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EXTRAPYRAMIDAL SYSTEM DISORDERS

Explore extrapyramidal system disorders, including Parkinson's disease, Huntington's disease, and Wilson's disease, their effects on movement, neurotransmitters, causes, clinical features, investigations, and treatment options.

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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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