Prevention (CDC) estimates that HIV
infects 40,000 Americans each year. Acquired Immune Deficiency
Syndrome (AIDS), a condition that occurs in the most advanced stages of
HIV, may take many years to develop following the initial infection. In the
U.S., approximately 40% of adults with AIDS experience neurological
59
CNS-P
complications, affecting CNS, PNS &/or ANS. Refer to Tab 6 (page xx) for
adults with AIDS and PNS involvement.
There are a number of CNS opportunistic infections found in patients
with HIV/AIDS, the most common of which is cerebral toxoplasmosis
(10-20%). Initial symptoms include malaise, fever, neck stiffness &
hemiparesis while less common symptoms include confusion, aphasia &
vision changes. Cryptococcal meningitis, the most common mycotic
infection in patients with AIDS, may take either an acute course (including
severe headache, mental status changes, fever, nuchal rigidity & focal
signs) or a subacute course (including malaise & headache without neck
stiffness) over several weeks. Cytomegalovirus (CMV) encephalitis usually
results in mental status changes evolving over several weeks & often
causes death. Other common CNS disorders found in patients with
advanced HIV/AIDS illness are AIDS-related dementia, CNS lymphoma &
progressive multi-focal leukoencephalopathy.
Special Tests
Laboratory tests to confirm diagnosis & determine viral load &
opportunistic infection type
CSF test (by lumbar puncture) to determine CNS opportunistic
infection type & rule out other diseases
MRI & CT brain scan to examine infection-related lesions
Classical bacterial cerebral abscess
Dementia
Other types of meningitis
Primary CNS lymphoma
Prognosis
90% of patients with cerebral toxoplasmosis respond to medications
within 2 to 4 weeks. CMV encephalitis often causes death in patients with
HIV/AIDS within weeks to months.
CNS-P
60
Infectious diseases specialists
Huntingtons Disease/Huntingtons Chorea
Special Tests
Genetic testing to confirm diagnosis & identify mutated gene
CT & MRI to verify atrophy of cerebral cortex & caudate nucleus
Benign hereditary chorea
Alzheimers disease
Wilsons disease
Basal ganglion or subthalamic infarction
Multiple sclerosis
Parkinsons disease treated with levodopa
Other drug-induced chorea
Prognosis
There is no cure for Huntingtons disease. Patients with HD usually die 10
to 30 years after the initial onset of clinical symptoms.
Dietitians
Psychiatrists
Speech & language pathologists
Specialists for genetic counseling
61
CNS-P
Lyme Disease
Lyme disease (LD) is a tick-borne disorder resulting from a systemic
infection with spirochete Borrelia burgdorferi & causing flu-like symp-
toms including fever, headache, malaise, muscle & joint pain, & swollen
lymph nodes. About 70% to 80% of patients develop a bulls eye rash
characteristic at the site of the tick bite after a delay of 3 to 30 days. If
untreated, patients may develop Bells palsy, meningitis, shooting pains,
& heart palpitations, though many symptoms resolve without treatment.
Dark-skinned people may develop a rash that resembles a bruise.
If detected early, most patients can be treated successfully with
antibiotics. If untreated, approximately 60% of patients with LD develop
intermittent bouts of arthritis (severe joint pain & swelling) several
months after infection whereas approximately 5% of untreated patients
with LD develop chronic neurological symptoms months to years after
infection. Chronic neurological symptoms include:
Brain dysfunction resulting in memory loss
Cranial nerve damage
Brain & spinal cord inflammation or meningitis
Corneal inflammation causing vision impairment & eye pain
Rapidly progressive motor neuron paralysis involving peripheral
nerve inflammation
Special Tests
Blood test to confirm B. burgdorferi existence
CSF examination (polymerase chain reaction) for patients demon-
strating neurological signs to determine lymphocytic pleocytosis &
slightly elevated proteins
Synovial fluid examination (from an affected joint) to confirm
spirochete presence
CT & MRI to rule out other diseases
CNS-P
62
Alzheimers disease
Bacterial or viral meningitis
Chronic fatigue syndrome
Dementia
Gout or pseudogout
Multiple sclerosis
Prion-related diseases
Radiculopathy
Rheumatoid arthritis
Prognosis
Most patients with LD can be treated successfully with a few weeks of
antibiotics, though patients with chronic, late-stage LD may develop
permanent neurological problems in varying degrees.
Infectious diseases specialists
Neurologists
Orthopedists
Multiple Sclerosis
Special Tests
MRI to detect acute & chronic lesions regardless of size
Visual evoked potentials to assess visual system
CSF analysis to rule out infections, neoplasm, & to confirm
immunoglobulin presence
Blood test to rule out other diseases
63
CNS-P
Kurtzke Expanded Disability Status Scale for Patients
with Multiple Sclerosis
Functional
Systems Findings
Pyramidal
functions
Cerebellar
functions
Brainstem
functions
0 Normal
1 Abnormal signs without disability
2 Minimal disability
3 Mild or moderate paraparesis or hemiparesis;
severe monoparesis
4 Marked paraparesis or hemiparesis; moderate
quadriparesis; or monoplegia
5 Paraplegia, hemiplegia, or marked quadriparesis
6 Quadriplegia
V Unknown
0 Normal
1 Abnormal signs without disability
2 Mild ataxia
3 Moderate truncal or limb ataxia
4 Severe ataxia, all limbs
5 Unable to perform coordinated movements due
to ataxia
V Unknown
X Is used throughout after each number when
weakness (grade 3 or more on pyramidal) inter-
feres with testing
0 Normal
1 Signs only
2 Moderate nystagmus or other mild disability
3 Severe nystagmus, marked extra-ocular weak-
ness, or moderate disability of other cranial
nerves
4 Marked dysarthria or other marked disability
5 Inability to swallow or speak
V Unknown
CNS-P
64
with Multiple SclerosisContd
Functional
Systems Findings
Sensory
functions
Bowel &
bladder
functions
0 Normal
1 Vibration of figure-writing decrease only, in one
or two limbs
2 Mild decrease in touch or pain or position
sense, and/or moderate decrease in vibration in
one or two limbs; or vibratory decrease alone in
three or four limbs
3 Moderate decrease in touch or pain or position
sense, and/or essentially lost vibration in one or
two limbs; or mild decrease in touch or pain
and/or moderate decrease in all proprioceptive
tests in three or four limbs
4 Marked decrease in touch or pain or loss of
proprioception, alone or combined, in one or two
limbs; or moderate decrease in touch or pain
and/or severe proprioceptive decrease in more
than two limbs
5 Loss (essentially) of sensation in one or two
limbs; or moderate decrease in touch or pain
and/or loss of proprioception for most of the body
below the head
6 Sensation essentially lost below the head
V Unknown
0 Normal
1 Mild urinary hesitancy, urgency, or retention
2 Moderate hesitancy, urgency, retention of bowel
or bladder, or rare urinary incontinence
3 Frequent urinary incontinence
4 In need of almost constant catheterization
5 Loss of bladder function
6 Loss of bowel & bladder function
V Unknown
65
CNS-P
Functional
Systems Findings
Visual
(optic)
functions
Cerebral
(mental)
functions
Other
functions
0 Normal
1 Scotoma with visual acuity (corrected) better
than 20/30
2 Worse eye with scotoma with maximal visual
acuity (corrected) of 20/30 to 20/59
3 Worse eye with large scotoma, or moderate
decrease in fields, but with maximal visual acuity
(corrected) of 20/60 to 20/99
4 Worse eye with marked decrease of fields &
maximal visual acuity (corrected) of 20/100 to
20/200; grade 3 plus maximal acuity of better eye
of 20/60 or less
5 Worse eye with maximal visual acuity (corrected)
les than 20/200; grade 4 plus maximal acuity of
better eye of 20/60 or less
6 Grade 5 plus maximal visual acuity of better eye
of 20/60 or less
V Unknown
X Is added to grades 0 to 6 for presence of temporal
pallor
0 Normal
1 Mood alteration only
2 Mild decrease in mentation
3 Moderate decrease in mentation
4 Marked decrease in mentation; chronic brain
syndrome; moderate
5 Dementia or chronic brain syndrome; severe or
incompetent
V Unknown
0 None
1 Any other neurologic findings attributed to MS
V Unknown
CNS-P
66
Functional
Systems Findings
Expanded
Disability
Status
Scale
(EDSS)
0 Normal neurologic examination (all grade 0
in functional systems [FS]; cerebral grade 1
acceptable)
1No disability, minimal signs in one FS (i.e., one
grade 1 excluding cerebral grade 1)
1.5No disability, minimal signs in more than one
FS (more than one grade 1 excluding cerebral
grade 1)
2.0Minimal disability in one FS (one FS grade 2,
others 0 or 1)
2.5Minimal disability in two FS (two FS grade 2,
others 0 or 1)
3.0Moderate disability in one FS (one FS grade 3,
others 0 or 1), or mild disability in three or four FS
(three or four FS grade 2, others 0 or 1)
3.5Fully ambulatory but with moderate disability
in one FS (one grade 3 & one or two FS grade 2)
or two FS grade 3, others 0 or 1, or five FS grade
2, others 0 or 1
4.0Fully ambulatory without aid, self-sufficient,
up & about some 12 hours a day despite relatively
severe disability consisting of one FS grade 4
(others 0 or 1), or combinations of lesser grades
exceeding limits of previous steps; able to walk
without aid or rest some 500 meters (0.3 miles)
4.5Fully ambulatory without aid, up & about
much of the day, able to work a full day, may
otherwise have some limitation of full activity or
require minimal assistance; characterized by rela-
tively severe disability, usually consisting of one
FS grade 4 (others 0 or 1) or combinations of lesser
grades exceeding limits of previous steps; able to
walk without aid or rest for some 300 meters
(975 ft)
67
CNS-P
Functional
Systems Findings
5.0Ambulatory without aid or rest for about
200 meters (650 feet); disability severe enough to
impair full daily activities (e.g., to work a full day
without special provisions); usual FS equivalents
are one grade 5 alone, others 0 or 1, or combina-
tions of lesser grades usually exceeding specifica-
tions for step 4.0
5.5Ambulatory without aid or rest for about
100 meters (325 ft); disability severe enough to
impair full daily activities; usual FS equivalents
are one grade 5 alone, others 0 or 1, or combina-
tions of lesser grades usually exceeding specifica-
tions for step 4.0
6.0Intermittent or constant unilateral assistance
(cane, crutch, brace) required to walk about
100 meters (325 ft) with or without resting; usual
FS equivalents are combinations with more than
two FS grade 3
6.5Constant bilateral assistance (canes, crutches,
braces) required to walk about 20 meters (65 ft);
usual FS equivalents are combinations with more
than two FS grade 3
7.0Unable to walk beyond about 5 meters (16 ft)
even with aid, essentially restricted to wheelchair;
wheels self in standard wheelchair a full day &
transfers alone; up & about in wheelchair some
12 hours a day; usual FS equivalents are combina-
tions with more than one FS grade 4; very rarely
pyramidal grade 5 alone
7.5Unable to take more than a few steps; restricted
to wheelchair; may need aid in transfers, wheels
self but cannot carry on in standard wheelchair a
full day; may require motorized wheelchair; usual
FS equivalents are combinations with more than
one FS grade 4
CNS-P
68
Functional
Systems Findings
8.0Essentially restricted to bed or chair or peram-
bulated in wheelchair; but may be out of bed
much of the day; retains many self-care functions;
generally has effective use of arms; usual FS
equivalents are combinations, generally grade 4
in several systems
8.5Essentially restricted to bed for much of the
day; has some effective use of arm(s); retains
some self-care functions; usual FS equivalents
are combinations, generally grade 4 in several
systems
9.0Helpless bed patient; can communicate & eat;
usual FS equivalents are combinations, mostly
grade 4
9.5Totally helpless bed patient; unable to com-
municate effectively or eat/swallow; usual FS
equivalents are combinations, almost all grade 4
10Death due to MS
Source: Kurtzke JF. On the evaluation of disability in multiple sclerosis. Neurology. 1961
Aug;11;686694, with permission.
Small-vessel cerebrovascular disease
Spinal cord tumor & cervical spondylosis
Spinocerebellar degeneration
Lyme disease
Guillain-Barr syndrome
Systemic lupus erythematosus
HIV/AIDS
69
CNS-P
Prognosis
The life expectancy for patients with MS is about 6 to 7 years less than
that for a control population without MS.
Referral to Other Health Care Providers
Neurologists
Ophthalmologists
Psychiatrist or psychologists
Parkinsons Disease
Special Tests
No laboratory or imaging tests can definitively diagnose PD
MRI, CT scan, & CSF examination to rule out other diseases
Medication-induced parkinsonism (usually from anti-psychotic drugs)
Normal pressure hydrocephalus
Progressive supranuclear palsy (rigid in neck extension & loss of
vertical eye control)
Corticobasal degeneration
Multiple system atrophy
Vascular parkinsonism from multiple strokes
Wilsons disease
Essential (benign, familial) tremor
Surgery
Deep brain stimulation
Thalamotomy or pallidotomy
CNS-P
70
Prognosis
There is no cure for PD at this time. PD is chronic & progressive. Some
patients with PD become severely disabled while others experience
onlyminor motor problems. Patients with PD often die from pulmonary
complications.
Dietitians
Neurologists
Neurosurgeons
Occupational therapists
Social workers
71
PNS
INJURY
Peripheral Nerve Injury (PNI)
Bells Palsy
According to NIH, Bells palsy (BP) is facial muscle weakness or paralysis
resulting from injury to one of the two facial nerves (LMN type). Facial
palsy resulting from facial nerve injury (LMN) differs from facial palsy
resulting from facial motor nucleus or corticobulbar fibers lesions (UMN
type). Generally, BP affects only one side of the face with the frontalis
muscle of the same side being affected, though in UMN types of facial
palsy, the frontalis muscle on both sides of the face remains intact.
BP symptoms usually occur suddenly & may include: inability to close
eye, drooping eyelid or corner of the mouth, drooling, dry eye or mouth,
taste impairment, & excessive tearing in the eye.
Special Tests
NCV & EMG 7 to 10 days after symptom onset to confirm diagnosis
Laboratory exams, MRI & CT scan to rule out other diseases
Lyme disease
Meningitis
Facial nerve or cerebellopontine tumors
UMN type of facial palsy (bilateral frontalis muscles are intact)
Amyloid angiopathy
Multiple sclerosis
Basal skull fracture
Prognosis
According to NIH, most patients with BP begin to recover within 2 weeks
after the initial onset of symptoms & recover completely within 3 to
6 months. Some patients may never completely recover. The extent of
nerve damage determines recovery timing & process.
PNS
INJURY
72
HIV Infection
The U.S. Centers for Disease Control & Prevention (CDC) estimate that HIV
infects 40,000 Americans each year. Acquired Immune Deficiency
Syndrome (AIDS), a condition that occurs in the most advanced stages of
HIV infection, may take many years to develop following initial infection.
In the United States, approximately 40% of adults with AIDS experience
neurological complications.
Peripheral neuropathy observed among patients with HIV/AIDS can be
divided into: HIV-associated sensory or toxic neuropathy, inflammatory
demyelinating polyneuropathy, & autonomic neuropathy.
Sensory neuropathies, particularly distal sensory polyneuropathy &
antiretroviral drug toxic neuropathy, are the most common types of
HIV-related peripheral neuropathies & affect approximately 30% of
patients with HIV/AIDS. Characterized by painful feet & lower limb
dysesthesia, sensory neuropathy symptoms generally worsen at
night & can be aggravated by innocuous stimuli, such as bed sheets
or shoes
Patients with advanced HIV/AIDS generally experience inflammatory
demyelinating polyneuropathies with Guillain-Barr syndromelike
symptoms: progressive weakness, decreased or absent deep tendon
reflexes, & minor sensory involvement
Symptoms of autonomic neuropathy, a rare condition occurring in
patients with advanced HIV/AIDS, include postural hypotension,
bowel & bladder dysfunction, impotence, sweating abnormalities,
presyncope, & arrhythmia
Special Tests
Blood tests to confirm HIV infection & to determine viral load
CSF to rule out Guillain-Barr syndrome
EMG & NCV to determine affected peripheral (motor & sensory)
nerves
Nerve biopsy to assess peripheral nerve inflammation
Diabetic neuropathy
Myopathy
Infection-related neuropathy (e.g., cytomegalovirus)
Syphilis
Vitamin B
6
toxicity
Prognosis
According to NIH, the prognosis for patients with HIV/AIDS has improved
significantly in recent years due to new drugs & treatments. However, the
prognosis for patients with HIV/AIDS & peripheral neuropathy worsens
unless the infection is adequately controlled.
Neurologist
Orthotist
73
PNS
INJURY
PNS
INJURY
74
Nerve Muscles Innervated Sensory Innervation
Sciatic nerve Semitendinosus, See common peroneal
(L45, S13) semimembranosus, biceps nerve & tibial nerve
femoris, adductor magnus
Common Tibialis anterior, peroneus Lateral side of lower
peroneal longus, peroneus brevis, leg, dorsum of foot
nerve (L45, peroneus tertius, extensor
S12, branch hallucis longus & brevis,
of Sciatic extensor digitorum longus &
nerve) brevis
Tibial nerve Soleus, gastrocnemius, Posterolateral half of
(L5, S12, plantaris, politeus, tibialis calf, most of sole
branch of posterior, flexor hallucis
Sciatic nerve) longus, flexor digitorum
longus, all foot muscles in
the sole
Peripheral Nerve Injuries of Lower Limbs
Sciatica
Overview/Description
Emerging from the lumbosacral plexus & branching into the common
peroneal nerves (L4, L5, S1, S2) & tibial nerves (L4, L5, S1, S2, S3), the
sciatic nerve may experience pressure caused by disc herniation, degen-
erative lumbar disc disorders, piriformis syndrome, spinal stenosis or
spondylolisthesis leading to pain radiating down the lower back & into
the back of the lower limbs. Patients with sciatica may also experience
motor problems, including weakened hip extensors & adductors, knee
flexors, ankle dorsiflexors, plantar flexors, evertors, invertors & big toe
extensors; sensory problems such as lower limb & buttock paresthesia or
numbness; & bowel & bladder control problems.
Special Tests
EMG & NCV to confirm sciatic nerve compression level & extent
Radiograph, CT scan, & MRI to examine lumbar spine & surrounding
soft tissue & to rule out other diseases
Aortic aneurysm
Lumbar spine fracture
Sciatic nerve tumor
Spinal tumor
Tarlov cysts
Surgery
Diskectomy or microdiskectomy can be effective if sciatica is caused by
disc herniation. Repeated epidural injections may temporarily decrease
sciatica.
Prognosis
According to Peul and associates, 95% of patients with sciatica show func-
tional improvement with either conservative treatment (medication &
physical therapy) or surgery at 1-year follow-up.
Neurologists
Orthopedists
75
PNS
INJURY
PNS
INJURY
76
Peripheral Nerve Injuries of Upper Limbs
Trunk of Division Cord of
Brachial of Brachial Brachial Muscles Sensory
Nerve Plexus Plexus Plexus Innervated Innervation
Median
nerve
(C58, T1)
Anterior
interosse-
ous nerve
(branch
of median
nerve)
Upper,
middle,
lower
Upper,
middle,
lower
Anterior
divisions
of the
upper,
middle,
& lower
trunk
Anterior
divisions
of the
upper,
middle,
& lower
trunk
Lateral &
medial
Lateral &
medial
Pronator
teres, flexor
carpi radialis,
palmaris
longus, flexor
digitorum
superficialis,
flexor pollicis
longus,
pronator
quadratus,
thenar mus-
cles, radial
half of two
lumbricals
Flexor pollicis
longus, radial
half of flexor
digitorum
profundus,
pronator
quadratus,
thenar
muscles,
radial two
lumbricals
Radial half
of palm;
palmar side
of thumb,
2nd, 3rd, &
radial half of
4th finger;
dorsal side
of distal
third of 2nd,
3rd, & radial
half of 4th
finger
None
77
PNS
INJURY
Peripheral Nerve Injuries of Upper LimbsContd
Radial
nerve
(C58, T1)
Posterior
interosse-
ous nerve
(branch
of radial
nerve)
Upper,
middle,
lower
Upper,
middle,
lower
Posterior
divisions
of the
upper,
middle,
& lower
trunk
Posterior
divisions
of the
upper,
middle,
& lower
trunk
Posterior
Posterior
Triceps,
brachioradialis.
extensor carpi
radialis longus
& brevis,
supinator,
extensor carpi
ulnaris,
extensor
digitorum,
extensor
pollicis longus
& brevis,
abductor
pollicis longus,
extensor
indices,
extensor digiti
minimi
Extensor carpi
radialis brevis,
extensor
digitorum,
extensor
pollicis longus
& brevis,
abductor
pollicis longus,
extensor carpi
ulnaris,
extensor
indices,
extensor digiti
minimi
Radial 2/3
dorsum of
the hand,
dorsum &
lateral half
aspect of
the thumb,
proximal
1/3 dorsum
of the 2nd &
3rd fingers
& radial
half of the
4th finger
None
Continued
PNS
INJURY
78
Peripheral Nerve Injuries of Upper LimbsContd
Ulnar
nerve
(C78, T1)
Lower Anterior
division
of the
lower
trunk
Medial Flexor carpi
ulnaris, ulnar
half of flexor
digitorum
profundus,
palmaris
brevis,
hypothenar
muscles,
adductor
pollicis,
ulnar two
lumbricals,
all interossei
muscles
Ulnar half of
palm and
dorsum of
hand, dorsal
& palmar
side of the
5th finger &
the ulnar
half of the
4th finger
Carpal Tunnel Syndrome
Carpal tunnel syndrome (CTS) occurs when the median nerve (C58, T1)
becomes compressed at the wrist. Sensory problems (tingling or
numbness of thumb, 2nd, & 3rd fingers, radial half of 4th finger, & radial
half of palm) normally are the first indicators of CTS with motor prob-
lems (weak grip or pinch & weak or no thumb flexion & opposition)
appearing later. CTS usually affects the dominant hand first. Patients
with CTS sometimes drop objects from the affected hand when distract-
ed due to sensory problems & weakness. Women are three times more
likely than men to develop CTS.
Median nerve injury at the elbow may cause pronator & wrist flexor
weakness & limited wrist radial deviation in addition to the above func-
tional impairments.
Special Tests
EMG & NCV to determine entrapment level & nerve injury extent
Radiographs, CT scan,& MRI of neck & upper limbs to rule out other
pathology
Surgery
Carpal tunnel release surgery (open release or endoscopic surgery) to
reduce compression of median nerve.
Pronator teres syndrome
To determine if the patient has pronator teres syndrome
Patient fully flexes elbow to 90
Examiner resists pronation as patient extends elbow
Tingling or paresthesia along median nerve distribution in
forearm or hand indicates pronator teres syndrome
Tendonitis
Tenosynovitis
Compressive neuropathies of cervical spine nerve roots & brachial
plexus
Proximal median neuropathy
Polyneuropathy
Prognosis
According to NIH, most patients with CTS respond well to treatment. The
less severe the symptoms, the better the prognosis.
Ergonomists
Occupational therapists or hand therapists
Orthopedists
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Radial Nerve Injury
Radial nerve (C58, T1) compression in the spiral groove of the humerus
is the most common cause of radial nerve injury (RNI).
Forearm RNI can lead to grasping, finger extension & thumb abduction
problems as well as sensory loss at the radial 2/3 dorsum of the hand,
dorsum, & lateral half aspect of the thumb, proximal 1/3 dorsum of the
2nd & 3rd fingers, & radial half of the 4th finger.
Elbow RNI can lead to difficulty with forearm supination & wrist exten-
sion and above-elbow RNI can cause elbow extension problems in addi-
tion to the aforementioned functional impairments.
Special Tests
EMG & NCV to confirm RNI level & extent
Radiograph to rule out mid-humeral fracture
MRI to rule out nerve tumor, aneurysm, & rheumatoid synovitis
Brachial plexopathy
Peripheral neuropathy from diabetes mellitus, renal failure, HIV/AIDS,
Lyme disease, systemic lupus erythematosus, vitamin deficiency, or
toxin exposure
Stroke
Lateral epicondylitis
Surgery
Depending on the site of compression, several types of decompression
surgery may relieve pressure on radial nerve.
Prognosis
Depends upon RNI type (refer to Tab 1).
Neurologists
Occupational or hand therapists
Orthopedists
Ulnar Nerve Injury (Entrapment)
Ulnar nerve (C78, T1) injury (UNI), the second most common upper
limb nerve entrapment syndrome, can occur either at the elbow or
wrist. Wrist UNI can lead to inability to: flex the 5th finger; abduct &
adduct fingers; extend the proximal & distal interphalangeal joints of
the 4th & 5th fingers; sense light touch, pain, & temperature on the
ulnar half of palm and dorsum of hand, dorsal & palmar side of the
5th finger, & the ulnar half of the 4th finger.
Elbow UNI can lead to wrist flexor weakness & ulnar deviation loss in
addition to the aforementioned functional impairments.
Special Tests
EMG & NCV tests to determine the location of ulnar nerve
entrapment
Radiograph to rule out upper limb fractures
MRI & CT scan to rule out cervical spine & disc problems
Blood & urine tests to rule out other diseases
Cervical disc disease
Brachial plexus abnormalities
Thoracic outlet syndrome
Elbow abnormalities (e.g., epicondylitis, previous medial humeral
epicondyle fractures, etc.)
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82
Neuropathy resulting from infections, tumors, diabetes mellitus,
hypothyroidism, or alcoholism
Other systematic diseases (e.g., ALS)
Wrist fractures
Ulnar artery aneurysms or thrombosis at the wrist
Surgery
May be necessary to decompress ulnar nerve.
Prognosis
Depends on injury severity
Ergonomist
Occupational or hand therapist
Orthopedist
Peripheral Vestibular Diseases
Benign Paroxysmal Positional Vertigo (BPPV)
Special Tests
Electronystagmography (ENG) to objectively quantify the velocity,
frequency, & amplitude of spontaneous-induced nystagmus
CT scan, evoked auditory potential studies, MRI to rule out acoustic
neuroma & other brain lesions
Central vestibular disorders (refer to Tab 4)
Cerebellar tumor or stroke involving cerebellar arteries
Acoustic neuroma
Vertebrobasilar vascular disease
Posterior cerebral vascular disease
Multiple sclerosis
Temporal lobe seizure
Perilymph fistula syndrome
Migraine
Surgery
If conservative treatment (canalith repositioning procedure) or habitua-
tion exercise are unsuccessful, consider bone plug implantation surgery
to block part of affected inner ear.
Prognosis
Most patients with BPPV respond well to the Epley/canalith repositioning
maneuver.
Ear, nose, & throat specialists
Menieres Disease
Menieres disease (MD), a vestibular disorder with an unknown cause, is
associated with a recurring symptom set that includes sudden onset of
severe vertigo, tinnitus, hearing loss, & pain & pressure in the affected
ear. MD attacks may last from 20 minutes to 24 hours & the duration
between attacks varies, ranging from less than a day to a number of
years. MD symptoms generally involve excessive endolymph in the inner
ear with progressive, fluctuating hearing loss being the most significant
(unlike other peripheral vestibular disorders). MD may affect both ears,
but generally, it affects only one.
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84
Special Tests
Serial audiograms to determine the extent & progression of hearing
loss
Brain stem auditory evoked potentials
Caloric test
Blood test, urine test, ENG, & MRI to rule out other diseases
Brain tumor
BPPV
Perilymph fistula syndrome
Central vestibular disorders (refer to Tab 4)
Labyrinthitis
Transient ischemic attacks
Stroke
Surgery
If medications are ineffective, surgery can be performed to relieve inner
ear pressure (endolymphatic shunt or endolymphatic sac decompression)
or destroy either the inner ear (labyrinthectomy) or vestibular nerve
(vestibular nerve section).
Prognosis
MD is progressive & incurable, but can be controlled through medication.
Audiologists
Ear, nose, & throat specialists
Trigeminal Neuralagia (Tic Douloureux)
Trigeminal neuralgia (TN), also known as tic douloureux due to associated
facial tics, is a chronic pain condition causing severe, sudden burning
episodes or shock-like facial pain lasting between seconds & minutes.
Although TN can affect anyone at any age, it most often occurs in patients
over age 50 & is more common in women. One potential cause of TN is a
blood vessel pressing on the trigeminal nerve as it exits the brain stem. TN
is also associated with demyelinating diseases, such as multiple sclerosis.
Pain
See the photo for Trigeminal Nerves in the Cranial/Peripheral Nerve
Integrity section of Tab 2.
Special Tests
CT scan & MRI to rule out other diseases
Atypical facial pain
Temporomandibular joint syndrome
Glossopharyngeal neuralgia
Trigeminal root compression due to tumors
Dental problems
Multiple sclerosis
Surgery
Microvascular surgery can decompress the nerve while different rhizotomy
procedures (alcohol injections, glycerol injections, or partial sensory
rhizotomy) can destroy the offending nerve.
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86
Prognosis
While TN is not fatal, it can cause incapacitation due to severe pain.
Neurologists
Axonal Polyneuropathy
Alcoholic Neuropathy
Alcoholic neuropathy results from long-term alcohol abuse, which causes
a toxic effect on the nerve. The symmetric pattern of neuropathy begins
distally and progresses proximally. Dysesthesia and pain are common
complaints.
Diabetic Neuropathy (Polyneuropathy in Diabetes)
Diabetic neuropathy (DN), one of the most common complications of
diabetes mellitus, can affect all tissues of the body. It usually occurs as
an insidious onset of symmetric damage to the nerve fibers and can
result in pain, impaired sensation, and motor dysfunction. Neuropathy
begins distally and advances proximally.
Diabetes can also result in autonomic neuropathy. The symptoms can
include:
Orthostatic hypotension
Dysphagia
Elevated heart rate
Myocardial infarction
Arrhythmia
Diminished thermoregulatory functions
Gastroesophageal reflux disease (GERD)
Delayed gastric emptying
Constipation
Diarrhea
Sexual dysfunction
Polyneuropathy Due to Other Toxic Agents
Exposure to environmental and occupational agents such as lead,
arsenic, and mercury can result in PN. Antiviral drugs, including ddI and
ddC, chemotherapy agents such as vinca alkaloids (vincristine), cisplatin,
and paclitaxel and other medications can also cause polyneuropathies.
Once symptoms are recognized, exposure should be eliminated;
87
POLY-PNS
POLY-PNS
88
chemotherapy dosage may have to be adjusted or terminated. Although
symptoms usually subside with removal of the neurotoxic agent, it may
take months for recovery. However, long-term exposure can result in
permanent neuronal damage, especially in the presence of other medical
conditions, including diabetes and alcoholism.
Polyneuropathy in Other Diseases
Chronic renal failure results in a buildup of uremic toxins. Patients
experience symmetrical sensory and motor neuropathy, which is more
common in the lower limbs than the upper limbs. PN is also a common
occurrence in HIV.
Special Tests
Blood glucose levels
Electromyography and nerve conduction studies
Genetic testing to rule out inherited neuropathies, such as
Charcot-Marie-Tooth disease
Tests and Measures
The values of the following tests & measures are generally within normal
limits in the absence of other complications: Arousal, attention, cognition;
ergonomics and body mechanics; joint integrity and mobility; neuromotor
development; ventilation and respiration; and work, community, and
leisure integration.
Considerations
Assessment
Assess skin temperature, usually performed by palpation, but can be
objectively measured with a hand-held infrared skin thermometer,
such as Temp Touch (Xilas Medical (obtain a baseline and compare
temperatures on each side of the body)
Muscle Performance
Assessment
Perform manual muscle testing
Use dynamometer to test grip strength
Orthotic, Protective, and Supportive Devices
Considerations
The need for properly fitted footwear is vital to protect the foot and
prevent damage
Bony prominences should be protected
Myelopathy
Radiculopathy
Muscle disease
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POLY-PNS
Medications
Indications Generic Name Brand Name Common Side Effects
Mild pain Local anesthetic Xylocaine Nervousness, lightheaded-
including: ness, and drowsiness
lidocaine
capsaicin
Peripheral Anticonvulsants Neurontin Low white blood cell
neuropathy including: Tegretol count, nausea, vomiting,
gabapentin Lyrica and dizziness
carbamazepine
pregabalin
POLY-PNS
90
Prognosis
Prognosis is dependent on the status of treatment of underlying
cause and/or disease process
Diabetic neuropathy progresses slowly and can be halted with
treatment
Referral To Other Health-Care Providers
Ophthalmologist for visual acuity
Mental health workers for depression that accompanies the pain
associated with polyneuropathies; for treatment of alcoholism
Podiatry for foot care
Diabetic specialist
Resources.
American Diabetes Association http://www.diabetes.org
Juvenile Diabetes Research Foundation International www.jdrf.org
Lower Extremity Amputation Prevention www.hrsa.gov/leap/default.htm
Guillain-Barr Syndrome
It occurs following an infectious illness, such as an upper respiratory or
bacterial infection. Progressive paralysis ensues in a matter of days to
weeks. A diagnosis of GBS is made based on clinical findings and his-
tory. Severity varies from mild to complete tetraplegia accompanied by
the need for tracheotomy and mechanical ventilation.
Special Medical Tests
EMGs reveal the pattern and severity of nerve involvement and to
confirm the diagnosis
Nerve conduction studies reveal slowed conduction velocity and
severity of peripheral neuropathy damage, which may include axonal
degeneration
Lumbar puncture usually contains an elevated level of CSF protein
Pulmonary function tests are used for measures of respiratory func-
tion to predict diaphragmatic and abdominal strength and need for
ventilatory support.
Videofluoroscopy is used to assess swallowing problems
Tests and Measures
The values of the following tests & measures are generally within normal
limits in the absence of other complications: Anthropometric characteris-
tics; arousal, attention, cognition; ergonomics and body mechanics; joint
integrity and mobility; neuromotor development; posture; and range of
motion including muscle length.
Assessment
Assess skin using the Classification of Pressure Sores (Refer to Tab 2).
Potential findings
In the early course of GBS, there is a risk of breakdown due to the
significant immobility
Motor Function
Assessment
Assess functional abilities, including getting up from a chair
Self-Care and Home Management
Assessment
Assess bowel and bladder function
Findings
Patients may have micturitional disturbances secondary to bladder
areflexia or impaired bladder sensation
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92
Ventilation and Respiration
Assessment
Potential findings
Patients requiring ventilatory support are at-risk for developing
pneumonia
GBS is questionable if findings reveal asymmetrical muscle weakness
or a demarcated line of sensory impairment
Lyme disease
Nutritional and toxic neuropathies
Acute myasthenia gravis
Chronic inflammatory demyelinating polyneuropathy
Neoplastic meningitis
Prognosis
Recovery from GBS varies; most patients have functional recovery within
6 to 9 months. The greatest residual effects are weakness in the anterior
tibialis and intrinsic muscles of the hands and feet. Residual weakness is
present after 3 years in about 30% of people with GBS.
The risk of death is low but increases with age and for those needing
ventilatory assistance, especially when complicated by pneumonia or
sepsis.
Speech-language pathologist to address dysphagia and other
oral-motor issues
Social work and psychology
Resources
Guillain-Barr Syndrome International http://www.gbsfi.com/
Nonprogressive Disorders of the Spinal Cord
(Spinal Cord Injury)
Spinal cord injury (SCI) exists in a 4:1 male versus female ratio
According to the National Spinal Cord Injury Database, the distribu-
tion of SCI injuries is as follows
Incomplete tetraplegia (34.5%)
Complete paraplegia (23.1%)
Complete tetraplegia (18.4%)
Incomplete paraplegia (17.5%)
Patients with paraplegia may demonstrate motor & sensory loss of
the trunk & lower limbs & functional loss in the pelvic organs
Patients with tetraplegia may demonstrate motor & sensory loss in
the trunk, upper, & lower limbs, & functional loss in the pelvic organs
Patients with complete SCI lack all motor & sensory function below
the injury level, including S4 to S5, whereas patients with incomplete
SCI retain some motor & sensory function below the injury level
The neurological level recorded for a patient with SCI is the most
caudal (distal) level of the spinal cord with normal motor & sensory
function on both sides of the body
Special Tests
Radiograph to determine the level & extent of injury to the spine
CAT scan to determine extent of the bony abnormality or fracture
MRI to determine extent of SCI or other soft tissue injury
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94
Brain tumor
Spinal cord tumor
Transverse myelitis
Prognosis
According to the Spinal Cord Injury Information Network, the life expectancy
for patients with SCI has increased over the last three decades, though the
higher the lesion level & involvement, the shorter the life expectancy. The
life expectancy for patients with paraplegia is on average 5 to 10 years
shorter than for those without SCI & the life expectancy is 15 to 22 years
shorter for patients with tetraplegia than for those without SCI.
The following referrals should be made:
Driver rehabilitation specialist
Nurse
Orthotist
Neurologist
Psychologist
Assistive technology/wheelchair specialist
Urologist
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Electrophysiologic Studies

Auditory-Evoked Potential (AER) uses sounds in the ear to determine the

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