Anti-myelin-associated glycoprotein (anti-MAG) neuropathy is a chronic demy elinating neuropathy with predominant involvement of large sensory fibers and de posits of IgM and complement on sural nerve myelinated fiber...Anti-myelin-associated glycoprotein (anti-MAG) neuropathy is a chronic demy elinating neuropathy with predominant involvement of large sensory fibers and de posits of IgM and complement on sural nerve myelinated fibers. We assessed the p resence of IgM deposits on skin myelinated nerve fibers and the involvement of u nmyelinated axons in anti-MAG neuropathy. Skin biopsies were performed in 14 pa tients with anti-MAG neuropathy, in 8 patients with chronic inflammatory demyel inating polyradiculoneuropathy (CIDP), and in 2 patients with IgM paraproteinemi c neuropathy. Biopsies were taken at the proximal thigh in 20 patients, at the d istal leg in 21 patients, at the proximal arm in 13 patients, and at the hand or fingertip in 10 patients. We found IgM deposits on dermal myelinated fibers in all anti-MAG neuropathy patients, with a greater prevalence at the distal site of the extremities. Deposits were located throughout the length of the fibers an d at the paranodal loops. CIDP and IgM paraproteinemic neuropathies did not show any deposit of IgM. Anti-MAG neuropathy and CIPD patients showed a decrease in epidermal nerve fiber density reflecting an associated axonal loss. In anti-MA G neuropathy,both large-and small-diameter nerve fibers are affected, and spec ific deposits of IgM are found on skin myelinated nerve fibers.展开更多
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Anti-myelin-associated glycoprotein (anti-MAG) neuropathy is a chronic demy elinating neuropathy with predominant involvement of large sensory fibers and de posits of IgM and complement on sural nerve myelinated fibers. We assessed the p resence of IgM deposits on skin myelinated nerve fibers and the involvement of u nmyelinated axons in anti-MAG neuropathy. Skin biopsies were performed in 14 pa tients with anti-MAG neuropathy, in 8 patients with chronic inflammatory demyel inating polyradiculoneuropathy (CIDP), and in 2 patients with IgM paraproteinemi c neuropathy. Biopsies were taken at the proximal thigh in 20 patients, at the d istal leg in 21 patients, at the proximal arm in 13 patients, and at the hand or fingertip in 10 patients. We found IgM deposits on dermal myelinated fibers in all anti-MAG neuropathy patients, with a greater prevalence at the distal site of the extremities. Deposits were located throughout the length of the fibers an d at the paranodal loops. CIDP and IgM paraproteinemic neuropathies did not show any deposit of IgM. Anti-MAG neuropathy and CIPD patients showed a decrease in epidermal nerve fiber density reflecting an associated axonal loss. In anti-MA G neuropathy,both large-and small-diameter nerve fibers are affected, and spec ific deposits of IgM are found on skin myelinated nerve fibers.
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.
