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Rapidly Progressive Fatal Neuromyositis

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A 42/f presented with rapidly progressive Neruo-myositis with quadriplegia unresponsive to Immunosuppression.

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Rapidly Progressive Fatal Neuromyositis

  1. 1. Dept of NeurologyGNRC Hospitals Guwahati Assam, India
  2. 2. Ms MB 42/F from Dispur admitted on19/11/12 at GNRC Dispur with progressiveweakness over all 4 limbs x 1.5 months
  3. 3.  15th September, 2012 :◦ Low grade fever with generalized weakness 24th Sept:◦ Pain in both lower limbs◦ Investigated for CBC, Urine RE, RBS, RFT,LFT, TFT, CPK –1496◦ Prednisolone 16mg tid x 10 days◦ No improvement October 2012 :◦ Gradual proximal limb weakness (UL= LL)
  4. 4.  Nov 16th◦ CBC, RFT, LFT, CPK – 28 UL, TSH – 5.16 mic IU/ml◦ NCV – Distal sensory-motor axonal neuropathy◦ MRI LS spine: Canal stenosis L4-5,S1 Nov 19th Admitted in Dispur GNRC◦ Hypotonic, Areflexic, Proximal > DistalQuadriparesis without Sensory or Autonomicinvolvement
  5. 5. Investigation◦NCV/EMG: Diffuse axonal motor neuropathy◦ CSF analysis: Normal.◦ MRI Cervical spine & Brain: Normal◦ Vitamin B12<150 pg/ml Dx:◦Subacute Inflammatory Axonal Motor Neuropathy◦B12 deficiency Rx◦IVMP 1g x 5days◦B12 1mg x 5 days -> 1mg/week◦Physiotherapy
  6. 6. Discharged on 05/12/12 (2 weeks) withoutimprovement
  7. 7. December 2012◦ CPK: 2625◦ EMG: Myogenic◦ NCV: Axonal neuropathy◦ Nerve biopsy: Chronic multifocal axonopathy withsparse inflammation – possible vasculitis◦ Muscle biopsy: Suggestive of possible inflammatorymyositis◦ TSH: 8.18 mic IU/ml◦ Vasulitis profile -ve
  8. 8. Discharged on 13/01/13Pulse Cyclophosphamide first dose (1.18g x 3 d)Plasmapharesis - patient could not tolerate.IVIG - could not affordPrednisolone 50 mg dailyIV Methyl Prednisolone x 7 daysDiagnosed- Inflammatory Neuromyopathy.Dx & Rx at NIMHANS
  9. 9.  Worsening of Quadriplegia (Proximal+ Distal)with dysphagia Generalized edema over the extremities. Erythematous rashes all over her body.
  10. 10.  LFT : Enzymes raised ↑ TC CPK (489 U/L) X Ray Chest- Right lung consolidation Viral markers: HIV, HCV, HBsAg, -ve
  11. 11.  Antibiotic Diuretic Vit B12 Thyroxine Potassium IV Steroid: Hydrocortisone
  12. 12. At 7 am, 07/02/13 (Day 2), suddenly becameunresponsive with hypotension, andbradycardiaShe was immediately intubated & ventilatedand shifted to ICU.Ionotropic support was provided.5pm Died
  13. 13.  Ms MB 42/f presented with progressiveNeuromyositis with Low B12, and mildlyraised TSH over 5months, unresponsive toimmuno-suppression.
  14. 14. • Large-vessel vasculitis– Aorta and the great vessels (subclavian, carotid)– Claudication, blindness, stroke• Medium-vessel vasculitis– Arteries with muscular wall– Mononeuritis multiplex (wrist/foot drop),mesenteric ischemia, cutaneous ulcers• Small-vessel vasculitis– Capillaries, arterioles, venules– Palpable purpura, glomerulonephritis, pulmonaryhemorrhage
  15. 15. • This case was suffering from rapidlyprogressive Neuromyositis (inflammatory)with negative vasculitis and connective tissuedisorder profile• Possible Differential Diagnosis1. Anti SRP positive polymyositis withcardiomyopathy2. ANCA negative polyarteritis nodosa3. Paraneoplastic neuromyositis

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