A Case of Hemichorea

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A Case of Hemichorea

  1. 1. PHYSICIANS MEET <ul><li>Prof T.V.K’S UNIT </li></ul><ul><li>Dr.D.Premkumar </li></ul>
  2. 2. HISTORY <ul><li>A 85yrs old lady admitted with complaints of spontaneous movements of left upper and left lower limb- one weeks </li></ul><ul><li>Involuntary movements- one week,sudden in onset,involving left upper and left lower limb,fast flinging movement, increased during emotions, decreased during rest. </li></ul>
  3. 3. <ul><li>No H/O--- Loss of consciousness, headache, vomiting, visual disturbance, weakness, seizures,cranial nerve,sensory involvement, bladder bowel disturbance,speech disturbance. </li></ul><ul><li>No H/O--- Fever, joint pain, skin lesions </li></ul><ul><li>No H/O---Chest pain,palpitation, syncope, dyspnoea </li></ul><ul><li>No H/O---Vomiting, loose stools, abdominal pain, abdominal distension </li></ul>
  4. 4. <ul><li>No H/O trauma, TIA,RIND. </li></ul><ul><li>PAST HISTORY: History of previous similar episodes present- one month back. </li></ul><ul><li>No H/O chronic fever, Rheumatic fever, dog bite,vaccination, chronic drug intake. </li></ul><ul><li>Not-DM,SHT, CAD, BA, PT. </li></ul><ul><li>PERSONAL HISTORY : Not a smoker, alcoholic, tobacco chewing, </li></ul><ul><li>No similar episodes in the family. </li></ul>
  5. 5. EXAMINATION <ul><li>GENERAL EXAMINATION : </li></ul><ul><li>Patient conscious,co-operative </li></ul><ul><li>afebrile </li></ul><ul><li>moderately built& nourished </li></ul><ul><li>no pallor, not icteric </li></ul><ul><li>no-cyanosis, clubbing </li></ul><ul><li>no-pedal edema, lymphadenopathy </li></ul><ul><li>No Neurocutaneous Markers </li></ul>
  6. 6. <ul><li>VITALS </li></ul><ul><li>pulse rate-84/min </li></ul><ul><li>Blood pressure-130/80 </li></ul><ul><li>respiratory rate-24/min </li></ul>
  7. 7. SYSTEMIC EXAMINATION <ul><li>CENTRAL NERVOUS SYSTEM </li></ul><ul><li>Higher motor function: Normal </li></ul><ul><li>Cranial nerves examination:Normal </li></ul><ul><li>Motor system: </li></ul><ul><li>bulk-normal </li></ul><ul><li>tone- slightly reduced </li></ul><ul><li>power-4+/5 </li></ul><ul><li>reflexes- normal, Plantar- b/l flexor </li></ul><ul><li>co-ordination-normal </li></ul>
  8. 8. <ul><li>Involuntary movements- non-rhythmic,rapid,flinging type of movement is present in the left upper and lower limb,which is aggravated during activity& emotion and decreased or absent during rest and sleep. </li></ul>
  9. 9. <ul><li>Sensory system- intact </li></ul><ul><li>Cerebellum- intact </li></ul><ul><li>Autonomic nervous system-no bladder bowel disturbance </li></ul><ul><li>Fundus – normal </li></ul><ul><li>Other systems - CVS, RS, ABDOMEN- Normal </li></ul>
  10. 10. investigations <ul><li>Hb %-9.7gm% </li></ul><ul><li>Total count-8200 </li></ul><ul><li>DC:P68,L30,E2 </li></ul><ul><li>ESR-5/10 </li></ul><ul><li>Platelet count-2.5lakhs </li></ul><ul><li>MCV-88.1 </li></ul><ul><li>MCH-29.0 </li></ul><ul><li>MCHC-32.9 </li></ul><ul><li>PCV-29.2 </li></ul>
  11. 11. <ul><li>RBS-122mg% </li></ul><ul><li>Urea-38 </li></ul><ul><li>Creatinine-1.1 </li></ul><ul><li>S.Electrolytes:Na-144,K+-4.5,HCO3-22, CI-98 </li></ul><ul><li>Urine Examination:alb-nil, sugar-nil, deposits-2-3 pus cells HPF </li></ul><ul><li>ECG-NSR </li></ul><ul><li>CXR-NAD </li></ul>
  12. 12. <ul><li>CT BRAIN- normal </li></ul><ul><li>MRI BRAIN - Degenerative changes in the CAUDATE NUCLEUS and PUTAMEN. </li></ul>
  13. 13. MRI BRAIN
  14. 14. MRI BRAIN
  15. 15. MRI BRAIN
  16. 16. OPINIONS <ul><li>Neurologist opinion : HEMICHOREA </li></ul><ul><li>Cardiologist opinion :mild AV sclerosis, no </li></ul><ul><li>RWMA, normal LV function </li></ul>
  17. 17. DIAGNOSIS <ul><li>This is a case of involuntary movement affecting the left side of the body without signs of cerebrovascular disease, rheumatic fever- HEMICHOREA (Senile Chorea) </li></ul>
  18. 18. <ul><li>Treatment : Haloperidol 1mg BD Trihexyphenidyl </li></ul>
  19. 19. HYPERKINETIC MOVEMENT DISORDERS <ul><li>CHOREA/HEMICHOREA </li></ul><ul><li>HEMIBALLISMUS </li></ul><ul><li>ATHETOSIS </li></ul><ul><li>CHOREO-ATHETOSIS </li></ul><ul><li>DYSTONIAS </li></ul><ul><li>MYOCLONUS </li></ul><ul><li>FASCICULATIONS </li></ul>
  20. 20. CHOREA <ul><li>Semi-purposive, flowing,irregular, non-repatitive and brief,jerky movement arising in the proximal joints and apper to flit from one part of the body to another randomly. </li></ul><ul><li>Absent during sleep and increased on attempting voluntary movement. </li></ul><ul><li>Due to lesion in CAUDATE NUCLEUS. </li></ul>
  21. 21. Causes of chorea <ul><li>Hereditary: 1.Huntington’s disease 2.Neuroacanthocytosis 3.Benign hereditary/senile chorea 4.Wilson’s disease 5.Hallervorden-Spatz syndrome </li></ul><ul><li>Post-infectious/autoimmune: 1.syndenham’s chorea </li></ul>
  22. 22. <ul><li>2.SLE 3.AIDS </li></ul><ul><li>Endocrinology </li></ul><ul><li>1.hyperthyroidism </li></ul><ul><li>2.chorea gravidarum </li></ul><ul><li>Drug induced Chorea </li></ul><ul><li>1.OCP </li></ul><ul><li>2.Levodopa </li></ul>
  23. 23. <ul><li>3. Anticonvulsants </li></ul><ul><li>4.neuroleptics </li></ul><ul><li>5.Antidepreesants </li></ul><ul><li>Vascular </li></ul><ul><li>1.Strokes </li></ul><ul><li>2.Polycythemia vera </li></ul><ul><li>Others </li></ul><ul><li>Neoplastic/metabolic </li></ul>
  24. 24. Signs elicitale in chorea <ul><li>‘ Jack in the box’tongue </li></ul><ul><li>Respiratory irregularity </li></ul><ul><li>Milk maid sign </li></ul><ul><li>Hung up reflex </li></ul><ul><li>Pronator sign </li></ul>
  25. 25. study <ul><li>Piccolo et al studied 51 cases of sporadic chorea. On follow up only 20 cases had chorea at the end of six months. </li></ul><ul><li>Analysis revealed 1.vascular related-21 cases 2.vasculitis-2 cases 3.hypoxia-2 cases 4.drug induced- 7 cases 5.AIDS-related-5 cases </li></ul>
  26. 26. <ul><li>6.Borreliosis-1 case 7.Syndenham’s Chorea-1 case 8.Hyperglycemia-2 cases 9.Hyponatremia-2 cases 10.Huntington’s chorea-5 cases 11.Neuroacanthocytosis-1 case 12.No causes found-3 cases </li></ul>
  27. 27. Areas involved <ul><li>Chorea- caudate nucleus </li></ul><ul><li>Athetosis-putamen </li></ul><ul><li>Hemiballismus-subthalamic nucleus </li></ul><ul><li>Dystonias-putamen </li></ul>
  28. 28. Hemiballismus <ul><li>Usually affects proximal joint of one arm resulting in wild,rapid,flinging movement of wide radius,occuring constantly, interspersed with short period of freedom. </li></ul><ul><li>Absent during sleep </li></ul><ul><li>Increased tone,reflexes </li></ul><ul><li>Sub thalamic nucleus </li></ul>
  29. 29. Athetosis <ul><li>Slow writhing movement,best seen at wrist, fingers and ankle. </li></ul><ul><li>Lesion in putamen </li></ul><ul><li>Absent during sleep </li></ul>
  30. 30. Dystonias <ul><li>Idiopathic/primary torsion dystonias is a disorder charecterised by involuntary sustained muscle contractions frequently causing twisting and repetitive movements or abnormal posture </li></ul><ul><li>Types-generalized, segmental,focal </li></ul>
  31. 31. <ul><li>Common causes of secondary dystonias 1.drugs-phenothiazine 2.Wilsons disease 3.Degenerative ataxias 4.kernicterus 5.sickle cell disease 6.stroke,tumor,trauma </li></ul>
  32. 32. Myoclonus <ul><li>Consists ofrapid,brief shock like muscle jerks which are often repetitive, sometimes rhythmical </li></ul><ul><li>Normal phenomenon or may be a manifestation of major seizures </li></ul>
  33. 33. Fasciculations <ul><li>Irregular non-rhythmical contraction of muscle fascicles. </li></ul><ul><li>Best seen in large muscles(deltoid/calf muscle) </li></ul><ul><li>Present at rest and may be increased after voluntary movement </li></ul><ul><li>Sign of LMN lesion </li></ul><ul><li>Precipitated – contracting the musce, hyperventilation, cooling the muscle </li></ul>
  34. 34. <ul><li>Causes of fasciculation 1.MND 2.Syringomyelia 3.Cervical spondylosis 4.PMA </li></ul><ul><li>5.Thyrotoxic myopathy </li></ul><ul><li>6.carcinomatous myopathy </li></ul><ul><li>7.OPC poisoning </li></ul>
  35. 35. <ul><li>Presence of fasciculation excludes myopathy except thyrotoxic and carcinomatous myopathies </li></ul>
  36. 36. TREATMENT <ul><li>CHOREA Huntington’s- no specific, phenothiazines or haloperidol, reserpine Syndenham’s chorea- Dopamine receptor blockers and anti-convulsants(valproic acid,carbamezapine) </li></ul><ul><li>Wilson’s disease- D-Penicillamine & trientin&zinc </li></ul><ul><li>HEMIBALLISMUS: haloperdol,clonazepam,baclofen </li></ul>
  37. 37. <ul><li>Dystonias :anticholinergics,BDZ,Baclofen botulinum toxin </li></ul><ul><li>Myoclonus : valproic acid, clonazepam, oxitriptan </li></ul>
  38. 38. thank you

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