EXTRAPYRAMIDAL

SYSTEM
DISORDERS
BASAL GANGLION
DYSFUNCTION
ANATOMY
Caudate nucleaus
Putamen
Globus pallidus
Substantia nigra
Subthalamic nuleus
Thallamus



EFFECTS OF DYSFUNCTION IN GENERAL
Involuntary movements
Altered movements
slow
Interrupted
Uncordinated
Posture and tone altered


MOVEMENT DISORDERS
BRADYKINETIC ---TOO LITTLE
Parkinson disease
Wilsons disease
Huntingtons disease
HYPERKINETIC TOO MUCH
Athetosis
Hemiballismus
Dystonia
Dyskinesia
Chorea
Myoclonus
Tremors
Tics

NEUROTRANSMITTERS

Dopamine > Ach = hyperkinetic
Ach > dopamine =hypokinetic
CAUSES OF EXTRAPYRAMIDAL DISORDERS

Drugs---chlorpromazine
---butyrophenons
---metochlorpramide
---reserpine
Causes cont.
ToxinsCO and manganese poisoning
Inherited and metabolic disorders :
wilsons disease
spinocerebellar ataxia
Encephalitis lethargica
Diffuse small vascular disease
Causes cont.
Inherited or degenerative disease
huntingtons disease
progressive supra nuclear gaze palsy
{Steel Richardson}
PARKINSONS DISEASE
Effects dopaminergic neurons
Neurons are lost from substantia nigra
Rarely presents before 50 years
Neurodegenerative disease
Equal sex distribution
CLINICAL FEATURES
Characterized by:
Tremors
Rigidity
bradykinesia
TREMORS
Rest tremor
Starts in the thumb
Adduction and abduction of the thumb
Pill rolling
Tremors may effect the legs, mouth or the
tongue

RIGIDITY
Leadpipe or plastic
Cogwheel
BRADYKINESIA
Slow movements
Develop gradually
Impairement of fine movements








General clinical features
Slow and monotonous speech
Greasy skin
Expressionless face ---mask face
Infrequent blinking
Flexed posture
Reduced arm swing
Clinical features cont.
Gaitslow in initiating
---rapid small steps tendency to run-
festination
---shuffling
Impaired balance
Glabber tap
Clinical features cont.
Muscle power is normal
Reflexes normal
Sensations normal
Cognitive abnormality as the disease
advances
INVESTIGATIONS
Clinical diagnosis
Exclude other causes pts who present
before 50 years
Brain CT scan or MRI
TREATMENT
Levodopa
Anticholinergic drugs
Amantadine
Dopamine agonistsbromocriptine, pergolide
COMT inhibitors (catechol-o-methyl
transferase)tolcapone
MAO inhibitors--selegine


HUNTINGTONS DISEASE
Inherited disorder
Autosomal dominant
Males females equally affected
Presents during the 4
th
decade
Chorea which worsens with time
Cognitive disorders
Dementia

Cont.
Abnormal facial movement
Mood swings
Jaw clenching
Slurred speech
Difficulty in walking
Personality changes
Cont.
Abnormal facial movement
Mood swings
Jaw clenching
Slurred speech
Difficulty in walking
Personality changes
WILSONS DISEASE
Hepato lenticular disorder
Autosomal recessive
Treatable cause of parkinsonsim
Due to deposition of copper in basal
ganglia
Onset during childhood rarely in adulthood
Cont.
Present with liver disease in childhood
Impaired concentration
Decling intellect
Behavioural problems
Involuntary movements
Generalized dystonia
Cont.
Ataxia
Kayser Fleicher ring
Diagnosis
Serum ceruloplasmin level
24 hour urine for copper
LFT
Liver biopsy
HYPERKINETIC MOVEMENTS

large variety of hyperkinetic disorders
Most are organic
All movements disappear during sleep
CHOREA

Continous unsustained rapid abrupt and
random contractures
Small fidgety movements
Distal muscles involved
CAUSES
Huntingtons disease
DrugsRx of parkinsonism , oral c.pills
SLE
Sydenhams chorea
Wilsons disease
Polycythemia
Friedricks ataxia
HEMIBALLISMUS
Throwing of the limbs on one side of the
body
Usually due to CVA involving the
subthalamic nucleus
MYOCLONUS
Simple jerky movements that are not co-
ordinated or suppressible
CAUSES
Renal failure
Hepatic failure
Creutz feldt jacob disease
Subacute sclerosing panencephalitis
DYSTONIA
Repeated patterned twisting and sustained
movements that may be either slow or
rapid
Involuntary movements occur before 20
years
Dystonia Cont.
Disturbance of the affected muscle groups
depend upon age
--distally---in children
---cranial - cervical ---adults
Dystonia cont.
Primary----focal----torticollis
----writers cramps
generalized
Secondary----wilsons disease
----toxins
ATHETOSIS
Writhing movements
Mainly due to cerebral palsy
DYSKINESIA
Tardivedrugs-> 6 wks exposure to
dopamine agonists
Orofacial repeated movements

TREMORS
Physiological
Familial
Restingparkinsons disease
Intention or action ---cerebellar

TICS
Brief stereotyped supressible movements
Worse with stress
Cause
Dopamine excess causes inhibition of limbic
system
Rx
Dopamine agonist