rapidly progressive fatal neuromyositis

Rapidly progressive case of

Neuromyositis

Dept of NeurologyGNRC Hospitals Guwahati Assam, India

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

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

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..

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

Discharged on 05/12/12 (2 weeks) without improvement

Course and Discharge

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

Vasculitis Profile

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

Worsening of Quadriplegia (Proximal+ Distal) with dysphagia

Generalized edema over the extremities. Erythematous rashes all over her body.

Readmitted in GNRC: 06/02/13

LFT : Enzymes raised ↑ TC CPK (489 U/L) X Ray Chest- Right lung consolidation Viral markers: HIV, HCV, HBsAg, -ve

Investigation

Antibiotic Diuretic Vit B12 Thyroxine Potassium IV Steroid: Hydrocortisone

Rx

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

Ms MB 42/f presented with progressive Neuromyositis with Low B12, and mildly raised TSH over 5months, unresponsive to immuno-suppression.

Summary

Discussion

Vasculitis: Classification

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

CNS AND VASCULITIS

Can it be a myopathy ?

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