Two patients with multiple sclerosis developed symptomatic chronic inflammator y demyelinating polyneuropathy with massive spinal or cranial nerve hypertrophy revealed by neuroimaging. Sural nerve biopsy in one showed...Two patients with multiple sclerosis developed symptomatic chronic inflammator y demyelinating polyneuropathy with massive spinal or cranial nerve hypertrophy revealed by neuroimaging. Sural nerve biopsy in one showed only moderate demyeli nation, axonal loss, and onion-bulb formation, illustrating dichotomy between s evere proximal and milder distal nerve involvement. Patients with coexistent cen tral and peripheral demyelination usually are symptomatic from dysfunction at on e site or the other, but not from both. Our patients showed minimal response to steroids, intravenous immunoglobulin, or azathioprine. These cases suggest that the mechanism of disease in symptomatic central and peripheral demyelination may differ from that of disease in only one region, and that optimal therapy in thi s situation must be explored further.展开更多
文摘Two patients with multiple sclerosis developed symptomatic chronic inflammator y demyelinating polyneuropathy with massive spinal or cranial nerve hypertrophy revealed by neuroimaging. Sural nerve biopsy in one showed only moderate demyeli nation, axonal loss, and onion-bulb formation, illustrating dichotomy between s evere proximal and milder distal nerve involvement. Patients with coexistent cen tral and peripheral demyelination usually are symptomatic from dysfunction at on e site or the other, but not from both. Our patients showed minimal response to steroids, intravenous immunoglobulin, or azathioprine. These cases suggest that the mechanism of disease in symptomatic central and peripheral demyelination may differ from that of disease in only one region, and that optimal therapy in thi s situation must be explored further.
