Combined central and peripheral demyelination
A 52-year-old male carpenter, vegetarian by diet, normotensive, non-diabetic and euthyroid presented to neurology department with history of progressive quadriparesis since 4 months. He was diagnosed having chronic active hepatitis 15 months back and was initially started on steroids and was tapered to prednisolone 5 mg/day. He attributed his weakness to hepatic ailment. He attended the neurology department when he developed dysarthria and became drowsy 2 days prior to admission.\nOn examination, patient was drowsy, but arousable, well oriented to time, place and person and would soon drift back to sleep. On general examination, he had no icterus, flaps and sphincter dysfunction or neck stiffness, but had mild pedal edema. Fundus was normal. Cranial nerve examination showed bilateral eye closure and neck flexor weakness. Tone was flaccid in the limbs; with power proximally 2-3/5 and distally 4/5. His liver function test revealed mild elevation of serum bilirubin 1.7 mg/dl. Full blood count, liver enzymes, coagulation screen, electrolytes, serum ammonia and renal function were normal. He had mild thrombocytopenia with a platelet count of 100,000 lakh/cumm.\nSerological testing for hepatitis A, hepatitis B and hepatitis C was negative. Ultrasound abdomen showed shrunken liver with irregular margins and diffusely altered echo texture suggestive of chronic liver disease. Immunological tests demonstrated positive smooth muscle antibody and positive anti-nuclear antibody homogenous staining pattern at a titre of 1 in 6. There was an accompanying hyper-gammaglobulinemia with elevated immunoglobulin G at 2507 (normal range 700-1600) and normal immunoglobulin A levels. Double stranded deoxyribonucleic acid antibodies, anti-mitochondrial antibodies and liver/kidney microsomal antibodies were not detected. Thyroid profile, serum B12, ceruloplasmin, alpha fetoprotein levels, protein electrophoresis was normal; Lupus anticoagulant, P-ANCA, C-ANCA was negative. The positive anti-nuclear and smooth muscle antibody results were strongly suggestive of autoimmune hepatitis and ruled out other autoimmune disorders. Magnetic resonance imaging of brain (T2-weighted) showed hyper intense signals in the basis pontis [] suggestive of CPM which accounted for his drowsiness. Nerve conduction studies showed prolonged distal latencies in median (5.5 ms), posterior tibial (6.6 ms) and peroneal nerves (6 ms); decreased motor conduction velocity in median (25 m/s), posterior tibial (30 m/s) and peroneal nerves (41 m/s). F waves were prolonged in median (56), post tibial nerves (70) and peroneal nerves (69). Sensory conductions were normal. The nerve conduction studies were suggestive of chronic inflammatory demyelinating polyneuropathy (CIDP).\nWith these parameters, patient was diagnosed to have central and peripheral demyelinating disorder. Patient was started on high dose methylprednisolone with meticulous glycemic control. After 10 h of admission, patient sensorium improved and started showing improvement in motor power after 5 days. On discharge, he was able to walk with minimal support on oral corticosteroids. On follow-up after 8 months, he was stable and was maintaining improvement.