EXTRAPYRAMIDAL SYSTEM DISORDERS

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EXTRAPYRAMIDAL SYSTEM DISORDERS
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• BASAL GANGLION DYSFUNCTION

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• ANATOMY
• Caudate nucleaus
• Putamen
• Globus pallidus
• Substantia nigra
• Subthalamic nuleus
• Thallamus

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

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• MOVEMENT DISORDERS
• BRADYKINETIC ---TOO LITTLE
• Parkinson disease
• Wilsons disease
• Huntingtons disease

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• HYPERKINETIC TOO MUCH
• Athetosis
• Hemiballismus
• Dystonia
• Dyskinesia
• Chorea
• Myoclonus
• Tremors
• Tics

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• NEUROTRANSMITTERS
• Dopamine gt Ach hyperkinetic
• Ach gt dopamine hypokinetic

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• CAUSES OF EXTRAPYRAMIDAL DISORDERS
• Drugs---chlorpromazine
• ---butyrophenons
• ---metochlorpramide
• ---reserpine

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• Causes cont.
• ToxinsCO and manganese poisoning
• Inherited and metabolic disorders
• wilsons disease
• spinocerebellar ataxia
• Encephalitis lethargica
• Diffuse small vascular disease

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• Causes cont.
• Inherited or degenerative disease
• huntingtons disease
• progressive supra nuclear gaze palsy
• Steel Richardson

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• PARKINSONS DISEASE
• Effects dopaminergic neurons
• Neurons are lost from substantia nigra
• Rarely presents before 50 years
• Neurodegenerative disease
• Equal sex distribution

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• CLINICAL FEATURES
• Characterized by
• Tremors
• Rigidity
• bradykinesia

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• TREMORS
• Rest tremor
• Starts in the thumb
• Adduction and abduction of the thumb
• Pill rolling
• Tremors may effect the legs, mouth or the tongue

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

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• General clinical features
• Slow and monotonous speech
• Greasy skin
• Expressionless face ---mask face
• Infrequent blinking
• Flexed posture
• Reduced arm swing

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• Clinical features cont.
• Gaitslow in initiating
• ---rapid small steps tendency to run-
• festination
• ---shuffling
• Impaired balance
• Glabber tap

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• Clinical features cont.
• Muscle power is normal
• Reflexes normal
• Sensations normal
• Cognitive abnormality as the disease advances

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• INVESTIGATIONS
• Clinical diagnosis
• Exclude other causes pts who present before 50
  years
• Brain CT scan or MRI

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• TREATMENT
• Levodopa
• Anticholinergic drugs
• Amantadine
• Dopamine agonistsbromocriptine, pergolide
• COMT inhibitors (catechol-o-methyl
  transferase)tolcapone
• MAO inhibitors--selegine

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

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• Cont.
• Abnormal facial movement
• Mood swings
• Jaw clenching
• Slurred speech
• Difficulty in walking
• Personality changes

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• Cont.
• Abnormal facial movement
• Mood swings
• Jaw clenching
• Slurred speech
• Difficulty in walking
• Personality changes

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• 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

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• Cont.
• Present with liver disease in childhood
• Impaired concentration
• Decling intellect
• Behavioural problems
• Involuntary movements
• Generalized dystonia

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• Cont.
• Ataxia
• Kayser Fleicher ring
• Diagnosis
• Serum ceruloplasmin level
• 24 hour urine for copper
• LFT
• Liver biopsy

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• HYPERKINETIC MOVEMENTS
• large variety of hyperkinetic disorders
• Most are organic
• All movements disappear during sleep

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• CHOREA
• Continous unsustained rapid abrupt and random
  contractures
• Small fidgety movements
• Distal muscles involved

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• CAUSES
• Huntingtons disease
• DrugsRx of parkinsonism , oral c.pills
• SLE
• Sydenhams chorea
• Wilsons disease
• Polycythemia
• Friedricks ataxia

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• HEMIBALLISMUS
• Throwing of the limbs on one side of the body
• Usually due to CVA involving the subthalamic
  nucleus

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• MYOCLONUS
• Simple jerky movements that are not co-ordinated
  or suppressible
• CAUSES
• Renal failure
• Hepatic failure
• Creutz feldt jacob disease
• Subacute sclerosing panencephalitis

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• DYSTONIA
• Repeated patterned twisting and sustained
  movements that may be either slow or rapid
• Involuntary movements occur before 20 years

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• Dystonia Cont.
• Disturbance of the affected muscle groups
• depend upon age
• --distally---in children
• ---cranial - cervical ---adults

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• Dystonia cont.
• Primary----focal----torticollis
• ----writers cramps
• generalized
• Secondary----wilsons disease
• ----toxins

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• ATHETOSIS
• Writhing movements
• Mainly due to cerebral palsy
• DYSKINESIA
• Tardivedrugs-gt 6 wks exposure to dopamine
  agonists
• Orofacial repeated movements

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

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

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