K13 Extrapyramydal Disorder
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Transcript of 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.