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.
 Toxins—CO 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.
 Gait—slow 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 agonists—bromocriptine, pergolide

 COMT inhibitors (catechol-o-methyl transferase)

—tolcapone
 MAO –inhibitors--selegine
HUNTINGTONS DISEASE
 Inherited disorder

 Autosomal dominant

 Males females equally affected

 Presents during the 4th 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

 Drugs—Rx 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
 Tardive—drugs-> 6 wks exposure to

dopamine agonists
 Orofacial repeated movements
TREMORS
 Physiological

 Familial

 Resting—parkinsons disease

 Intention or action ---cerebellar

TICS
 Brief stereotyped supressible movements

 Worse with stress

Cause
Dopamine excess causes inhibition of limbic
system
Rx
Dopamine agonist