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Kuliah Mov Disorder

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    MOVEMENT DISORDER

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    THE EXTRAPYRAMIDAL SYSTEM

    DEFINITION: The extrapyramidal system is a general

    term for the basal ganglia

    STRUCTURE:

    Centers: the corpus striatum, the substantianigra, thalamic and subthalamic nuclei

    Inter connections: the above centers areinterconnected with: The cerebral cortex, the

    cerebellum, the reticular formation, the cranialnerve nuclei (particularly the vestibular nerve)and the spinal cord

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    Functions of the extrapyramidal

    system

    Regulation of voluntary motor activity

    Control of the muscle tone

    Maintenance of emotional and associative

    movements

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    Movement disorders

    Hypokinetic

    Idiopathic Parkinson's diseaseParkinsonism

    Parkinsonism-plus

    Hyperkinetic

    Essential tremor

    Dystonia

    Hemiballismus/Ballismus

    Chorea-atetosa

    Myoclonuus

    Hemiballismus

    TIC

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    PARKINSONISM

    Parkinsonism (shaking palsy) is a condition in

    which there are static regular tremors,

    hypertonia of the muscles of the body withbradykinesia and postural instability

    Parkinson Disease : idiophatic Parkinsonism

    doe to damage on Subtansia nigra andStriatum

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    PARKINSON DISEASE

    RESTING TREMOR ( T )

    RIGIDITY ( R )

    AKINESIA ( A )

    POSTURAL INSTABILITY ( P )

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    History of Parkinsons disease (PD)

    First described in 1817 by an English physician,

    James Parkinson, in An Essay on the Shaking

    Palsy.

    The famous French neurologist, Charcot, further

    described the syndrome in the late 1800s.

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    Epidemiology of PD

    The most common movement disorder

    affecting 1-2 % of the general

    population over the age of 65 years.

    The second most common

    neurodegenerative disorder afterAlzheimers disease (AD).

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    Risk factors of PD

    Age -the most important risk factor

    Positive family history

    Male gender

    Environmental exposure: Herbicide and pesticideexposure, metals (manganese, iron), well water, farming,

    rural residence, wood pulp mills; and steel alloy

    industries

    Race

    Life experiences (trauma, emotional stress, personality

    traits such as shyness and depressiveness)?

    An inverse correlation between cigarette smoking and

    caffeine intake in case-control studies.

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    Functional neuroanatomy of PD

    Substantia nigra: The major origin of the dopaminergic

    innervation of the striatum.

    Part of extrapyramidal system which processes

    information coming from the cortex to the striatum,

    returning it back to the cortex through the thalamus.

    One major function of the striatum is the regulation of

    posture and muscle tonus.

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    Dopamine pathways in human brain

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    Dopamine synthesis

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    PATHOGENESIS

    Changes in neuromuscular profile occur as follows:

    1.Putamen/Striatum:

    Dopamine 60%

    Noradrenaline 60%

    5- hydroxytryptamine 60%2. Substantia nigra:

    Dopamine 60%

    Glutamic acid decarboxylate (GDA)+-amino butyric acid

    (GABA)3. Cerebral cortex

    Glutamic acid decarboxylate (GDA)+-amino butyric acid(GABA)

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    Acethylcholine

    PARKINSON A CRITICAL BALANCE

    IDopamine

    Feb 4, 2005

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    WHEN TO START TREATMENT FOR

    PARKINSON DISEASE

    WHEN DISEASE MANIFESTATIONS INTERFERE

    WITH SOCIAL AND VOCATIONAL ACTIVITIES,WORSENING OR GAIT OR BALANCE OR OTHER

    ACTIVITIES OF DAILY LIVING.

    PARTNERSHIP WITH PATIENT!

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    TREATMENT OF

    PARKINSON DISEASE

    MEDICAL

    DOPAMINERGIC AGENTS

    DOPAMINE AGONIST

    ANTI-CHOLINERGICS

    SURGICAL

    ABLATIVE

    Talamotomy.Pallidotomy

    D.B.S.(Deep Brain

    Stimulation )

    PHYSICAL THERAPIES

    P.T.

    O.T.

    SPEECH

    EXERCISE Rx, TAI-CHI

    PSYCHOTHERAPIES

    COUNSELLING

    SOCIAL WORK

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    Strategy of Treatment

    (A) DopamineCarbidopa/l-dopa

    Dopamine agonists: Apomorphine,

    CabergolineRopinirole,Pramipexole

    COMT inhibitors: Entacapone

    MAO Inhibitors: Selegiline

    Inhibitors of dopamine re-uptake: Amantadine

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    Strategy of Treatment

    (2) Acetylcholine

    Anticholinergic

    Antihistaminics

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    TREATMENT OF

    PARKINSON DISEASE

    Drugs

    DOPAMINERGIC AGENTS

    L-Dopa, L-Dopa+ Benzeraside,

    L-Dopa+ Benzeraside+ Entacapone

    DOPAMINE AGONIST

    Pramipexole, Ropinelrole

    ANTI-CHOLINERGICS

    Trihexyphenidyl

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    WHAT ABOUT LEVODOPA/L-DOPA

    STILL THE BEST, ESPECIALLY SHORT TERM

    LONG TERM USE . Side effect

    MOTOR FLUCTUATIONS, DYSKINESIASON-OFF Phenomenon, WEARING -OFF

    FIRST CHOISE

    BUT NEARLY ALL PATIENTS EVENTUALLY REQUIRE IT

    ANTICHOLENERGIC : Good for Tremor, but NotGOOD, fore Age > 70 years

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    Essential Tremor

    Bilateral action tremor of the hands andforearms

    Absence of other neurologic signs

    Long duration (>3 years)

    Family history

    Beneficial response to ethanol

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    Tremor

    Rest tremor Action tremor

    PD

    Tardive dyskinesia

    Severe ET

    Physiologic

    ET

    PD

    Midbrain

    Cerebellar

    Handwriting

    Postural Kinetic Task-Specific

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    Classification Age of onse

    Distribution

    Etiology

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    Classification: Age of Onset Early Onset

    childhood, adolescence.

    Late Onsetadulthood (peak 30 50 years).

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    Classification: Distribution Focal Dystonia affecting a single body part or

    location

    Segmental Dystonia affecting adjacent body

    parts

    Hemidystonia affecting one side of the body

    Generalized affecting more than 2 segment

    of the body

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    Classification: Etiology1.Primary dystonia

    No structural abnormality in the CNS (oftengenetic)

    2.Dystonia Plus Syndromes Primary dystonia + parkinsonism, myoclonus

    3.Secondary Dystonia

    demonstrable exogenous or structural4.Heredodegenerative Dystonia

    underlying brain degeneration

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    Treatment

    Focal / Segmental

    Botulinum toxin injections to affected areas

    Medications

    Primary Generalized

    Oral Medications

    Intrathecal baclofen

    Surgery

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    Medications

    Levodopa (Sinemet)

    Everyone should be given a trial of sinemet

    especially children.

    Treatment of choice for Dopa-Responsive Dystonia(low doses 100 to 300 mg)

    Other causes of dystonia may respond to

    levodopa

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    Medications

    Anticholinergics

    Trihexiphenidyl (Artane).

    Start at 1 mg daily and increase weekly to effect,

    in children may go up to 30 or 40 mg. Not tolerated well in adults.

    Side effects - confusion, drowsiness,hallucinations,forgetfulness, personality changes,

    dry mouth, blurred vision, and urinary retention. One poorly designed RTC showed 50 %

    improvement.

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    Medications

    Dopamine Depletors/ Blockers

    Tetrabenzine.

    Clozapine.

    Olanzapine.

    Benzodiazepines

    Baclofen (oral and intrathecal)

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    Surgery

    Pallidal Deep Brain Stimulation

    Series only ~ 50 % improvement.

    Better results with primary generalized dystonia.

    Mixed results with secondary generalized

    dystonia.

    Series only, no RTCs.

    Effects delayed by months after surgery.

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    Drug-Induced Movement Disorders

    Neuroleptic

    Parkinsonism

    Acute dystonia

    Tardive dyskinesia

    Neuroleptic Malignant Syndrome

    Central Anti emetics

    Dystonia

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    Non Medication Treatment

    Occupational focal dystonia.

    Supportive therapy.

    Physiotherapists, occupational therapist, speech

    therapists.

    Education, support groups.

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