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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

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EXTRAPYRAMIDAL SYSTEM DISORDERS

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  1. EXTRAPYRAMIDALSYSTEM DISORDERS

  2. BASAL GANGLIONDYSFUNCTION

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

  4. EFFECTS OF DYSFUNCTION INGENERAL • Involuntary movements • Altered movements • slow • Interrupted • Uncordinated • Posture and tone altered

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

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

  7. NEUROTRANSMITTERS • Dopamine > Ach = hyperkinetic • Ach > dopamine =hypokinetic

  8. CAUSES OF EXTRAPYRAMIDALDISORDERS • Drugs---chlorpromazine ---butyrophenons ---metochlorpramide ---reserpine

  9. Causes cont. • Toxins—CO and manganese poisoning • Inherited and metabolic disorders : • wilsons disease • spinocerebellar ataxia • Encephalitis lethargica • Diffuse small vascular disease

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

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

  12. CLINICAL FEATURES Characterized by: • Tremors • Rigidity • bradykinesia

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

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

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

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

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

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

  19. TREATMENT • Levodopa • Anticholinergic drugs • Amantadine • Dopamine agonists—bromocriptine, pergolide • COMT inhibitors (catechol-o-methyl transferase)—tolcapone • MAO –inhibitors--selegine

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

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

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

  23. 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

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

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

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

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

  28. CAUSES • Huntingtons disease • Drugs—Rx of parkinsonism , oral c.pills • SLE • Sydenhams chorea • Wilsons disease • Polycythemia • Friedricks ataxia

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

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

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

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

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

  34. ATHETOSIS • Writhing movements • Mainly due to cerebral palsy DYSKINESIA • Tardive—drugs-> 6 wks exposure to dopamine agonists • Orofacial repeated movements

  35. TREMORS • Physiological • Familial • Resting—parkinsons disease • Intention or action ---cerebellar

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

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