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brain nearly 2%

of weight of the

body

consumption 15%

of cardiac output

15-20% totaloxygen

Supply of O2 and glucose is maintained by stable CBF (range 40-

60 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 keepblood flow constant in response to decreased or increased

perfusion pressure .

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Two essential formulas of cerebral vascular regulation

CPPCBF = --------------- CPP = MAP - ICP

CVR

CBF cerebral blood flow ICP intracranial pressureCPP - cerebral perfusion pressure

MAP mean arterial pressureCVR cerebrovascular resistance

cerebral vascular resistance resistance of cerebralvessels and rheological (flow) propertis of blood (viscosity,

erythrocyte blood count and rigidity etc.

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After cessation of blood supply following occlusion of a cerebralvessel, neuronal cell death occurs in the core of the infarcted areawithin few minutes. The area surrounding the core, called theischaemicpenumbra, contains functionally impaired but still viablebrain tissue supplied from collateral vessels. This area may be

transformed into infarction due to secondary neuronal damage

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1. unaffected area2. benign oligemia3. ischemic penumbra4. ischemic core (infarction)

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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, accumulationof lipids, due to activity of LDL makrophageschange to foam cells and arise atheroscleroticplaques.

In intimal sclerotic plaques further changes couldarise as calcifications, ulcerations, necrosis andbleeding, continue further adhesion andaggregation of thrombocytes and thrombusformation

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Atherosclerosis

the transportation of cholesterol by LDL(lipoprotein) to peripheral sites. Monocytesthen adhere to endothelium injured by localaccumulation of cholesterol and migrate to

subendothelial area as foam cells. Smoothmuscle cells proliferate and fibrous plaquesdevelop, with progressive stenosis of thevascular lumen as the process continues.

Increased platelet activation and fibrinformation, decreased fibrinolytic andanticoagulant activity

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Atherosclerotic risk factorsAtherosclerotic 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)

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Risk factorsRisk factors

II. modifiable cigarette smoking (contributes to endothelial

dysfunction, decrease of HDL and increase LDL,incr. fibrinogenaemia and changed reactivity ofplatelets) . 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

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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 atleast in 2 from 3 measures in minimal 2 visits

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Risk factorsRisk factors

II. modifiable obesity only supporting factor, often associated

with hypertension, diabetes, increased cholesterol

and lipids and lack of physical activity.

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Risk factorsRisk 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

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Risk factorsRisk factors

IV. associate

lack of physical activity. Physical activity decreasesBP, increases HDL cholesterol level, decreases LDLcholesterol and improve glucose tolerance.

excessive alcohol consumption is a risk factor,moderate consumption (1224 g/day) could beprotective. Recommended daily doses: 1 l 10o beeror 3 dcl wine or weak 1 dcl 40% spirits.

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The major subtypes of strokeThe major subtypes of stroke

40%

20%

12%

8%

16%4%

Cardioembolicstroke

Small vessel disease

Large vesseldisease

Intracerebralhemorrhage

Other

SAH

Small vessel occlusion - lacunar infarction - lacunes are small cavities mainly

deep in the brain - basal ganglia (internal capsule, thalamus), brain stem.

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Risk factors of strokeRisk factors of stroke

Heart diseaseHeart disease part. coronary heart disease,congestive heart failure, atrial fibrillation and valvularheart disease. Atrial fibrillation increases risk of strokeup to 6

Significant risk factor isprior stroke andtransient ischemic attack (TIA)

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STROKESTROKE

A

n acute neurologic injury occurring as a result ofvascular 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 leadingto death without any other etiology other than a

vascular abnormality

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Transient ischemic attack (TIA)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 structuralchanges of brain

Amaurosis fugax (transientocclusion of the ophtalmic artery)

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major territories

Large vessel occlusion -territorial infarcts

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Brainstem crossed or alternating syndromes

ipsilateralycranial nerve lesion,Horners sy, gaze palsy(horizontal to the side

of lesion), cerebellarsigns

contralateraly

tract signs

(hemiparesis,hemihypestesia)

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Time to peak- TTPFlow perfusion

Blood volumeBlood volume

Perfusion CT before andafter thrombolytic therapy

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CT AG MIP reconstruction(maximum intensityprojection)

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Thrombolytictherapy

1) Intravenousrecombinant tissue

plasminogen

activator- rtPA

Intra-arterial

thrombolytictherapy

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Ultrasound - duplex scanning -noninvasive investigation canidentify occlusive/stenotic

vascular disease.

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Carotid Stenosis

Occlusion of the internalcarotid artery may beasymptomatic or acatastrophic infarctionmay result.

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CTA stenosis + plaquesMRA only stenosis

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Carotid surgery- endarterectomyendarterectomy

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Carotid angioplasty, withor without stenting

PTA - percutaneoustransluminal angioplastywith or without stenting

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

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Extracerebral subarachnoid

haemorrhage SAHaneurysms (berry, saccular)

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SAH - severeheadache, acute,nausea, vomiting,

loss ofconsciousness orepileptic seizure.Neck stiffnessdevelops after 3-12

hours. No focalsigns, bleeding isextracerebral

Complications -intracerebralhaematoma,rebleeding,reruprure, arterialspasms (ischaemia),hydrocephalus

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surgery (clipping, wrapping)

endovascular - coiling

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ArteriovenousArteriovenousmalformationmalformation AVMAVM --masses of dilated vesselsmasses of dilated vessels

Treatment surgery ortransvascular embolisation

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cavernoushemangiomas - clusterdilated vessels (veins) withoutinterposed glial or nervous

tissue; angiographically occult

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Intracranial tumors

primary arise from glial cells (50-60%gliomas - glioblastoma, astrocytoma,oligodendroglioma), ependymal cells

(ependymoma), supporting tissue (meningioma,

schwannoma), in childhood from more primitivecells (meduloblastoma)

secondary metastatic 20-40%

When do we suggest possibility of intracranial

tumor? Any patients with focal signs or raisedintracranial pressure (or epilepsy as well) should

be considered brain tumor.

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WHO Classification of CNS TumorsWHO Classification of CNS Tumors

I. 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,

neurofibromaIII. Meningeal tumors - meningioma

IV. Hematopoetic tumors - lymphoma

V. Germ Cell Tumors - germinoma, terratoma

VI. Cysts and Tumor-like LesionsVII. Tumors of the Sellar Region

VIII. Metastatic tumors

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Cerebellar hemangioblastoma (with a cystic cavity), occurs in themiddle-aged.

Medulloblastoma (primitive neuroectodermal tumor) - in

childhood, about 5 years. Arise in cerebellar vermis and extendinto 4th ventricle. All are highly malignant and spread throughoutthe CSF pathways. Truncal and gait ataxia, signs and symptomsof raised intracranial pressure, sometimes particularly vomitingwithout headache!

Another tumor in childhood is cerebellar astrocytoma - usually lowgrade and benign

Pituitary adenoma - classification based on the hormone typesecreted: GH secreting tumor, prolactinoma - hyperprolactinemiacan 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 meningitiswith reduction in CSF glucose. Malignant cells

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Tumors of spine and spinal cord extramedullary primary (meningioma,

schwannoma, less common angioma and AVmalformation

metastatic secondary, in particular cancer ofbreast, lung, prostate, kidney, gynecological

cancer, lymphogranuloma and leukaemicinfiltrates.

intramedullary - mostly ependymoma and glioma Symptoms and signs - pain, particularly in

extramedullary processes, can be of radicularpattern and propagation in dermatome.Compressive radicular syndrome continue tocompressive spinal cord syndrome

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Craniocerebral injuries

Traumatic brain injury(TBI)

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Skull fractures Linear

Compound -comminution offragments

Depressed

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Skull Fractures Basilar skull fracture

Penetrating - dural tear,communication between theexternal environment andthe cranial cavity.

SAH and intracranialSAH and intracranialair (pneumocephalus)air (pneumocephalus)

temporalemporalfracturefracture

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Battles signracoon sign

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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 focalsignsClinical assessment: conscious level, pupil response, limb

weakness

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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 -usuallymultiple, on the site of the blow - coup or on

opposite site - contrecoup.

The clinical signs produced by contusions vary with

their location and size3. Diffuse axonal injury, white matter lesion.

Widespread acute disruption of axons.

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Contusion - directly underneath the point of impact(coup injury) or sudden deceleration results in an injuryto the brain on the opposite side (countercoup injury).

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Diffuseaxonal

injury

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2. Secondary brain damage

A. Traumatic intracranial hematomas1) Epidural hematoma

a skull fracture tearing the middle meningeal vessels

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2) Subdural hematoma- acute- subacute- chronic - hygroma

rupture bridgingveins from thecortical surface tothe venous sinuses.

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3) Traumatic subarachnoid hemorrhage -common in cerebral contusions

4) Intracerebral hematomas - severe injurieswith skull fractures, cerebral contusions,

lacerations and diffuse axonal injury

B. Cerebral edema with venousturgescence

C. Hypoxic-ischemic injury(encephalopathy )

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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

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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 spinethe most common in C5 a Th12

segmentalsymptoms andsigns

tractsymptoms andsigns

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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 damageoften produces a "flaccid paralysis", spinalshock - inhibition of all activity (reflex also)below the lesion.

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Traumatic spinal cord injury

Neurologic level of injury - Mostcaudal level at which both motor andsensory levels are intact,

Zone of partial preservation - Thisindex is used only when the injury iscomplete. All segments below theneurologic level of injury withpreservation of motor or sensoryfindings

Warning - bedsores

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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

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Key sensory testing T8 - 8th IS (midway between T6 and

T10)

T9 - 9th IS (midway between T8 andT10) T10 - 10th IS or umbilicus T11 - 11th IS (midway between T10

and T12) T12 - Midpoint of inguinal ligament

L1 - Half the distance between T12and 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)

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Key muscles - myotome

C5 - Elbow flexors (biceps, brachialis)

C6 - Wrist extensors (extensor carpi radialis longusand 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)

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Traumatic incomplete syndromesTraumatic incomplete syndromesCentral cord syndromeCentral cord syndrome often is

associated with a cervical region injuryleading to greater weakness in the upperlimbs than in the lower limbs with sacralsensory sparing.BrownBrown--Squard syndromeSquard syndrome often isassociated with a hemisection lesion of thecord, causing a relatively greater ipsilateralproprioceptive and motor loss withcontralateral loss of sensitivity to pain andtemperature.

Anterior cord syndromeAnterior cord syndrome often isassociated with a lesion causing variableloss of motor function and sensitivity topain and temperature, while proprioceptionis preserved.

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Traumatic incomplete syndromesTraumatic incomplete syndromesConus medullaris syndrome is associated with

injury to the sacral cord and lumbar nerveroots leading to areflexic bladder, bowel, andlower limbs, while the sacral segmentsoccasionally may show preserved reflexes (eg.

bulbocavernosus and micturition reflexes).Cauda equina syndrome is due to injury to the

lumbosacral nerve roots in the spinal canalleading to areflexic bladder, bowel, and lowerlimbs.

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Whiplash injury

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EpilepsySeizure, epileptic attack Definitionsphysiological occasional sudden

excessive rapid and local discharges of gray matter(neurons)

clinical intermittent, paroxysmal, stereotypeddisturbance of consciousness, behavior, emotion,

motor function, perception or sensation epileptic focus group of neurons with pathologicalelectrical activity - impaired resting membranepotential onto action membrane depolarization -hyperexcitability.

abnormal neuronal discharges with signs ofhyperautorytmicity (repetitive bursts of highpotentials) a hypersynchronism (neurons dischargeall at once).

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Essential classification and types of seizures

GENERALIZEDGENERALIZED PARTIAL/FOCALPARTIAL/FOCAL CONVULSIVEtonic or clonicconvulsions,myoclonic, loss ofconsciousness

SIMPLEno alteration ofconsciousnessfocal motor or sensorysigns, adversiveseizures

NONCONVULSIVEabsences (petit mal),atonic, akinetic, disorder ofconsciousness

COMPLEXepisodic changes inbehavior, disorder ofconsciousness,automatisms

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Complex partial seizures

episodic changes in behaviour in which anindividual loses conscious contact with theenvironment.

Psychomotor or temporal lobe epilepsy. Gustatory(taste) or olfactory (smell) hallucinations (as aburning rubber), lip smacking (mlaskn),swallowing, walking aimlessly - automatisms.

Memory disturbances - deja vu (currentexperience has happened before), jamais vu

(feeling unfamiliarity), dreamy states. Visceraldisturbances (epigastric fullness, nausea, pallor),affective disturbance (displeasure, fear).

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Seizure, epileptic attack Theprodrome 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 originwithin the brain.

The ictus refers to the attack or seizure itself. Thepostictalperiodrefers 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 hasrecurrent seizures due to a chronic, underlying process.

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

Bilateral hyperintensity, decreasedhippocampal volumetric capacity.

Changes more expressed on right

Abnormal structureAbnormal structureof left hippocampusof left hippocampus

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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

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EEG . spike and wavesdischarges

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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 symptomatictreatment

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 andtemporal lobe, altered mental state. MR, CSF, earlytreatment with Acyclovir is a must ! High mortality

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M lti l l i

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Multiple sclerosis inflammatory, demyelinating disease of the CNS. MS

lesions, characterized by perivascular infiltration ofmonocytes and lymphocytes, appear as induratedareas in pathologic specimens; hence, the termsclerosis in plaques.

MS is a dynamic disease, with almost constant lesionformation and a progressive clinical course leading tophysical disability

can present in different forms, such as relapsingremitting, relapsing progressive secondary progressiveand less common primary progressive.

patients who improve after acute attacks have relapsingremitting MS (RRMS). However, during the naturalcourse of RRMS, approximately 75-85% of patientsenter a stage referred to as secondary progressive MS(SPMS).

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CSF with oligoclonal bands (7, 9, 11) in

electrophoresis with isoelectric focusing

MS

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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

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Neurodegenerative diseases

Neurodegeneration is the term for theprogressive loss of structure or function of

neurons, including death of neurons.

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Mental function subparts

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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 immediaterecall C. delayed recall Calculation Abstract reasoning

Praxis Spatial skills (visuospatial and right-left orientation)

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Al h i ' di

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Alzheimer's disease progressive cognitive deterioration, is characterized by

senile plaques, -amyloid deposits, and neurofibrillarytangles in the cerebral cortex and subcortical graymatter.

Dementia established clinically and documented by a

formal mental status examination Deficits in 2 areas of cognition

Gradual onset and progressive worsening of memoryand other cognitive functions

No disturbance of consciousness Onset after age 40, most often after age 65

No systemic or brain disorders that could account forthe progressive deficits in memory and cognition

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PET with special radioligand (PIB - Pittsburgh), which selectivelybinds amyloidal plaques. Maximum frontally, though the maximalmetabolic failure (using FDG - 18F-fluorodeoxyglucose) wasobserved parietally.

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Lewy body dementia

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Lewy body dementia

chroniccognitive deterioration characterizedbycellular

inclusions calledLewybodies in the cytoplasm ofcorticalneurons.

Extrapyramidal symptoms occur. However, unlike inParkinson's disease, in Lewy body dementia, cognitiveand extrapyramidal symptoms usually begin within 1 yrof each other. Also the extrapyramidal symptoms differfrom those of Parkinson's disease: In Lewy bodydementia, tremor does not occur early, rigidity of axialmuscles 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 hallucinationsare common

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Mild Cognitive Impairment

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Mild Cognitive Impairment

The most studied form of MCI is that of

isolated recent memory loss, oramnestic MCI. Diagnostic criteria are as follows: a subjective

or objective impairment in recent memory,relatively preserved cognitive functions inother domains, and presence of normallyperformed everyday activities of daily living.Persons who present with amnestic MCI havean increased risk of developing diagnosableAD at rates of12% to 15% per year, in contrastwith 1% to 2% per year in age-matched normalsubjects

Parkinson's disease

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Parkinson s disease

idiopathic, slowly progressive, degenerative CNS

disorder characterized by resting tremor, muscularrigidity, slow and decreased movement, and posturalinstability.

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 rigidityprogresses. 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

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p p pthe region of the caudate and putamen

loss of nigro-striatal neurons anddopamine decifiency

Secondary parkinsonism: vascular, traumatic, drug ortoxin induced (carbon monoxide, manganese), post-encephalic

Dystonias

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y

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 Focaldystonias -affect a single body part.

Torticollis cervical dystonie - begins with a pulling sensation followed bysustained torsion and deviation of the head and neck. The cause is oftenunknown but, in some cases, is probably genetic. In early stages, it can bevoluntarily overcome. Patients may discover sensory or tactile tricks that

make the spasm stop, such as touching the face on the side contralateralto the deviation.

Occupational dystonia consists of focal dystonic spasms initiated byperforming skilled acts (eg, writer's or typist's cramp, the yips in golfers).

Spasmodic (spastic) dystonia consists of a strained, hoarse, or creakyvoice due to abnormal involuntary contraction of laryngeal muscles.

Segmentaldystonias: These dystonias affect 2 contiguous body parts.

Meige's disease (blepharospasm-oromandibular dystonia) consists ofinvoluntary blinking, jaw grinding, and grimacing, usually beginning inlate middle age. It may mimic the buccal-lingual-facial movements oftardive dyskinesia.

Huntington's disease

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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 CAGrepetitions, the earlier the disease begins and the more severe theeffects.

Symptoms and signs develop insidiously, starting at about age 35 to 50but can develop before adulthood. Dementia or psychiatric disturbances(eg, depression, apathy, irritability, antisocial behavior) develop before or

simultaneously with the movement disorder. Abnormal movementsappear; they include myoclonic jerks or irregular movements of theextremities, a lilting gait (like a puppet's), facial grimacing, ataxia, andinability to sustain a motor act (motor impersistence) such as tongueprotrusion.

The disorder progresses, making walking impossible, swallowing

difficult, and dementia severe. Most patients eventually requireinstitutionalization. Death usually occurs 13 to 15 yr after symptomsbegin. The cause is usually pneumonia or coronary artery disease

Diagnosis is based on typical symptoms and signs plus a positive familyhistory and is confirmed by genetic testing.

Parkinson's disease (PD)

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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 sleepproblems.

Some pts experience a subtle decrease in dexterity andmay notice a lack of coordination with activities suchas playing golf or dressing.

Some pts complain of aching or tightness in the calf orshoulder region

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Fibers are grouped inbundles called

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bundles calledfascicles, eachfascicle being

enveloped byperineurium, severalfascicles are collected,outer sheath of thenerve is epineurium.

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Longthoracicnerve

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- serratus anteriormuscle weakness,

winging of thescapula

radial nerve compression at the spiral groove

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

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Polyneuropathies

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Demyelination myelinopathy

Causes: Inflammatory-immunological - GBs (AIDP),CIDP, Type I HMSNAxonal - axonopathy

Diabetic neuropathy

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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

CombinationsDiabetic neuropathic cachexia acute painful neuropathy

Hereditary polyneuropathy

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y p y p y- Charcot-Marie-Tooth disease

foot abnormalities such as

hammer toes or high arches

CMT1, CMT2, CMTX

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spondylosis, osteophyteformation (outgrowths),spondylotic spurs,

degenerative joint disease(hypertrophic facet joints - superiorand inferior articular facet, narrowingfrom osteophytic encroachment) Slip occurs due to

degenerative disease ofthe facet joints

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Developmental stenosis of thevertebral canal

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Disc herniations: midline (dorsal),posteromedial, posterolateral, far lateral,

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poste o ed a , poste o ate a , a ate a ,foraminal, ev. extraforaminal

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MIGRAINE

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with or withour aura = focal neurologic

(cerebral) symptoms or signs usuallypreceding, but sometimes accompanying oreven following the onset of headache. Typicallyvisual disturbances - flashing lights,

shimmering zig-zag lines, visual field lesion,scotoma. Sensory symptoms - usuallyhemitype, paresthesias (pins and needles), evenhemiparesis and dysphasia

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Typical aura with migraine headache

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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, lossof vision)

2. fully reversible sensory symptoms including positive features (ie,pins and needles) and/or negative features (ie, numbness)

3. fully reversible dysphasic speech disturbanceAt least two of the following:

1. homonymous visual symptoms and/or unilateral sensorysymptoms

2. at least one aura symptom develops gradually overu5 minutes

and/or different aura symptoms occur in succession overu

5minutes

3. each symptom lasts u5 and e60 minutes

Attack ofAttack of migrmigraiainnee

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HeadacheHeadache

IIIIII IVIVBolest hlavy ZotavenBolest hlavy Zotaven

II IIIINormln stavNormln stav Prodromy AuraProdromy Aura

NormNormalal

conditioncondition

Chu kChu kjdlujdlu

Bdn/spnekBdn/spnek

Tolerance svtlaTolerance svtla

ZpachZpach

HlukHluk

RovnovhaRovnovhatekutintekutin

navanava

VVPostdromyPostdromy

Tolerance svtlaTolerance svtla

HlukHluk

ZpachZpach

Rovnovha tekutinRovnovha tekutin

PocitPocitpovznesenpovznesenosti neboosti nebosklenostisklenosti

DiurzaDiurza

Chu k jdluChu k jdlu

Bdn/spnekBdn/spnek

tolerance potravytolerance potravy

NormNormaall

cond.cond.

Tension-type headache (TTH)

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Headache lasting from 30 minutes to 7 days1. bilateral location2. pressing/tightening (non-pulsating) quality3. mild or moderate intensity

4. not aggravated by routine physical activitysuch as walking or climbing stairs

Chronic TTH - headache occurring on u15

days/month on average for >3 months (u18

0days/year) Headache lasts hours or may be continuous

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Trigeminal neuralgia MicrovascularDecompression

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p

Radiosurgery -

using theGamma Knife

Persistent idiopathic facial pain[Atypical facial pain]

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[Atypical facial pain]

Pain in the face, present daily and persistingfor 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 poorlylocalised

Pain is not associated with sensory loss orother physical signs

Investigations including X-ray of face andjaws do not demonstrate any relevantabnormality

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Anxiety Disorders

Categorize a large number of disorders where the

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Categorize a large number of disorders where theprimary feature is abnormal orinappropriate anxiety.

Everybody has experienced anxiety.Think about the last time a loud noise frightened youand remember the feelings inside your body. Chancesare you experienced an increased heart rate, tensed

muscles, and perhaps an acute sense of focus as youtried to determine the source of the noise. These are allsymptoms of anxiety.

These symptoms become a problem when they occurwithout any recognizable stimulus or when the stimulusdoes not warrant such a reaction. In other words,inappropriate anxiety is when a person's heart races,breathing increases, and muscles tense without anyreason for them to do so.

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Anxiety disorders

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Anxiety is defined as an unpleasant emotional

state for which the cause is either not readilyidentified or perceived to be uncontrollable orunavoidable

Fearis an emotional and physiologicalresponse to a recognized external threat. Theterm anxiety disorder, however, includes fearsas well as anxieties. Indeed, phobias (fearswhich are "persistent or irrational") constitutethe majority of anxiety disorder cases.

Psychogenic - Anxiety Disorders categorize a large number of disorders where the

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categorize a large number of disorders where theprimary feature is abnormal or inappropriate anxiety

Acute stress disorderis a result of a traumatic event inwhich the person experienced or witnessed an eventthat involved threatened or actual serious injury ordeath and responded with intense fear andhelplessness. Symptoms include dissociativesymptoms such as numbing, detachment, a reductionin awareness of the surroundings, derealization, ordepersonalization; re-experiencing of the trauma,avoidance of associated stimuli, and significant

anxiety, including irritability, poor concentration,difficulty sleeping, and restlessness. The symptomsmust be present for a minimum of two days and amaximum of four weeks and must occur within fourweeks of the traumatic event for a diagnosis to be made

Anxiety Disorders

Panic Disorder is characterized by sudden attacks of intense

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Panic Disorderis characterized by sudden attacks of intensefear or anxiety, usually associated with numerous physical

symptoms such as heart palpitations, rapid breathing orshortness of breath, blurred vision, dizziness, and racingthoughts. Often these symptoms are thought to be a heartattack by the individual, and many cases are diagnosed inhospital emergency rooms.

Generalized Anxiety Disordertypically develops over aperiod of time and may not be noticed until it is significantenough to cause problems with functioning.

GAD is evidenced by general feelings of anxiety such as mildheart palpitations, dizziness, and excessive worry. The

symptoms are difficult to control for the individual and arenot related to a specific event and are not as severe as thosefound with Panic Disorder.

Phobias Symptoms include either extreme anxiety and fear

i t d ith th bj t it ti id T b

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associated with the object or situation or avoidance. To be

diagnosed, the symptoms must be disruptive to everydayfunctioning Agoraphobia is the fear about being in places where escape

might be difficult or embarrassing or in which help may notbe available should a panic attack develop

Social phobia - an intense fear of negative public scrutiny orof public embarrassment or humiliation. Can be specific toparticular social situations (public speaking) or, moretypically, 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 enclosedspaces), catching a specific illness

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Some of the many symptoms that can occurwith somatization disorder include:

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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

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