MOVEMENT DISORDER

THE EXTRAPYRAMIDAL SYSTEM

DEFINITION: The extrapyramidal system is a general
term for the basal ganglia
STRUCTURE:
Centers: the corpus striatum, the substantia
nigra, thalamic and subthalamic nuclei
Inter connections: the above centers are
interconnected with: The cerebral cortex, the
cerebellum, the reticular formation, the cranial
nerve nuclei (particularly the vestibular nerve)
and the spinal cord
Functions of the extrapyramidal
system

Regulation of voluntary motor activity

Control of the muscle tone

Maintenance of emotional and associative
movements
Movement disorders
Hypokinetic
Idiopathic Parkinson's disease
Parkinsonism
Parkinsonism-plus

Hyperkinetic
Essential tremor
Dystonia
Hemiballismus/Ballismus
Chorea-atetosa
Myoclonuus
Hemiballismus
TIC
PARKINSONISM

Parkinsonism (shaking palsy) is a condition in
which there are static regular tremors,
hypertonia of the muscles of the body with
bradykinesia and postural instability
Parkinson Disease : idiophatic Parkinsonism
doe to damage on Subtansia nigra and
Striatum
PARKINSON DISEASE

RESTING TREMOR ( T )
RIGIDITY ( R )
AKINESIA ( A )
POSTURAL INSTABILITY ( P )
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.
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 after
Alzheimers disease (AD).
Risk factors of PD


Age - the most important risk factor
Positive family history
Male gender
Environmental exposure: Herbicide and pesticide
exposure, 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.
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.
Dopamine pathways in human brain
Dopamine synthesis

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)




Acethylcholine
PARKINSON A CRITICAL BALANCE
IDopamine
Feb 4, 2005
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!
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


Strategy of Treatment
(A) Dopamine
Carbidopa/l-dopa
Dopamine agonists: Apomorphine,
Cabergoline
Ropinirole,
Pramipexole
COMT inhibitors: Entacapone
MAO Inhibitors: Selegiline
Inhibitors of dopamine re-uptake: Amantadine
Strategy of Treatment
(2) Acetylcholine
Anticholinergic
Antihistaminics



TREATMENT OF
PARKINSON DISEASE
Drugs
DOPAMINERGIC AGENTS
L-Dopa, L-Dopa+ Benzeraside,
L-Dopa+ Benzeraside+ Entacapone

DOPAMINE AGONIST
Pramipexole, Ropinelrole

ANTI-CHOLINERGICS
Trihexyphenidyl




WHAT ABOUT LEVODOPA/L-DOPA
STILL THE BEST, ESPECIALLY SHORT TERM
LONG TERM USE . Side effect
MOTOR FLUCTUATIONS, DYSKINESIAS
ON-OFF Phenomenon, WEARING -OFF
FIRST CHOISE
BUT NEARLY ALL PATIENTS EVENTUALLY REQUIRE IT
ANTICHOLENERGIC : Good for Tremor, but Not
GOOD, fore Age > 70 years

Essential Tremor
Bilateral action tremor of the hands and
forearms
Absence of other neurologic signs
Long duration (>3 years)
Family history
Beneficial response to ethanol

Tremor
Rest tremor Action tremor
PD
Tardive dyskinesia
Severe ET
Physiologic
ET
PD
Midbrain
Cerebellar

Handwriting

Postural Kinetic Task-Specific
Classification

Age of onse

Distribution

Etiology
Classification: Age of Onset
Early Onset
childhood, adolescence.
Late Onset
adulthood (peak 30 50 years).
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
Classification: Etiology
1.Primary dystonia
No structural abnormality in the CNS (often
genetic)
2.Dystonia Plus Syndromes
Primary dystonia + parkinsonism, myoclonus
3.Secondary Dystonia
demonstrable exogenous or structural
4.Heredodegenerative Dystonia
underlying brain degeneration
Treatment
Focal / Segmental
Botulinum toxin injections to affected areas
Medications
Primary Generalized
Oral Medications
Intrathecal baclofen
Surgery
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
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.
Medications
Dopamine Depletors/ Blockers
Tetrabenzine.
Clozapine.
Olanzapine.
Benzodiazepines
Baclofen (oral and intrathecal)
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.
Drug-Induced Movement Disorders
Neuroleptic
Parkinsonism
Acute dystonia
Tardive dyskinesia
Neuroleptic Malignant Syndrome
Central Anti emetics
Dystonia

Non Medication Treatment
Occupational focal dystonia.
Supportive therapy.
Physiotherapists, occupational therapist, speech
therapists.
Education, support groups.