Extrapyramydal disorder-1.ppt...

EXTRAPYRAMIDAL EXTRAPYRAMIDAL

DISORDERSDISORDERS

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= Movement disorders= Movement disorders

= Degenerative disease= Degenerative disease

4 types of movements:4 types of movements:

• Voluntary movement

• Semivoluntary movement (=unvoluntary)

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• Involuntary movement

• Automatic movement

Movement disordersMovement disorders

= Extrapyramidal disorder

Disorder of regulation of voluntary motoric activity without direct influence towards

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activity without direct influence towards muscle strength, sensibility, or cerebellum.

Extrapyramidal disorderExtrapyramidal disorder

• Hyperkinetic disorder: abnormal

involuntary movements

• Hypokinetic disorder: reduction of

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• Hypokinetic disorder: reduction of

movements.

= Movement disorders are caused by

dysfunction of basal ganglia

Basal ganglia:Basal ganglia:

• Caudate nucleus

• Putamen

• Globus pallidus

• Subthalamic nucleus

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• Subthalamic nucleus

• Substantia nigra

Diseases & syndrome with Diseases & syndrome with

abnormal movements:abnormal movements:

• Essential tremor (familial / benign)

• Parkinsonism

• Progressive supranuclear palsy

• Idiopathic torsion dystonia

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• Chorea sydenham

• Sindroma Gilles de la Tourette

• Drug-induced movement disorders, etc.

Parkinsonism = Parkinson Parkinsonism = Parkinson

syndromesyndrome

1. Primary / Idiopathic

= Paralysis agitans = Parkinson disease

2. Secondary/Symptomatic

= Causes:

- arteriosclerosis

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- arteriosclerosis

- anoxia /cerebral ischemia

- drugs

- toxic agent

- encephalitis etc.

Parkinsonism (CON’T)Parkinsonism (CON’T)

3. Paraparkinson / Parkinson plus

- Wilson disease

- Huntington disease

- Normotensive Hydrocephalus

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Parkinson diseaseParkinson disease

= Parkinson idiopathic

= Paralysis agitans

- Most commonly be found

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- Most commonly be found

- Middle age to old age

Etiology and pathogenesisEtiology and pathogenesis

• The certain Etiology is unknown

• PD is due to cells degeneration �dopamine decreased in substantia nigra

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dopamine decreased in substantia nigra

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

I

DA

ACh I

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ACh I

ACh

i DA

Hypotesis:Hypotesis:

• Aging process: dopaminergic neuronal death !!! anti – oxydative-protective mechanism decreased

• Environmental toxin: - heavy metal (Iron,

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• Environmental toxin: - heavy metal (Iron, Zinc, mercury etc) - MPTP

• Genetic sensitivity

• Oxydative stress: dopamine reaction �free radicals

PathologyPathology

Cutting of mid-brain:

degeneration & neuronal death which

contain melanin in substansia-nigra.

Microscopic:

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Microscopic:

In compacta zone:

- Most of neuron are loss

- Residual : containings Lewy-bodies

Lewy bodies in subst.nigra:Lewy bodies in subst.nigra:

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Clinical findings.Clinical findings.

• Triad of symptoms: - rigidity

- tremor

- bradikinesis

1. tremor: - pill rolling tremor

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1. tremor: - pill rolling tremor

- during tension !!!, sleep -

disappear

- resting tremor = Parkinsonian –

tremor

Clinical findings (Con’t)Clinical findings (Con’t)

2. Rigidity : “cogwheel” phenomenon.

3. Bradikinese

4. Weakness & fatique

5. Dystonia

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5. Dystonia

6. Parkinsonian facies “mask-like”

7. Micrographia

8. Gait: Festinant gait = Parkinsonian gait

Clinical findings (con’t)Clinical findings (con’t)

9. freezing: late stage, suddlenly stop walking

10. Slowly speaking and monotone

11. Eye movements: - lack of blinking

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11. Eye movements: - lack of blinking

- eye movement disturb.

- Glabella reflex (+)

12. Pain: paresthesia of limbs

Clinical findings (con’t)Clinical findings (con’t)

13. ANS disturbance:

- salivary / excessive sweating

-urinary incontinence etc.

14. Saliva !!! sialorrhea

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14. Saliva !!! � sialorrhea

15. Hypotension � orthostatic hypotension

16. Depression

17. Dementia

Gait:Gait:

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Tremor:Tremor:

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Rising:Rising:

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Sitting:Sitting:

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Hypomimia:Hypomimia:

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Retropulsion:Retropulsion:

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Freezing:Freezing:

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Diagnostic procedureDiagnostic procedure

• Diagnosis is made clinically based on clinical symptoms found

• EEG examination : may show diffuse slow wave

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wave

• MRI & CT Scan : may show cortical atrophy.

TreatmentTreatment

It is considered: due to dopamine deficiency in CNS.

1. Levodopa

2. Anticholinergic drugs: trihexyphenidyl

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2. Anticholinergic drugs: trihexyphenidyl

3. Antihistamine: diphenhydramine

4. Bromocriptine

5. Amantadine

6. Selegiline

TH DD

L-tyrosine -----� l-dopa -----� DA

TH: tyrosine hydroxylase

DD: dopa-decarboxylase

DA located outside vesicles will be catalyzed

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DA located outside vesicles will be catalyzed by enzymes :

MAO(monoamine-oxydase) inside the neuron cell

COMT(catechol-o-methyl-transferase) outside the neuron cell.

In peripheral :

1. l-dopa � dopamine

I

dopa-decarboxylase

v

^

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bb

^

dopa-decarboxylase inhibitor

= carbidopa � sinemet

= benserazide � madopar

2. l-dopa � dopamine

I

catechol-O-methyltransferase (COMT)

v

^

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^

COMT – inhibitor : - tolcapone

- entacapone

Prognosis:Prognosis:

The prognosis is still poor .

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Diseases & syndromes with Diseases & syndromes with

abnormal movements :abnormal movements :

• Essential tremor (familial / benign)

• Parkinsonism

• Progressive supranuclear palsy


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• Chorea sydenham

• Sindroma Gilles de la Tourette

• Drug-induced movement disorders, etc.

Parkinsonism = Parkinson Parkinsonism = Parkinson

syndromesyndrome

1. Primary/ Idiopathic

= Paralysis agitans = Parkinson disease

2. Secondary / Symptomatic

= Well known causes :

- arteriosclerosis

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- arteriosclerosis

- anoxia / cerebral ischemia

- drugs

- toxic agents

- encephalitis, etc.

Parkinsonism (Con’t)Parkinsonism (Con’t)

3. Paraparkinson / Parkinson plus

- Wilson disease

- Huntington disease


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ESSENTIAL TREMORESSENTIAL TREMOR

• “ Familial tremor “

• “ Benign tremor “

• “ Autosomal dominant “

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Begin at teen-agers or young adults

• 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

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• Despite the longer the tremor the clearer the symptoms, usually it does not disturb ,but the patient feels ashamed.

• Sometimes it disturbs fine and difficult movements, writing and speaking when laryngeal muscle is involved .

• Consuming small amount of alcohol �

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• Consuming small amount of alcohol �temporary improvement is reported, but the mechanism has not been known yet.

Treatment

- Diazepam 6 – 15 mg per day

- Propranolol 40 - 120 mg per day

- Primidone

- Alprazolam

- Not responsive:

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- Not responsive:

# thalamotomy

# thalamic stimulation

CHOREA SYDENHAMCHOREA SYDENHAM

• Affecting children and young adults resulting from complication of streptococcus hemolytic A infection and the possible basic pathological features is arteritis.

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arteritis.

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

• 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

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• Changes of behavior

• In children may reveal easily to anger and doughty

• Labile of emotions

• In 30% of cases involves the heart

• Treatment: bed rest, sedatives, prophylaxis antibiotics even though without acute rheumatoid fever .

• Penicillin injection to be continued orally until 20 years of age to prevent

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until 20 years of age to prevent staphylococcal infection.

• The prognosis basically lies on the complication of the heart.

DrugDrug--induced movement disordersinduced movement disorders

1. Parkinsonism

2. Acute dystonia or dyskinesis

3. Akathisia

4. Tardive dyskinesis

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4. Tardive dyskinesis

5. Neuroleptic malignant syndrome

6. Others.

Parkinsonism.Parkinsonism.

Complication of dopamine receptor inhibitor drugs:

- phenothiazine

- butyrophenone

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- butyrophenone

- thioxanthene

- metoclopramide

Parkinsonism (Con’t)Parkinsonism (Con’t)

Symptoms : - rigidity

- mask-like face

- bradykinesis

- tremor (seldom)

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- tremor (seldom)

Treatment : - Discontinue the drug

- anti-cholinergic drugs (trihexy-

phenidyl)

Acute dystonia / dyskinesiaAcute dystonia / dyskinesia

• blepharospasm

• torticollis

• facial grimacing

following phenothiazine administration.

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following phenothiazine administration.

Treatment: Intravenous anti-cholinergic-

drug (benztropine 2 mg or diphenhydra-

mine 50 mg).

BlepharospasmBlepharospasm

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AkathisiaAkathisia

• Motoric restlessness, compulsion to move or inability to sit still for more than a short period of time, keep standing or walking.

• Receiving phenothiazine

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• Receiving phenothiazine

• Treatment:

- Lowering the dose �if possible

- propranolol 40-120 mg/hari

- anticholinergic drugs / benzodiazepines

Tardive dyskinesiaTardive dyskinesia

• Repetitive involuntary movements of the mouth, tongue and lips, occasionally accompanied by dystonic posturing or choreoathetotic movements of trunk and limbs.

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limbs.

• Mostly following administration of neurolep tic, metoclopramide, antihistamines, tri-cyclic antidepressant and chronic alcohol abuse.

Tardive dyskinesia (Con’t)Tardive dyskinesia (Con’t)

• Treatment:

- Dopamine antagonists : haloperidol

- Catecholamine-depleting agents:

reserpine, tetrabenazine.

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reserpine, tetrabenazine.

- Baclofen

- Calcium channel blocking agents

- Vitamin E, etc.

Neuroleptic malignant syndromeNeuroleptic malignant syndrome

• An acute idiosyncratic reaction to -neuroleptic medication due to central dopamine receptor blockade.

• May also originate from non-neurololeptic

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• May also originate from non-neurololeptic drugs: metoclopramide, domperidone, amphetamines, reserpine, tetrabenazine, tricyclic antidepressants with lithium or with MAO inhibitor and withdrawal of anti-parkinsonian medication.

Neuroleptic malignant Neuroleptic malignant

syndrome(Con’t)syndrome(Con’t)

• The use of cocaine and 3.4 methy- lene dioxymethamphetamine (MDMA) = “Ecstasy”

• Symptoms are usually abrupt in onset, fever, encephalopathy with fluctuating confusion and agitation progressing to coma, labile BP,

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agitation progressing to coma, labile BP, tachycardia, diaphoresis in association with dystonia and tremor.

• The couse is often rapid in untreated cases wit deterioration leading to cardiac failure , pulmonary congestion, pneumonia, renal failure and death.

Neuroleptic malignant syndrome Neuroleptic malignant syndrome

(Con’t)(Con’t)

Treatment:

- Discontinue the neuroleptic drugs

- Control of body temperature

- Monitor the BP, urine, electrolytes, temperature

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- Monitor the BP, urine, electrolytes, temperature

change

- Administer bromocriptine 75 -100 mg/day by

NGT

- Dantrolene sodium i.v and convert to oral as

improvement occurs.

Others:Others:

• Levodopa � various abnormal movement is related to dosage.

• Bromocriptine, anticholinergic drugs, phenytoin, carbamazepine, amphetamine,

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phenytoin, carbamazepine, amphetamine, lithium and oral contraceptive � may also result in chorea

• ect.

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