EXTRAPYRAMIDAL

DISORDERS
= Movement disorders
= Degenerative disease

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

Extrapyramidal disorder
Hyperkinetic disorder: abnormal
involuntary movements
Hypokinetic disorder: reduction of
movements.
= Movement disorders are caused by
dysfunction of basal ganglia

Basal ganglia:

Caudate nucleus
Putamen
Globus pallidus
Subthalamic nucleus
Substantia nigra

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.

Parkinsonism (CONT)
3. Paraparkinson / Parkinson plus
- Wilson disease
- Huntington disease
- Normotensive Hydrocephalus

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

dopamine decreased 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.
3.
4.
5.
6.
7.
8.

Rigidity : cogwheel phenomenon.

Bradikinese
Weakness & fatique
Dystonia
Parkinsonian facies mask-like
Micrographia
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. Depression
17. 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
in CNS.
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 -----
DA
TH: tyrosine hydroxylase
DD: dopa-decarboxylase
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.
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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
I
catechol-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.
33

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 when
laryngeal muscle is involved .
Consuming small amount of alcohol

temporary 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
resulting from complication of
streptococcus hemolytic A infection and
the possible basic pathological features is
arteritis.
Approximately 30% of cases occur 2 or 3
months following rheumatoid fever or
polyarthritis, 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,
prophylaxis antibiotics even though
without acute rheumatoid fever .
Penicillin injection to be continued orally
until 20 years of age to prevent
staphylococcal infection.
The prognosis basically lies on the
complication of the heart.

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Drug--induced movement disorders

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

Parkinsonism
Acute dystonia or dyskinesis
Akathisia
Tardive dyskinesis
Neuroleptic malignant syndrome
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 (trihexyphenidyl)
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Acute dystonia / dyskinesia

blepharospasm
torticollis
facial grimacing
following phenothiazine administration.
Treatment: Intravenous anti-cholinergicdrug (benztropine 2 mg or diphenhydramine 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 dose
if possible
- propranolol 40-120 mg/hari
- anticholinergic drugs / benzodiazepines
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Tardive dyskinesia
Repetitive involuntary movements of the
mouth, tongue and lips, occasionally
accompanied by dystonic posturing or
choreoathetotic movements of trunk and
limbs.
Mostly following administration of neurolep
tic, metoclopramide, antihistamines, tricyclic 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 central
dopamine receptor blockade.
May also originate from non-neurololeptic
drugs: metoclopramide, domperidone,
amphetamines, reserpine, tetrabenazine,
tricyclic antidepressants with lithium or
with MAO inhibitor and withdrawal of antiparkinsonian medication.
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Neuroleptic malignant
syndrome(Cont)
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,
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.
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Neuroleptic malignant syndrome

(Cont)
Treatment:
- Discontinue the neuroleptic drugs
- Control of body temperature
- 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.
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Others:
Levodopa
various abnormal movement
is related to dosage.
Bromocriptine, anticholinergic drugs,
phenytoin, carbamazepine, amphetamine,
lithium and oral contraceptive
may also
result in chorea
ect.

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