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

1. Dept of Neurology
GNRC Hospitals Guwahati Assam, India

2. 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

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 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. 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
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

10.  Worsening of Quadriplegia (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 MB 42/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 case was 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