K13 Extrapyramydal Disorder

7/30/2019 K13 Extrapyramydal Disorder

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EXTRAPYRAMIDAL DISORDERS

= Movement disorders

= Degenerative disease

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4 types of movements:

Voluntary movement

Semivoluntary movement (=unvoluntary)

Involuntary movement

Automatic movement

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Movement disorders

= Extrapyramidal disorder

Disorder of regulation of voluntary motoricactivity without direct influence towards

muscle strength, sensibility, or cerebellum.

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Extrapyramidal disorder

Hyperkinetic disorder: abnormal

involuntary movements

Hypokinetic disorder: reduction ofmovements.

= Movement disorders are caused bydysfunction of basal ganglia

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Basal ganglia:

Caudate nucleus

Putamen

Globus pallidus Subthalamic nucleus

Substantia nigra

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Diseases & syndrome with abnormal

movements:

Essential tremor (familial / benign)

Parkinsonism

Progressive supranuclear palsy Idiopathic torsion dystonia

Chorea sydenham

Sindroma Gilles de la Tourette Drug-induced movement disorders, etc.

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Parkinsonism = Parkinson syndrome

1. Primary / Idiopathic

= Paralysis agitans = Parkinson disease

2. Secondary/Symptomatic

= Causes:- arteriosclerosis

- anoxia /cerebral ischemia

- drugs

- toxic agent- encephalitis etc.

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Parkinsonism (CONT)

3. Paraparkinson / Parkinson plus

- Wilson disease

- Huntington disease- Normotensive Hydrocephalus

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Parkinson disease

= Parkinson idiopathic

= Paralysis agitans

- Most commonly be found

- Middle age to old age

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Etiology and pathogenesis

The certain Etiology is unknown

PD is due to cells degeneration

dopaminedecreased in substantia nigra

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ACh --------------------------------- DA

I

DA

ACh I

ACh

i DA

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Hypotesis:

Aging process: dopaminergic neuronal death

!!! anti oxydative-protective mechanism

decreased

Environmental toxin: - heavy metal (Iron, Zinc,

mercury etc) - MPTP

Genetic sensitivity

Oxydative stress: dopamine reaction free

radicals

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Pathology

Cutting of mid-brain:

degeneration & neuronal death which

contain melanin in substansia-nigra.Microscopic:

In compacta zone:

- Most of neuron are loss- Residual : containings Lewy-bodies

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Lewy bodies in subst.nigra:

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Clinical findings.

Triad of symptoms: - rigidity

- tremor

- bradikinesis

1. tremor: - pill rolling tremor

- during tension !!!, sleep -

disappear

- resting tremor = Parkinsonian

tremor

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Clinical findings (Cont)

2. Rigidity : cogwheel phenomenon.

3. Bradikinese

4. Weakness & fatique5. Dystonia

6. Parkinsonian facies mask-like

7. Micrographia8. Gait: Festinant gait = Parkinsonian gait

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Clinical findings (cont)

9. freezing: late stage, suddlenly stop walking

10. Slowly speaking and monotone

11. Eye movements: - lack of blinking- eye movement disturb.

- Glabella reflex (+)

12. Pain: paresthesia of limbs

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Clinical findings (cont)

13. ANS disturbance:

- salivary / excessive sweating

-urinary incontinence etc.14. Saliva !!! sialorrhea

15. Hypotension orthostatic hypotension

16. Depression17. Dementia

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Gait:

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Tremor:

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Rising:

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Sitting:

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Hypomimia:

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Retropulsion:

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Freezing:

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Diagnostic procedure

Diagnosis is made clinically based on clinical

symptoms found

EEG examination : may show diffuse slow

wave

MRI & CT Scan : may show cortical atrophy.

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Treatment

It is considered: due to dopamine deficiency inCNS.

1. Levodopa

2. Anticholinergic drugs: trihexyphenidyl

3. Antihistamine: diphenhydramine

4. Bromocriptine

5. Amantadine

6. Selegiline

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TH DD

L-tyrosine ----- l-dopa ----- DATH: tyrosine hydroxylase

DD: dopa-decarboxylase

DA located outside vesicles will be catalyzed byenzymes :

MAO(monoamine-oxydase) inside the neuroncell

COMT(catechol-o-methyl-transferase) outsidethe neuron cell.

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In peripheral :

1. l-dopa dopamine

I

dopa-decarboxylase

v

^dopa-decarboxylase inhibitor

= carbidopa sinemet

= benserazide

madopar

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2. l-dopa dopamine

Icatechol-O-methyltransferase (COMT)

v

^

COMT inhibitor : - tolcapone

- entacapone

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Prognosis:

The prognosis is still poor .

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Diseases & syndromes with abnormal

movements :

Essential tremor (familial / benign)

Parkinsonism

Progressive supranuclear palsy

Idiopathic torsion dystonia

Chorea sydenham

Sindroma Gilles de la Tourette

Drug-induced movement disorders, etc.

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Parkinsonism = Parkinson syndrome

1. Primary/ Idiopathic

= Paralysis agitans = Parkinson disease

2. Secondary / Symptomatic

= Well known causes :

- arteriosclerosis

- anoxia / cerebral ischemia

- drugs

- toxic agents- encephalitis, etc.

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Parkinsonism (Cont)

3. Paraparkinson / Parkinson plus

- Wilson disease

- Huntington disease

- Normotensive Hydrocephalus

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ESSENTIAL TREMOR

Familial tremor

Benign tremor

Autosomal dominant

Begin at teen-agers or young adults

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Tremor usually affect one or both hands,

head and voices, whereas foot is not affected.

Neurological assessments show no other

abnormality.

Despite the longer the tremor the clearer the

symptoms, usually it does not disturb ,but the

patient feels ashamed.

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Sometimes it disturbs fine and difficult

movements, writing and speaking whenlaryngeal muscle is involved .

Consuming small amount of alcoholtemporary improvement is reported, but the

mechanism has not been known yet.

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Treatment

- Diazepam 6 15 mg per day

- Propranolol 40 - 120 mg per day

- Primidone

- Alprazolam

- Not responsive:

# thalamotomy

# thalamic stimulation

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CHOREA SYDENHAM

Affecting children and young adults resultingfrom complication of streptococcus hemolyticA infection and the possible basic pathological

features is arteritis.

Approximately 30% of cases occur 2 or 3months following rheumatoid fever orpolyarthritis, the rest without such historys.

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Onset can be acute or gradually within 4-6 months,can also occur during pregnancy or oral

contraceptive users. Symptoms including abnormal chorea movements,

sometimes unilaterally in mild cases.

Changes of behavior

In children may reveal easily to anger and doughty

Labile of emotions

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In 30% of cases involves the heart

Treatment: bed rest, sedatives, prophylaxisantibiotics even though without acuterheumatoid fever .

Penicillin injection to be continued orally until

20 years of age to prevent staphylococcalinfection.

The prognosis basically lies on the

complication of the heart.

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Drug-induced movement disorders

1. Parkinsonism

2. Acute dystonia or dyskinesis

3. Akathisia

4. Tardive dyskinesis

5. Neuroleptic malignant syndrome

6. Others.

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Parkinsonism.

Complication of dopamine receptor inhibitor

drugs:

- phenothiazine

- butyrophenone

- thioxanthene

- metoclopramide

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Parkinsonism (Cont)

Symptoms : - rigidity

- mask-like face

- bradykinesis

- tremor (seldom)

Treatment : - Discontinue the drug

- anti-cholinergic drugs (trihexy-phenidyl)

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Acute dystonia / dyskinesia

blepharospasm

torticollis

facial grimacing

following phenothiazine administration.

Treatment: Intravenous anti-cholinergic-

drug (benztropine 2 mg or diphenhydra-

mine 50 mg).

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Blepharospasm

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Akathisia

Motoric restlessness, compulsion to move or

inability to sit still for more than a short

period of time, keep standing or walking.

Receiving phenothiazine

Treatment:

- Lowering the doseif possible

- propranolol 40-120 mg/hari

- anticholinergic drugs / benzodiazepines

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Tardive dyskinesia

Repetitive involuntary movements of themouth, tongue and lips, occasionallyaccompanied by dystonic posturing or

choreoathetotic movements of trunk andlimbs.

Mostly following administration of neuroleptic, metoclopramide, antihistamines, tri-cyclic

antidepressant and chronic alcohol abuse.

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Tardive dyskinesia (Cont)

Treatment:

- Dopamine antagonists : haloperidol

- Catecholamine-depleting agents:

reserpine, tetrabenazine.

- Baclofen

- Calcium channel blocking agents- Vitamin E, etc.

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Neuroleptic malignant syndrome

An acute idiosyncratic reaction to -neuroleptic medication due to centraldopamine receptor blockade.

May also originate from non-neurololepticdrugs: metoclopramide, domperidone,amphetamines, reserpine, tetrabenazine,tricyclic antidepressants with lithium or with

MAO inhibitor and withdrawal of anti-parkinsonian medication.

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Neuroleptic malignant syndrome(Cont)

The use of cocaine and 3.4 methy- lenedioxymethamphetamine (MDMA) = Ecstasy

Symptoms are usually abrupt in onset, fever,encephalopathy with fluctuating confusion andagitation progressing to coma, labile BP, tachycardia,diaphoresis in association with dystonia and tremor.

The couse is often rapid in untreated cases witdeterioration leading to cardiac failure , pulmonarycongestion, pneumonia, renal failure and death.

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Neuroleptic malignant syndrome (Cont)

Treatment:

- Discontinue the neuroleptic drugs

- Control of body temperature

- Monitor the BP, urine, electrolytes, temperaturechange

- Administer bromocriptine 75 -100 mg/day by NGT

- Dantrolene sodium i.v and convert to oral asimprovement occurs.

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Others:

Levodopa various abnormal movement is

related to dosage.

Bromocriptine, anticholinergic drugs,

phenytoin, carbamazepine, amphetamine,

lithium and oral contraceptivemay also

result in chorea

ect.

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K13 Extrapyramydal Disorder