brain nearly 2% of weight of the body consumption 15% of cardiac output 15-20% total oxygen

Supply of O2 and glucose is maintained by stable CBF (range 4060 ml/100 g of tissue/min) by intrinsic homeostatic control over a wide range of mean arterial blood pressure (60-160 mmHg) = autoregulation = ability of the cerebral vasculature to keep blood flow constant in response to decreased or increased perfusion pressure .

Two essential formulas of cerebral vascular regulation CPP CBF = --------------CVR

CPP = MAP - ICP

CBF cerebral blood flow ICP intracranial pressure CPP - cerebral perfusion pressure MAP mean arterial pressure CVR cerebrovascular resistance

cerebral vascular resistance resistance of cerebral vessels and rheological (flow) propertis of blood (viscosity, erythrocyte blood count and rigidity etc.

After cessation of blood supply following occlusion of a cerebral vessel, neuronal cell death occurs in the core of the infarcted area within few minutes. The area surrounding the core, called the ischaemic penumbra, contains functionally impaired but still viable brain tissue supplied from collateral vessels. This area may be transformed into infarction due to secondary neuronal damage

1. 2. 3. 4.

unaffected area benign oligemia ischemic penumbra ischemic core (infarction)

Atherosclerosis
endothelial dysfunction intimal proliferation of fibrous and muscular tissue elements - fibromuscular plaque Lipoprotein particles brings into the injured parts cholesterol, which is deposit here, accumulation of lipids, due to activity of LDL makrophages change to foam cells and arise atherosclerotic plaques. In intimal sclerotic plaques further changes could arise as calcifications, ulcerations, necrosis and bleeding, continue further adhesion and aggregation of thrombocytes and thrombus formation

Atherosclerosis
the transportation of cholesterol by LDL (lipoprotein) to peripheral sites. Monocytes then adhere to endothelium injured by local accumulation of cholesterol and migrate to subendothelial area as foam cells. Smooth muscle cells proliferate and fibrous plaques develop, with progressive stenosis of the vascular lumen as the process continues. Increased platelet activation and fibrin formation, decreased fibrinolytic and anticoagulant activity

Atherosclerotic risk factors

I. non-modifiable
 age atherosclerosis is a disease of higher age,

increase incidence in 6th decade, but common even in younger age. Risk of stroke increases any 20 yrs approx. 10  heredity - more common in some families, familial aggregation of heart disease and stroke  gender in younger age more common in males, after menopause increases risk in female (perhaps protective influence of estrogens)

Risk factors II. modifiable

 cigarette smoking (contributes to endothelial

dysfunction, decrease of HDL and increase LDL, incr. fibrinogenaemia and changed reactivity of platelets) . Risk of stroke increases approx. 2  hypertension important not only current BP but duration of hypertension. Risk factors of hypertension are high intake of salt, diabetes, obesity, alcohol abuse and stress. Risk of stroke increases approx. 6

Blood pressure Optimal < 120 / < 80 mmHg Normal 120-129 / 80-84 High normal 130-139 / 85-89 Mild hypertension 140-159 / 90-99 Moderate hypertension 160-179 / 100-109
Hypertension = repeated increase of BP 140/90 at least in 2 from 3 measures in minimal 2 visits

Risk factors
II. modifiable
 obesity only supporting factor, often associated

with hypertension, diabetes, increased cholesterol and lipids and lack of physical activity.

Risk factors
III. partially modifiable
 diabetes mellitus, even impaired glucose tolerance as

a praediabetic state. Diabetes increases risk of stroke approx. 1.5-3, part. in younger patients under 55 yrs.
 Hyperlipidaemia - increased serum lipids partic.

cholesterol in fraction of plasma lipoproteins with low density (LDL, VLDL). Risk factor is cholesterol > 5,2, LDL > 3,4 and HDL < 0,9 mmol/l. Ratio cholesterol/HDL indicates risk atherosclerotic index (normal <4).

Risk factors
IV. associate  lack of physical activity. Physical activity decreases BP, increases HDL cholesterol level, decreases LDL cholesterol and improve glucose tolerance.  excessive alcohol consumption is a risk factor, moderate consumption (1224 g/day) could be protective. Recommended daily doses: 1 l 10o beer or 3 dcl wine or weak 1 dcl 40% spirits.

The major subtypes of stroke

Cardioembolic stroke Other

4% 16% 8%
SAH

Large vessel disease

40%

Intracerebral hemorrhage

12%

20%
Small vessel disease

Small vessel occlusion - lacunar infarction - lacunes are small cavities mainly deep in the brain - basal ganglia (internal capsule, thalamus), brain stem.

Risk factors of stroke

 Heart disease part. coronary heart disease, congestive heart failure, atrial fibrillation and valvular heart disease. Atrial fibrillation increases risk of stroke up to 6
 Significant risk factor is

prior stroke and transient ischemic attack (TIA)

STROKE
An acute neurologic injury occurring as a result of vascular lesion, predominantly focal and rarely global There are two main types of stroke. Ischemic stroke is caused by blockage of a blood vessel; hemorrhagic stroke is caused by bleeding. WHO (World Health Organization) criteria, defining stroke as rapidly developing clinical symptoms lasting more than 24 hours or leading to death without any other etiology other than a vascular abnormality

Transient ischemic attack (TIA)

sudden loss of focal cerebral function sudden disorder of vision (one eye) duration less then 24 hrs
50% duration up to 1 hr 90% up to 4 hrs over 4 hrs increasing risk of stroke and structural changes of brain Amaurosis fugax (transient occlusion of the ophtalmic artery)

Large vessel occlusion territorial infarcts major territories

Brainstem crossed or alternating syndromes

ipsilateraly
cranial nerve lesion, Horners sy, gaze palsy (horizontal to the side of lesion), cerebellar signs

contralateraly
tract signs (hemiparesis, hemihypestesia)

Time to peak- TTP Flow perfusion Blood volume

Perfusion CT before and

after thrombolytic therapy

CT AG MIP reconstruction (maximum intensity projection)

Thrombolytic therapy
1) Intravenous recombinant tissue plasminogen activator - rtPA

Intra-arterial thrombolytic therapy

Ultrasound - duplex scanning noninvasive investigation can identify occlusive/stenotic vascular disease.

Occlusion of the internal carotid artery may be asymptomatic or a catastrophic infarction may result.

Carotid Stenosis

CTA stenosis + plaques

MRA only stenosis

Carotid surgery- endarterectomy

Carotid angioplasty, with or without stenting

PTA - percutaneous
transluminal angioplasty with or without stenting

In elderly amyloid angiopathy often multiple lobar hemorrhages - not very severe

Extracerebral subarachnoid haemorrhage SAH aneurysms (berry, saccular)

SAH - severe
headache, acute, nausea, vomiting, loss of consciousness or epileptic seizure. Neck stiffness develops after 3-12 hours. No focal signs, bleeding is extracerebral

Complications intracerebral haematoma, rebleeding, reruprure, arterial spasms (ischaemia), hydrocephalus

surgery (clipping, wrapping) endovascular - coiling

Arteriovenous malformation AVM masses of dilated vessels

Treatment surgery or transvascular embolisation

cavernous hemangiomas - cluster

dilated vessels (veins) without interposed glial or nervous tissue; angiographically occult

Intracranial tumors primary arise from glial cells (50-60% gliomas - glioblastoma, astrocytoma, oligodendroglioma), ependymal cells (ependymoma), supporting tissue (meningioma, schwannoma), in childhood from more primitive cells (meduloblastoma) secondary metastatic 20-40% When do we suggest possibility of intracranial tumor? Any patients with focal signs or raised intracranial pressure (or epilepsy as well) should be considered brain tumor.

WHO Classification of CNS Tumors

Neuroepithelial Tumors - gliomas (astrocytoma, glioblastoma, oligodendroglioma, ependymoma, tumors of the choroid plexus, primitive neuroectodermal tumors (PNET - medulloblastoma) II. Tumors of cranial and spinal nerves - schwannoma, neurofibroma III. Meningeal tumors - meningioma IV. Hematopoetic tumors - lymphoma V. Germ Cell Tumors - germinoma, terratoma VI. Cysts and Tumor-like Lesions VII. Tumors of the Sellar Region VIII. Metastatic tumors I.

 Cerebellar hemangioblastoma (with a cystic cavity), occurs in the middle-aged. Medulloblastoma (primitive neuroectodermal tumor) - in childhood, about 5 years. Arise in cerebellar vermis and extend into 4th ventricle. All are highly malignant and spread throughout the CSF pathways. Truncal and gait ataxia, signs and symptoms of raised intracranial pressure, sometimes particularly vomiting without headache! Another tumor in childhood is cerebellar astrocytoma - usually low grade and benign Pituitary adenoma - classification based on the hormone type secreted: GH secreting tumor, prolactinoma - hyperprolactinemia can be proved (amenorrhea, galactorhea, AG syndrome impotence, infertility Carcinomatous meningitis - evidence of primary neoplasm malignancy (lung, breast, gastrointestinal tract, leukemia/lymphoma. CSF signs of chronic/subacute meningitis with reduction in CSF glucose. Malignant cells

Tumors of spine and spinal cord extramedullary primary (meningioma, schwannoma, less common angioma and AV malformation metastatic secondary, in particular cancer of breast, lung, prostate, kidney, gynecological cancer, lymphogranuloma and leukaemic infiltrates. intramedullary - mostly ependymoma and glioma Symptoms and signs - pain, particularly in extramedullary processes, can be of radicular pattern and propagation in dermatome. Compressive radicular syndrome continue to compressive spinal cord syndrome

Craniocerebral injuries

Traumatic brain injury (TBI)

Skull fractures
Linear Compound comminution of fragments Depressed

Skull Fractures
Basilar skull fracture Penetrating - dural tear,
communication between the external environment and the cranial cavity.

SAH and intracranial air (pneumocephalus)

temporal emporal fracture

racoon sign

Battles sign

Traumatic brain injury (TBI) 1. Primary

 Minor head injury (MHI). Mild traumatic brain

injury. Cerebral concussion (commotio cerebri)

 Contusion (contusio cerebri)  Diffuse axonal injury

Principal criteria: loss of consciousness, Glasgow Coma Score, retrograde or post-traumatic amnesia and focal signs Clinical assessment: conscious level, pupil response, limb weakness

Primary brain injury

1. Concussion - transient loss of consciousness not associated with focal neurologic deficits, retrograde amnesia, autonomic dysfunction, vomiting, dizziness 2. Contusion - frontal and temporal lobes -usually multiple, on the site of the blow - coup or on opposite site - contrecoup.
The clinical signs produced by contusions vary with their location and size

3. Diffuse axonal injury, white matter lesion. Widespread acute disruption of axons.

Contusion - directly underneath the point of impact

(coup injury) or sudden deceleration results in an injury to the brain on the opposite side (countercoup injury).

Diffuse axonal injury

2. Secondary brain damage A. Traumatic intracranial hematomas

1) Epidural hematoma

a skull fracture tearing the middle meningeal vessels

2) Subdural hematoma - acute

- subacute - chronic - hygroma
rupture bridging veins from the cortical surface to the venous sinuses.

3) Traumatic subarachnoid hemorrhage common in cerebral contusions

4) Intracerebral hematomas - severe injuries

with skull fractures, cerebral contusions, lacerations and diffuse axonal injury

B. Cerebral edema with venous turgescence C. Hypoxic-ischemic injury (encephalopathy )

Delayed effects

Postconcussion syndrome - recurrent headache, tinnitus, dizziness Epilepsy - early, late. Common after penetrating wounds CSF fluid leak (rhinorrhoea, otorrhoea) meningitis Multiple contusions - posttraumatic encephalopathy, dementia

Spinal cord injury (SCI) spinal cor lesion: destruction,

compresion, ischemia + edema

Traumatic SCI partial (incomplete) complete

spine fractures and dislocation 1/2 in C, 1/6 v Th a 1/3 v LS spine the most common in C5 a Th12

segmental
symptoms and signs

tract
symptoms and signs

Traumatic spinal cord injury quadriparesis/tetraparesis 29,5 % paraplegia 27,9 % paraparesis 21,3 % quadriplegia/tetraplegia 18,5 %

Transient Cord Injury - spinal cord concussion = complete recovery in 24, but even in 72 hrs clinical term only Rapidly progressive motor neuron damage often produces a "flaccid paralysis", spinal shock - inhibition of all activity (reflex also) below the lesion.

Traumatic spinal cord injury

Neurologic level of injury - Most

caudal level at which both motor and sensory levels are intact,

Zone of partial preservation - This

index is used only when the injury is complete. All segments below the neurologic level of injury with preservation of motor or sensory findings

Warning - bedsores

Key sensory testing - dermatome

C2 - Occipital protuberance C3 - Supraclavicular fossa C4 - Top of the acromioclavicular joint C5 - Lateral side of antecubital fossa C6 - Thumb C7 - Middle finger C8 - Little finger T1 - Medial side of antecubital fossa T2 - Apex of axilla T3 - Third intercostal space (IS) T4 - 4th IS at nipple line T5 - 5th IS T6 - 6th IS at the level of the xiphisternum

Key sensory testing

T8 - 8th IS (midway between T6 and T10) T9 - 9th IS (midway between T8 and T10) T10 - 10th IS or umbilicus T11 - 11th IS (midway between T10 and T12) T12 - Midpoint of inguinal ligament L1 - Half the distance between T12 and L2 L2 - Mid-anterior thigh L3 - Medial femoral condyle L4 - Medial malleolus L5 - Dorsum of the foot at third metatarsophalangeal joint S1 - Lateral heel S2 - Popliteal fossa in the midline S3 - Ischial tuberosity S4-5 - Perianal area (taken as one level)

Key muscles - myotome

C5 - Elbow flexors (biceps, brachialis) C6 - Wrist extensors (extensor carpi radialis longus and brevis) C7 - Elbow extensors (triceps) C8 - Finger flexors (flexor digitorum profundus) to the middle finger T1 - Small finger abductors (abductor digiti minimi) L2 - Hip flexors (iliopsoas) L3 - Knee extensors (quadriceps) L4 - Ankle dorsiflexors (tibialis anterior) L5 - Long toe extensors (extensors hallucis longus) S1 - Ankle plantar flexors (gastrocnemius, soleus)

Traumatic incomplete syndromes

Central cord syndrome often is
associated with a cervical region injury leading to greater weakness in the upper limbs than in the lower limbs with sacral sensory sparing. BrownBrown-Squard syndrome often is associated with a hemisection lesion of the cord, causing a relatively greater ipsilateral proprioceptive and motor loss with contralateral loss of sensitivity to pain and temperature. Anterior cord syndrome often is associated with a lesion causing variable loss of motor function and sensitivity to pain and temperature, while proprioception is preserved.

Traumatic incomplete syndromes

Conus medullaris syndrome is associated with injury to the sacral cord and lumbar nerve roots leading to areflexic bladder, bowel, and lower limbs, while the sacral segments occasionally may show preserved reflexes (eg. bulbocavernosus and micturition reflexes). Cauda equina syndrome is due to injury to the lumbosacral nerve roots in the spinal canal leading to areflexic bladder, bowel, and lower limbs.

Whiplash injury

Epilepsy
Seizure, epileptic attack Definitions physiological occasional sudden excessive rapid and local discharges of gray matter (neurons) clinical intermittent, paroxysmal, stereotyped disturbance of consciousness, behavior, emotion, motor function, perception or sensation epileptic focus group of neurons with pathological electrical activity - impaired resting membrane potential onto action membrane depolarization hyperexcitability. abnormal neuronal discharges with signs of hyperautorytmicity (repetitive bursts of high potentials) a hypersynchronism (neurons discharge all at once).

Essential classification and types of seizures

GENERALIZED
 CONVULSIVE tonic or clonic convulsions, myoclonic, loss of consciousness  NONCONVULSIVE absences (petit mal), atonic, akinetic, disorder of consciousness

PARTIAL/FOCAL
 SIMPLE no alteration of consciousness focal motor or sensory signs, adversive seizures  COMPLEX episodic changes in behavior, disorder of consciousness, automatisms

Complex partial seizures

episodic changes in behaviour in which an individual loses conscious contact with the environment. Psychomotor or temporal lobe epilepsy. Gustatory (taste) or olfactory (smell) hallucinations (as a burning rubber), lip smacking (mlaskn), swallowing, walking aimlessly - automatisms. Memory disturbances - deja vu (current experience has happened before), jamais vu (feeling unfamiliarity), dreamy states. Visceral disturbances (epigastric fullness, nausea, pallor), affective disturbance (displeasure, fear).

Seizure, epileptic attack

The prodrome refers to mood or behavioral changes which may precede the attack by some hours. The aura refers to the symptom immediately before the seizure and will localize the attack to its point of origin within the brain. The ictus refers to the attack or seizure itself. The postictal period refers to the time immediately after the ictus during which the patient may be confused, disorientated and demonstrate automatic behavior

Epilepsy describes a condition in which a person has

recurrent seizures due to a chronic, underlying process.

Status epilepticus Continuous seizures or repetitive, discrete seizures with impaired consciousness in the interictal period. The duration of seizure activity sufficient to meet the definition of status epilepticus has traditionally been specified as 15 to 30 min.

Fits or blackouts
Describe the attack - do the attack occur during sleep, work, standing or sitting position, does he fall? Are there any after-symptoms (sleep, headache, confusion, paralysis). It is important to obtain description from an eye-witness.

Non epileptic blue breath-holding attacks

night terrors febrile seizures

Syncope, fainting - transient loss of consciousness with

postural collapse caused by an acute decrease in cerebral blood flow orthostatic - postural - hypotension, abrupt loss of consciousness on assuming the erect posture

Mesial Temporal Sclerosis (MTS) - degenerative affection of internal parts of temporal lobe with atrophy, loss of neurons and restructuring of tissue

Bilateral hyperintensity, decreased hippocampal volumetric capacity. Changes more expressed on right

Abnormal structure of left hippocampus

Cavernous angiomas (cavernomas, cavernous hemangioma) Dilated venous sinusoids without intervening brain parenchyma consist of large sinusoidal vascular spaces densely clustered to form a compact mass that is surrounded by fibrous strands or septa, not normal neural tissue Symtoms seizures or hemorrhage

(A) The T1-weighted image shows an irregularly enhancing mass of various signal intensities in the medial right temporal lobe. (B) On a long TR image, a hypointense ring can be seen around the malformation as a result of hemosiderin deposition in the surrounding brain. (C) A postoperative T1 image shows a gross total resection of the lesion.

EEG . spike and waves discharges

Infections of CNS- bacterial meningitis

Bacterial organisms gain acces to the meninges primarily by the blood-borne route, spread from contiguous site of infection (paranasal sinusitis, mastoiditis, otitis, osteomyelitis) or direct implantation (penetration injury, surgery, lumbar pucture). Invasive type of meningococcus C (Neisseria meningitidis) - meningococcal septicaemia Course could be dramatic, its medical emergency. High fever, altered mental state and unconsciousness delirium meningeal sepsis - disseminated intravascular coagulation - DIC with petechiae, purpura, bleeding into the skin

Bacterial meningitis CSF - cloudy, white cells count increased (polymorphonuclear leukocytes, low glucose) Antibiotics administered early in propriate doses Rapid test - latex agglutination detect specific bacterial antigens, more rapid than cultivation Mortality up to 20-30%, residual sequelae: epilepsy, deafness, hydrocefalus, mental retardation, neurological deficit.

Tuberculous meningitis - basilar

subacute, mental changes, altered mental state, cranial nerves, nystagmus, neck stiffness CSF - low glucose, low chlorid. Start the treatment early, before culture results, which may take up to 6 weeks Risk of spinal block, vasculitis, hydrocephalus Fungal infections - clinical symptoms may be minimal, infection ondolent, mainly in imunocompromised patients, AIDS Incluide all: mild infection, chronic, subacute meningitis, acute, meningoencephalitis, brain absces Brain abscess - combination of signs of infection, neurological deficit, raised intracranial pressure. CT scan Risk: chronic infections, bronchiectasis, pulmonary abscess, bacterial endocarditis, congenital cyanotic heart disease

Viral - aseptic meningitis

low grade fever, headache, neck stiffness. Virus - rise in the specific serum and CSF antibody titer. Lyme disease CSF - mononucler cout cells Spontaneous recovery within 1-2 weeks, only symptomatic treatment Viral encephalitis in conjunction with other viral illness - mumps, measles (now rare) Tick-borne encephalitis the most common in this country. Lyme disease Herpes simplex encephalitis - particularly frontal and temporal lobe, altered mental state. MR, CSF, early treatment with Acyclovir is a must ! High mortality

Lyme disease / Lyme borreliosis

localised infection - erythema migrans generalised - nervous system

Multiple sclerosis
inflammatory, demyelinating disease of the CNS. MS lesions, characterized by perivascular infiltration of monocytes and lymphocytes, appear as indurated areas in pathologic specimens; hence, the term sclerosis in plaques. MS is a dynamic disease, with almost constant lesion formation and a progressive clinical course leading to physical disability can present in different forms, such as relapsing remitting, relapsing progressive secondary progressive and less common primary progressive. patients who improve after acute attacks have relapsing remitting MS (RRMS). However, during the natural course of RRMS, approximately 75-85% of patients enter a stage referred to as secondary progressive MS (SPMS).

Multiple sclerosis - recurrent attacks of focal or

multifocal neurologic dysfunction within CNS. The clinical picture is determined by the location of foci of demyelination within CNS. Classic features: impaired vision, nystagmus, dysartria, sensory symptoms (positive - paresthesias), weakness, paralysis, instability, ataxia, bladder dysfunction.

CSF with oligoclonal bands (7, 9, 11) in electrophoresis with isoelectric focusing

MS treatment

1. MS relaps (attack) - high doses of intravenous methylprednisolone (3-5 g) 2. DMD (disease modyfiing drugs)

Interferons - derived from human cytokines which help regulate the immune system.
Interferon beta-1a (Avonex , Rebif), beta-1b (Betaferon).

Glatiramer acetate (Copaxone) - a synthetic medication stimulates T cells in the body's immune systm

Natalizumab (Tysabri).

3. Symptomatic

Nutritional - vitamin deficiency

Wernickes encephalopathy (alcoholic, following resection of stomach and small bowel) B1 deficiency. Global confusional-apathetic state, ocular motor signs, nystagmus, ataxia - affects stance and gait predominantly.
Administering dextrose in a thiamine-deficient state exacerbates the process of cell death by providing more substrate for biochemical pathways that lack sufficient amounts of coenzymes

Alcoholic cerebellar degeneration Alcoholic polyneuropathy - B1 deficiency + toxic Subacute combined degeneration of the spinal cord B12 deficiency, pernicious anemia + polyneuropathy, optic neuritis, mental signs

Neurodegenerative diseases
Neurodegeneration is the term for the progressive loss of structure or function of neurons, including death of neurons.

Dementia
Progressive deterioration of intellectual and cognitive functions, behavior and personality as a consequence of diffuse disease of the cerebral hemispheres (diffuse but not the whole). Cognition includes orientation, level of awareness, thought, attention, language, judgment, and insight into illness. Investigation: identification of dementia, features of dementia (must be distinguished from pseudodementia - depression). Another is mental retardation - insult to the brain early in the life which has prevented the development of normal intelligence. Mini mental state and others. Causes of dementia: common and uncommon, treatable and untreatable

Mental function subparts

Level of responsivity, alertness (consciousness) Motivation, spontaneity, mood Thought and behavior patterns Attention (including directed attention and neglect) Orientation Memory A. learning B. retention and immediate recall C. delayed recall Calculation Abstract reasoning Praxis Spatial skills (visuospatial and right-left orientation)

Alzheimer's disease
progressive cognitive deterioration, is characterized by senile plaques, -amyloid deposits, and neurofibrillary tangles in the cerebral cortex and subcortical gray matter. Dementia established clinically and documented by a formal mental status examination Deficits in 2 areas of cognition Gradual onset and progressive worsening of memory and other cognitive functions No disturbance of consciousness Onset after age 40, most often after age 65 No systemic or brain disorders that could account for the progressive deficits in memory and cognition

Alzheimers disease

Amyloid senile plaques - neuritic plaques) and neurofibrillary tangles

pathological accumulation of beta-amyloid, intracelullary abnormal fosforylation of tauprotein

PET with special radioligand (PIB - Pittsburgh), which selectively binds amyloidal plaques. Maximum frontally, though the maximal metabolic failure (using FDG - 18F-fluorodeoxyglucose) was observed parietally.

Vascular dementia is acute or chronic cognitive

deterioration due to diffuse or focal cerebral infarction that is most often related to cerebrovascular disease.

Vascular dementia Symptoms and signs are similar to those of other dementias. However, because infarction is the cause, vascular dementia tends to progress in discrete steps; each episode is accompanied by intellectual decline, sometimes followed by modest recovery. As the disease progresses, focal neurologic deficits often develop: Exaggeration of DTR, extensor plantar response Gait abnormalities Weakness of an extremity, hemiparesis Pseudobulbar palsy with pathologic laughing and crying Other signs of extrapyramidal dysfunction

Lewy body dementia

chronic cognitive deterioration characterized by cellular inclusions called Lewy bodies in the cytoplasm of cortical neurons. Extrapyramidal symptoms occur. However, unlike in Parkinson's disease, in Lewy body dementia, cognitive and extrapyramidal symptoms usually begin within 1 yr of each other. Also the extrapyramidal symptoms differ from those of Parkinson's disease: In Lewy body dementia, tremor does not occur early, rigidity of axial muscles with gait instability occurs early, and deficits tend to be symmetric. Repeated falls are common. Fluctuating cognitive function is a relatively specific feature of Lewy body dementia. Visual hallucinations are common

Frontotemporal dementias
FTD affects personality, behavior, and usually language function more and memory less than does AD. Development of behavioral or cognitive deficits manifested by the following: Early and progressive change in personality, characterized by difficulty modulating behavior, often resulting in inappropriate responses or activities, or Early and progressive change in language, characterized by problems with expression of language or severe naming difficulty and problems with word meaning.

Mild Cognitive Impairment

The most studied form of MCI is that of isolated recent memory loss, or amnestic MCI. Diagnostic criteria are as follows: a subjective or objective impairment in recent memory, relatively preserved cognitive functions in other domains, and presence of normally performed everyday activities of daily living. Persons who present with amnestic MCI have an increased risk of developing diagnosable AD at rates of 12% to 15% per year, in contrast with 1% to 2% per year in age-matched normal subjects

Parkinson's disease
idiopathic, slowly progressive, degenerative CNS disorder characterized by resting tremor, muscular rigidity, slow and decreased movement, and postural instability. In most patients, the disease begins insidiously. A resting tremor of one hand is often the first symptom. The tremor is slow and coarse, maximal at rest, lessening during movement, absent during sleep, amplitude increased by emotional tension or fatigue Rigidity develops without tremor in many patients. Slow movements (bradykinesia) are typical as rigidity progresses. Movement also becomes decreased (hypokinesia) and difficult to initiate (akinesia).
Parkinsonism refers to symptoms similar to PD but caused by another condition.

Parkinsons disease PET impaired fluoro-dopa uptake in the region of the caudate and putamen

loss of nigro-striatal neurons and dopamine decifiency

Secondary parkinsonism: vascular, traumatic, drug or toxin induced (carbon monoxide, manganese), postencephalic

Dystonias
sustained involuntary muscle contractions, often distorting body posture. Dystonias can be primary or secondary, and they can be generalized, focal, or segmental. Diagnosis is clinical Focal dystonias - affect a single body part. Torticollis cervical dystonie - begins with a pulling sensation followed by sustained torsion and deviation of the head and neck. The cause is often unknown but, in some cases, is probably genetic. In early stages, it can be voluntarily overcome. Patients may discover sensory or tactile tricks that make the spasm stop, such as touching the face on the side contralateral to the deviation. Occupational dystonia consists of focal dystonic spasms initiated by performing skilled acts (eg, writer's or typist's cramp, the yips in golfers). Spasmodic (spastic) dystonia consists of a strained, hoarse, or creaky voice due to abnormal involuntary contraction of laryngeal muscles. Segmental dystonias: These dystonias affect 2 contiguous body parts. Meige's disease (blepharospasm-oromandibular dystonia) consists of involuntary blinking, jaw grinding, and grimacing, usually beginning in late middle age. It may mimic the buccal-lingual-facial movements of tardive dyskinesia.

Huntington's disease
results from a gene mutation causing abnormal repetition of the DNA sequence CAG that codes for the amino acid glutamine. The resulting gene product, a large protein called huntingtin. The more CAG repetitions, the earlier the disease begins and the more severe the effects. Symptoms and signs develop insidiously, starting at about age 35 to 50 but can develop before adulthood. Dementia or psychiatric disturbances (eg, depression, apathy, irritability, antisocial behavior) develop before or simultaneously with the movement disorder. Abnormal movements appear; they include myoclonic jerks or irregular movements of the extremities, a lilting gait (like a puppet's), facial grimacing, ataxia, and inability to sustain a motor act (motor impersistence) such as tongue protrusion. The disorder progresses, making walking impossible, swallowing difficult, and dementia severe. Most patients eventually require institutionalization. Death usually occurs 13 to 15 yr after symptoms begin. The cause is usually pneumonia or coronary artery disease Diagnosis is based on typical symptoms and signs plus a positive family history and is confirmed by genetic testing.

Parkinson's disease (PD)

Progressive neurodegenerative disorder associated with a loss of dopaminergic nigrostriatal neurons. Cardinal features include resting tremor, rigidity, bradykinesia, and postural instability. The initial symptoms of PD may be nonspecific and include fatigue, depression, constipation, and sleep problems. Some pts experience a subtle decrease in dexterity and may notice a lack of coordination with activities such as playing golf or dressing. Some pts complain of aching or tightness in the calf or shoulder region

Parkinsons disease PET impaired fluoro-dopa uptake in the region of the caudate and putamen

loss of nigro-striatal neurons and dopamine decifiency

Secondary parkinsonism: vascular, traumatic, drug or toxin induced (carbon monoxide, manganese), postencephalic

Essential tremor
The most common movement disorder. It is a syndrome characterized by a slowly progressive postural and/or kinetic tremor, usually affecting both upper extremities. Tremor also may affect the head, voice, and lips. Primidone and propranolol are the cornerstones of maintenance medical therapy for essential tremor. These medications provide benefit in reducing tremor amplitude in approximately 75% of patients. Tremor reduction by alcohol, benzodiazepines, particularly clonazepam and alprazolam, gabapentin, topiramate

Huntington disease (HD)

Incurable, adult-onset, autosomal dominant inherited disorder associated with cell loss within a specific subset of neurons in the basal ganglia and cortex. Characteristic features include involuntary movements, dementia, and behavioral changes. The genetic basis of HD is the expansion of a cysteineadenosine-guanine (CAG) repeat encoding a polyglutamine tract in the N -terminus of the protein product called huntingtin Choreoathetotic movement disorders could be present in developmental disabilities (in mental retardation, cerebral palsy) due to brain malformations, asphyxia in the infants with diffuse brain damage.

Motor neurone disease - amyotrofic lateral sclerosis

Fibers are grouped in bundles called fascicles, each fascicle being enveloped by perineurium, several fascicles are collected, outer sheath of the nerve is epineurium.

Bell's palsy - the

lid remains open (lagophthalmos), and on attempted closure the globe turns up and out
(Bell's phenomenon)

Upper motor neuron disorders with unilateral facial paresis affect the voluntary movements of the lower face

Synkinesis - aberrant reinnervation, mass action

Accesory nerve - surgical injury during biopsy of lymph nodes in the posterior triangle of the neck

Brachial plexus Injury supra and infraclavicular

Root avulsion - it consists of tearing of the dorsal and ventral roots of one or more spinal nerves from the spinal cord Upper trunk (shoulder), lower trunk - hand Acute brachial plexus neuropathy (neuralgic amyotrophy, idiopathic). Malignant invasion

Long thoracic nerve - serratus anterior muscle weakness, winging of the scapula

radial nerve

compression at the spiral groove

extension in interphalangeal jonts is controlled by interossei and lumbrical muscles = median and ulnar nerves

ulnar nerve

Interossei - adduction of dig. min, abduction, wasting of first interossei, flexor dig. prof. (flexion of terminal phalanx)

median nerve

Median - predominantly sensory signs, wasting of part of the thenar muscles, weakness of opponens pollicis

Lateral cutaneous nerve of the thigh meralgia paresthetica

Polyneuropathies

Demyelination myelinopathy Causes: Inflammatoryimmunological - GBs (AIDP), CIDP, Type I HMSN Axonal - axonopathy

Diabetic neuropathy
Symmetrical polyneuropathies Distal sensory or sensorimotor polyneuropathy Small fiber neuropathy Autonomic neuropathy Large fiber neuropathy Asymmetrical neuropathies Cranial neuropathy (single or multiple) Truncal neuropathy (thoracic radiculopathy) Limb mononeuropathy (single or multiple) Lumbosacral radiculoplexopathy (asymmetrical proximal motor neuropathy) - Proximal diabetic amyotrophy Entrapment neuropathy Combinations Diabetic neuropathic cachexia acute painful neuropathy

Hereditary polyneuropathy - Charcot-Marie-Tooth disease

foot abnormalities such as hammer toes or high arches CMT1, CMT2, CMTX

myopathic climbing

Myopathies
Muscular dystrophies are genetic myopathies, usually caused by a disturbance of a structural protein Myositis implies an inflammatory disorder and is usually reserved for disorders in which the muscle histology shows an inflammatory response Endocrine myopathy Myotonias are diseases in which the normal contractile process is distorted by the occurrence of involuntary, persistent muscle activity

Muscular dystrophies

spondylosis, osteophyte formation (outgrowths), spondylotic spurs, degenerative joint disease

(hypertrophic facet joints - superior and inferior articular facet, narrowing from osteophytic encroachment)

Slip occurs due to degenerative disease of the facet joints

Intervertebral disk herniation

tears in anulus fibrosus and nucleus pulposus herniates displacement into the spinal canal or posterolaterally toward the intervertebral foramen. This may produce local spinal pain and tenderness, paravertebral muscle spasm. If the disc prolapse is more extensive, nerve root compression will result, causing radicular syndromes related to the distribution of the root compression (predominantly space L4/5, L5/S1, less frequently L3/4)

Developmental stenosis of the vertebral canal

Cervical spondylotic myelopathy

degenerative disease of the
spine involving the lower and midcervical vertebrae, it narrows the spinal canal and intervertebral foramina and causes progressive injury of the spinal cord, roots, or both chronic affection due to deg. changes, spondylosis and disk herniation dynamic factors contribute, perhaps vascular changes as well

Cervical spondylotic myelopathy

slow development, weakness and stiffness of legs, unstable gait, clumsiness or weakness of a hand pain in the neck, shoulders, and upper arms, numbness and paresthesias in hands atrophy of intrinsic hand muscles, part. first. dorsal interosseal, supra and infraspinous increased reflexes, part. triceps and finger flexor, Hoffmann's sign positive spastic legs from a compressive myelopathy, most often presents as a complaint of weakness of a leg and slight unsteadiness of gait. Impaired vibratory sensation and diminished position sense in the toes and feet

Disc herniations: midline (dorsal),

posteromedial, posterolateral, far lateral, foraminal, ev. extraforaminal

straight leg raising Lasegues sign

Lumbar spinal stenosis Neurospinous claudication cramp-like pain or aching pain induced by standing or walking, felt in buttock, thigh or leg and relieved by rest, particularly in bending posture.
history is crucial, all signs could be normal. often low back pain and radicular pains

HEADACHE

- the primary or secondary

establishing a diagnosis depends almost entirely on taking an accurate patient history. Age of onset, duration of complaint, onset - time, sudden, subacute, course, frequency and duration of each headache, site. Headache danger signals: sudden onset of new, severe headache, progressively worsening headache, first ever headache after the age of 50 years, associated symptoms (drowsiness, confusion, memory loss), any abnormality on neurological or general physical examination. The most commom - TENSION-TYPE HEADACHE closely related to stress. Can progress to chronic daily headache

MIGRAINE
with or withour aura = focal neurologic (cerebral) symptoms or signs usually preceding, but sometimes accompanying or even following the onset of headache. Typically visual disturbances - flashing lights, shimmering zig-zag lines, visual field lesion, scotoma. Sensory symptoms - usually hemitype, paresthesias (pins and needles), even hemiparesis and dysphasia

Migraine without aura

1. 2. 3. 4. 1. 2. Headache attacks lasting 4-72 hours (untreated or unsuccessfully treated) Headache has at least 2 of the following characteristics: unilateral location pulsating quality moderate or severe pain intensity aggravation by or causing avoidance of routine physical activity (eg, walking or climbing stairs) D. During headache at least 1 of the following: nausea and/or vomiting photophobia and phonophobia

Typical aura with migraine headache

At least 1 of the following: 1. fully reversible visual symptoms including positive features (eg, flickering lights, spots or lines) and/or negative features (ie, loss of vision) 2. fully reversible sensory symptoms including positive features (ie, pins and needles) and/or negative features (ie, numbness) 3. fully reversible dysphasic speech disturbance At least two of the following: 1. homonymous visual symptoms and/or unilateral sensory symptoms 2. at least one aura symptom develops gradually over u5 minutes and/or different aura symptoms occur in succession over u5 minutes 3. each symptom lasts u5 and e60 minutes

Attack of migraine migrain

Normal Normal condition Headache Norma Normal cond.
Chu k jdlu Bd n/spnek Tolerance sv tla nava
tolerance potravy

Chu k jdlu

Bd n/spnek
Pocit povznesen osti nebo skl enosti Tolerance sv tla

Hluk Zpach
Rovnovha tekutin

Hluk Zpach

Diurza

Rovnovha tekutin

Normln stav

I Prodromy

II Aura

III

IV

Bolest hlavy Zotaven

V Postdromy

Tension-type headache (TTH)

Headache lasting from 30 minutes to 7 days 1. bilateral location 2. pressing/tightening (non-pulsating) quality 3. mild or moderate intensity 4. not aggravated by routine physical activity such as walking or climbing stairs Chronic TTH - headache occurring on u15 days/month on average for >3 months (u180 days/year) Headache lasts hours or may be continuous

Cluster headache
Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 minutes if untreated Headache is accompanied by at least 1 of the following: ipsilateral conjunctival injection and/or lacrimation ipsilateral nasal congestion and/or rhinorrhoea ipsilateral eyelid oedema ipsilateral forehead and facial sweating ipsilateral miosis and/or ptosis a sense of restlessness or agitation

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

Giant cell (temporal) arteritis

Occurence over 60 yrs, headache - new event, sometimes fatigue, myalgia, subfebrile, pain during chewing (jaw claudication). Elevated ESR, CRP. Risk of complications, part. ischemic optic neuropathy and even stroke. Systemic symptoms - weight loss, muscle aches. Relationship with polymyalgia rheumatica

Trigeminal neuralgia
Paroxysmal attacks of pain lasting from a fraction of 1 sec to 2 min, affecting one or more divisions of the trigeminal nerve and fulfilling criteria B and C B. Pain has u1 of the following characteristics: 1. intense, sharp, superficial or stabbing 2. precipitated from trigger areas or by trigger factors C. Attacks are stereotyped in the individual patient D. There is no clinically evident neurological deficit

Classical or Symptomatic

Trigeminal neuralgia

Microvascular Decompression Radiosurgery using the Gamma Knife

Persistent idiopathic facial pain [Atypical facial pain]

Pain in the face, present daily and persisting for all or most of the day Pain is confined at onset to a limited area on one side of the face, and is deep and poorly localised Pain is not associated with sensory loss or other physical signs Investigations including X-ray of face and jaws do not demonstrate any relevant abnormality

Herpes zoster, shingles

Mental Disorders - neuroses three broad categories

1. Anxiety disorders (which include panic

states, with and without agoraphobia, and the phobic and obsessive-compulsive neuroses)

2. Somatoform disorders (comprising

hypochondriasis)

3. Dissociative disorders.
Psychotic Disorders - the major symptoms are delusions and hallucinations. Delusions are false beliefs that significantly hinder a person's ability to function (e.g. believing that people are trying to hurt you when there is no evidence of this, or believing that you are somebody else). Hallucinations are false perceptions.

Anxiety Disorders
Categorize a large number of disorders where the primary feature is abnormal or inappropriate anxiety. Everybody has experienced anxiety. Think about the last time a loud noise frightened you and remember the feelings inside your body. Chances are you experienced an increased heart rate, tensed muscles, and perhaps an acute sense of focus as you tried to determine the source of the noise. These are all symptoms of anxiety. These symptoms become a problem when they occur without any recognizable stimulus or when the stimulus does not warrant such a reaction. In other words, inappropriate anxiety is when a person's heart races, breathing increases, and muscles tense without any reason for them to do so.

Psychogenic Disorders
Anxiety is a complex feeling of apprehension, fear, and worry often accompanied by by physical sensations such as heart palpitations, nausea, chest pain, shortness of breath, stomach aches, or headache Anxiety is a normal reaction to stress. It helps one deal with a tense situation in the office, study harder for an exam, keep focused on an important speech. But when anxiety becomes an excessive, irrational dread of everyday situations, it has become a disabling disorder. Chronic, exaggerated worry, tension, and irritability that appear to have no cause or are more intense than the situation warrants. A phobia is a type of anxiety disorder, an irrational, intense, persistent fear of certain situations, activities, things, or persons. The main symptom of this disorder is the excessive, unreasonable desire to avoid the feared subject

Anxiety disorders
Anxiety is defined as an unpleasant emotional state for which the cause is either not readily identified or perceived to be uncontrollable or unavoidable Fear is an emotional and physiological response to a recognized external threat. The term anxiety disorder, however, includes fears as well as anxieties. Indeed, phobias (fears which are "persistent or irrational") constitute the majority of anxiety disorder cases.

Psychogenic - Anxiety Disorders

categorize a large number of disorders where the primary feature is abnormal or inappropriate anxiety Acute stress disorder is a result of a traumatic event in which the person experienced or witnessed an event that involved threatened or actual serious injury or death and responded with intense fear and helplessness. Symptoms include dissociative symptoms such as numbing, detachment, a reduction in awareness of the surroundings, derealization, or depersonalization; re-experiencing of the trauma, avoidance of associated stimuli, and significant anxiety, including irritability, poor concentration, difficulty sleeping, and restlessness. The symptoms must be present for a minimum of two days and a maximum of four weeks and must occur within four weeks of the traumatic event for a diagnosis to be made

Anxiety Disorders
Panic Disorder is characterized by sudden attacks of intense fear or anxiety, usually associated with numerous physical symptoms such as heart palpitations, rapid breathing or shortness of breath, blurred vision, dizziness, and racing thoughts. Often these symptoms are thought to be a heart attack by the individual, and many cases are diagnosed in hospital emergency rooms. Generalized Anxiety Disorder typically develops over a period of time and may not be noticed until it is significant enough to cause problems with functioning. GAD is evidenced by general feelings of anxiety such as mild heart palpitations, dizziness, and excessive worry. The symptoms are difficult to control for the individual and are not related to a specific event and are not as severe as those found with Panic Disorder.

Phobias
Symptoms include either extreme anxiety and fear associated with the object or situation or avoidance. To be diagnosed, the symptoms must be disruptive to everyday functioning Agoraphobia is the fear about being in places where escape might be difficult or embarrassing or in which help may not be available should a panic attack develop Social phobia - an intense fear of negative public scrutiny or of public embarrassment or humiliation. Can be specific to particular social situations (public speaking) or, more typically, is experienced in most (or all) social interactions. Often manfiests specific physical symptoms, including blushing, sweating, and difficulty speaking. Specific phobias - fear of a single specific trigger such as spiders, snakes, elevators (claustrophobia - fear of enclosed spaces), catching a specific illness

Somatoform Disorders
symptoms suggest a medical condition but where no medical condition can be found by a physician. body dysmorphic disorder, conversion disorder, hypochondriasis disorder, pain disorder, somatization disorder Somatization Disorder - long-term (chronic) condition in which a person has physical symptoms that are caused by psychological problems, and no physical problem can be found history of physical complaints prior to age 30 which occur over a period of several years, multiple and chronic complaints, multiple pain symptoms, gastrointestinal symptoms (nausea and indigestion)

Some of the many symptoms that can occur with somatization disorder include:
Abdominal pain Amnesia Back pain Bloating Chest pain Diarrhea Difficulty swallowing Dizziness Excessive menstrual bleeding Headaches Impotence Irregular menstruation Joint pain Nausea Pain during intercourse Pain during urination Painful menstruation Pain in the legs or arms Palpitations Paralysis or muscle weakness Sexual apathy Shortness of breath Vision changes Vomiting

Somatoform Disorders
Conversion disorders (unconscious conflicts
cause the conversion into physical symptoms)

Somatoform disorder characterized by a sudden loss of neurological function, usually in the context of a severe stressor. Specific characteristics of conversion disorder include loss of voluntary motor or sensory function, eg, inability to walk, sudden blindness, pseudoseizure, paralysis, becoming mute