rapidly progressive fatal neuromyositis
DESCRIPTION
A 42/f presented with rapidly progressive Neruo-myositis with quadriplegia unresponsive to Immunosuppression.TRANSCRIPT
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Rapidly progressive case of
Neuromyositis
Dept of NeurologyGNRC Hospitals Guwahati Assam, India
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Ms MB 42/F from Dispur admitted on 19/11/12 at GNRC Dispur with progressive weakness over all 4 limbs x 1.5 months
Introduction
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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)
History
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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
Hx cont..
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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
Dx,Course and Rx
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Discharged on 05/12/12 (2 weeks) without improvement
Course and Discharge
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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
Investigated at NIMHANS Bangaluru India
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Vasculitis Profile
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Discharged on 13/01/13
Pulse Cyclophosphamide 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
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Worsening of Quadriplegia (Proximal+ Distal) with dysphagia
Generalized edema over the extremities. Erythematous rashes all over her body.
Readmitted in GNRC: 06/02/13
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LFT : Enzymes raised ↑ TC CPK (489 U/L) X Ray Chest- Right lung consolidation Viral markers: HIV, HCV, HBsAg, -ve
Investigation
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Antibiotic Diuretic Vit B12 Thyroxine Potassium IV Steroid: Hydrocortisone
Rx
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At 7 am, 07/02/13 (Day 2), suddenly became unresponsive with hypotension, and bradycardiaShe was immediately intubated & ventilated and shifted to ICU. Ionotropic support was provided.5pm Died
Course
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Ms MB 42/f presented with progressive Neuromyositis with Low B12, and mildly raised TSH over 5months, unresponsive to immuno-suppression.
Summary
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Discussion
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Vasculitis: Classification
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Vasculitis: Classification
• 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
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CNS AND VASCULITIS
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Can it be a myopathy ?
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Summary
• This case was suffering from rapidly progressive Neuromyositis (inflammatory) with negative vasculitis and connective tissue disorder profile
• Possible Differential Diagnosis1. Anti SRP positive polymyositis with
cardiomyopathy2. ANCA negative polyarteritis nodosa 3. Paraneoplastic neuromyositis