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Rapidly Progressive Fatal Neuromyositis
A 42-year-old female with progressive quadriparesis and low vitamin B12 was admitted to GNRC hospitals, later diagnosed with inflammatory neuromyopathy and underwent various treatments without improvement. She experienced severe complications, including sudden unresponsiveness and hypotension, and ultimately died after several months of worsening symptoms. The clinical case highlighted the challenges in diagnosing and treating rapid neuromyositis with negative vasculitis and connective tissue disorder profiles.
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Rapidly Progressive Fatal Neuromyositis
- 1. Dept of Neurology GNRCHospitals Guwahati Assam, India
- 2. Ms MB 42/Ffrom Dispur admitted on 19/11/12 at GNRC Dispur with progressive weakness over all 4 limbs x 1.5 months
- 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. 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 > Distal Quadriparesis without Sensory or Autonomic involvement
- 5. Investigation ◦NCV/EMG: Diffuse axonalmotor 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. Discharged on 05/12/12(2 weeks) without improvement
- 7. December 2012 ◦ CPK:2625 ◦ EMG: Myogenic ◦ NCV: Axonal neuropathy ◦ Nerve biopsy: Chronic multifocal axonopathy with sparse inflammation – possible vasculitis ◦ Muscle biopsy: Suggestive of possible inflammatory myositis ◦ TSH: 8.18 mic IU/ml ◦ Vasulitis profile -ve
- 9. Discharged on 13/01/13 PulseCyclophosphamide first dose (1.18g x 3 d) Plasmapharesis - patient could not tolerate. IVIG - could not afford Prednisolone 50 mg daily IV Methyl Prednisolone x 7 days Diagnosed- Inflammatory Neuromyopathy. Dx & Rx at NIMHANS
- 10. Worsening ofQuadriplegia (Proximal+ Distal) with dysphagia Generalized edema over the extremities. Erythematous rashes all over her body.
- 11. LFT :Enzymes raised ↑ TC CPK (489 U/L) X Ray Chest- Right lung consolidation Viral markers: HIV, HCV, HBsAg, -ve
- 12. Antibiotic Diuretic Vit B12 Thyroxine Potassium IV Steroid: Hydrocortisone
- 13. At 7 am,07/02/13 (Day 2), suddenly became unresponsive with hypotension, and bradycardia She was immediately intubated & ventilated and shifted to ICU. Ionotropic support was provided. 5pm Died
- 14. Ms MB42/f presented with progressive Neuromyositis with Low B12, and mildly raised TSH over 5months, unresponsive to immuno-suppression.
- 17. • 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, pulmonary hemorrhage
- 21. • This casewas suffering from rapidly progressive Neuromyositis (inflammatory) with negative vasculitis and connective tissue disorder profile • Possible Differential Diagnosis 1. Anti SRP positive polymyositis with cardiomyopathy 2. ANCA negative polyarteritis nodosa 3. Paraneoplastic neuromyositis