Transcript EXTRAPYRAMIDAL SYSTEM DISORDERS

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 runfestination
---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 coordinated 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