Chorea and ballismus

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Chorea and ballismus

  1. 1. CHOREA AND BALLISMUS Dr PS Deb Director Neurology GNRC Hospitals Assam Guwahati
  2. 2. CHOREA  State of excessive, spontaneous movement, irregularly timed, non repetitive, randomly distributed and abnormal in character.  It may very in severity from restlessness with mild intermittent exaggeration of gesture and expression, fidgeting movement of hands unstable, movement of hands.  Unstable gait to continuous flow of disabling violent movement.  Random – distribution, time, and duration
  3. 3. CHOREA  Associated with  Hypotonia – Reduced long latency reflex  Motor impersistence → inability to sustain vol. movement  Pronator sign  Milkmaids sign  Trombone tongue  Hung-up reflex – Prolonged contraction of stretched muscles by late sensory provoked choric movement.
  4. 4. PATHOPHYSIOLOGY Stiatum Pallidum ThalamusPC PR SN Cortex Cord Chorea DA GABA GABA GABA GABA
  5. 5. CHOREA MECHANISM - NEUROTRANSMITTER DISTURBANCE  Reduced: GABA, Sub P, Ach  Increased: Dopamine, NA in Striatum and pallidum, Somatostatine  GABA in SN and GP due to degeneration of striatum, but gabargic drugs does not reduce chorea  Dopamine Tyrosin hydroxylase enzyme in SN, chorea reduced by anti dopa drugs and by Ldopa  Ach marginally reduced, choline esterase enzyme mild reduced, large striatal interneuron well maintained, Ach agonist does not improve chorea
  6. 6. CHOREA PATHOGENESIS  PET -  Histochemistry  Drug effect  Surgical  Chorea reduced by Pallidotomy, Nigrotomy, Thalamotomy  Pathological  Animal Model
  7. 7. ELECTROPHYSIOLOGICAL  Choric discharges on EMG resemble normal voluntary contraction  Active inhibition of innervation  Readiness potential not tested id Idea Programming Execution Discharge DischargeChorea Association Cortex Basal Ganglia Motor Cortex
  8. 8. MECHANISM OF CHOREA  Chorea appears to be a fragments of normal movements, appearing in inappropriate circumstances and lacking any purpose  They may be determined by peripheral stimuli which in ordinary circumstances would be ignored.  Basal ganglia normally filter the mass of cortical input, they receive to select movement appropriate to the circumstances.  Striatal damage might prevent normal suppression of unwanted motor response to external stimuli → Chorea
  9. 9. CAUSE OF CHOREA AND CHOREO-ATHETOSIS 1. Trauma 1. Concussion 2. Neoplasm 1. Primary brain tumor 2. Metastasis 3. CNS leukemia 3. Cerebrovascular disease 1. Epidural subdural hemorrhage 2. Hemorrhage 3. Lacunar infarct 4. AVM 5. Polycythemia 6. Migraine 4. Infection 1. Post streptococcal 2. Typhoid 3. Pertusis 4. Dyphtheria 5. Neurosyphilis 6. Tuberculosis 7. Mycoplasma 5. Viral infection 1. Exanthema 2. Encephalitis 6. Collagen vascular disease 1. MSV
  10. 10. CAUSE OF CHOREA AND CHOREO-ATHETOSIS CONT…. 7. Metabolic 1. Electrolyte imbalance 2. Glucose metabolic disorder 3. Endocrinal – Thyroid, Adrenal 4. Copper metabolic impairment – Wilson 5. Renal failure 6. Nutritional – thiamine, Niacin, B12 8. Intoxication 1. Alcohol 2. CO 3. Mercury 4. Manganese 5. Thallium 6. Glue sniffing 9. Drugs 1. Neurolaptics 2. Dopa agonists 3. Anti cholinergic 4. Sympathomimetics 5. Steroid, estrogen 6. Opiates 7. INH 8. Reserpine 9. Anti-histaminics 10. Tricycle antidepressants 11. Lithium 12. Metaclopromide 13. Digoxin 14. Lithium 15. Diazoxide
  11. 11. CAUSE OF CHOREA AND CHOREO-ATHETOSIS CONT…. 10. Hereditary 1. Aminoaciduria 2. Lipid 3. Glucose 4. Protein metabolic 5. Huntington’s chorea 6. Benign familial chorea 7. Chorea with Acanthocytosis 8. Familial inverted choreoathetosis 9. Familial striatal necrosis 10. Familial basal ganglionic calcification 11. Spinocerebellar degeneration 11. Other 1. Hallevorden spatz disease 2. Ataxia Telengectasia 3. Tuberous sclerosis 4. Stuge Weber Syndrome 5. Myoclonus epilepsy with chorea 6. Paraxysmal dystonic choreoathetosis 7. Kernicterus 8. Cerebral palsy 9. Electrical injury 10. Thalamic dementia
  12. 12. THERAPY  Drugs  Dopamine antagonist  Haloperidol  Tetrabenazine  Pimozide  Perphenazine  Cholinergic drugs  Lecithine → ↑ cerebral choline  GABA agonist  INH  Sod. Valproate
  13. 13. SURGICAL THERAPY
  14. 14. BALLISM Proximal, flinging, violent, involuntary movement
  15. 15. MECHANISM OF BALLISMUS  Surgical Pallidotomy, Nigrotomy, thalamotomy reduces ballismus  Animal Substantia Nigra lesion causes hemiballismus  Experimental destruction of Striata nigra no ballismus, 20% destruction → hemiballismus  Striatal dopamine increased  Subthalamic GABA reduced
  16. 16. SITE OF LESION CAUSES BALLISMUS  Sbuthalamus  Pallidum  Substantia nigra  Thalamus  Post. Central gyrus  Superior frontal gyrus  Precentral gyrus
  17. 17. PATHOPHYSIOLOGY Subthalamic.n eucli Pallidum ThalamusPC PR SN Cortex Cord Ballismus GABA GABA GABA GABA
  18. 18. ETIOLOGY BALLISMUS  Vascular – commonest  Lacunar infarct, TIA  Hemorrhage  Subarachnoid hemorrhage  AVM  Venous angioma  Tumor  Secondaries  Cyst  Infection  Tuberculloma, TBM  Syphilis  Metabolic  Hyperglycemia  Drugs  Contraceptive  L-dopa  Truama  Head injury  Post surgical (Parkinson disease)  Multiple sclerosis
  19. 19. TYPES  Monoballismus  Hemiballismus  Paraballismus  Biballismus  Prognosis  Variable – few days to years
  20. 20. TREATMENT  Drug  Dopamine antagonists  Halloparidol  Tetrabenazine  Thiopropazate  Pimozide  Perphenazine  GABAargic  Sod. Valproate  Benzodiazepine  Diazepam  Clonazepam  Surgical –  Pallidotomy,  Nigrotomy,  Thalamotomy
  21. 21. THANKS

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