2. EXTRAPYRAMIDAL SYSTEM DISORDERS

3. BASAL GANGLION DYSFUNCTION BASAL GANGLION DYSFUNCTION

4. ANATOMY  Caudate nucleaus  Putamen  Globus pallidus  Substantia nigra  Subthalamic nuleus  Thallamus

7. EFFECTS OF DYSFUNCTION IN GENERAL  Involuntary movements  Altered movements slow slow Interrupted Interrupted Uncordinated Uncordinated  Posture and tone altered

8. MOVEMENT DISORDERS BRADYKINETIC ---TOO LITTLE  Parkinson disease  Wilsons disease  Huntingtons disease

9. HYPERKINETIC –TOO MUCH  Athetosis  Hemiballismus  Dystonia  Dyskinesia  Chorea  Myoclonus  Tremors  Tics

10. NEUROTRANSMITTERS  Dopamine > Ach = hyperkinetic  Ach > dopamine =hypokinetic

11. CAUSES OF EXTRAPYRAMIDAL DISORDERS  Drugs---chlorpromazine ---butyrophenons ---butyrophenons ---metochlorpramide ---metochlorpramide ---reserpine ---reserpine

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

13. Causes cont.  Inherited or degenerative disease  huntingtons disease  progressive supra nuclear gaze palsy {Steel Richardson} {Steel Richardson}

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

15. CLINICAL FEATURES Characterized by:  Tremors  Rigidity  bradykinesia

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

17. RIGIDITY  Leadpipe or plastic  Cogwheel BRADYKINESIA  Slow movements  Develop gradually  Impairement of fine movements

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

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

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

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

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

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

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

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

28. 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

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

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

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

32. CHOREA  Continous unsustained rapid abrupt and random contractures  Small fidgety movements  Distal muscles involved

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

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

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

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

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

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

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

40. TREMORS  Physiological  Familial  Resting—parkinsons disease  Intention or action ---cerebellar

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