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 motoric

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

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Extrapyramidal disorder
Hyperkinetic disorder: abnormal
involuntary movements

Hypokinetic disorder: reduction of

movements.

= Movement disorders are caused by

dysfunction 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

dopamine decreased in substantia nigra

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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 & fatique
5. Dystonia
6. Parkinsonian facies mask-like
7. Micrographia
8. 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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