TY - JOUR
T1 - Multifocal acquired demyelinating sensory and motor neuropathy
T2 - The Lewis-Sumner syndrome
AU - Saperstein, David S.
AU - Amato, Anthony A.
AU - Wolfe, Gil I.
AU - Katz, Jonathan S.
AU - Nations, Sharon P.
AU - Jackson, Carlayne E.
AU - Bryan, Wilson W.
AU - Burns, Dennis K.
AU - Barohn, Richard J.
PY - 1999
Y1 - 1999
N2 - We report 11 patients with multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy, defined clinically by a multifocal pattern of motor and sensory loss, with nerve conduction studies showing conduction block and other features of demyelination. The clinical, laboratory, and histological features of these patients were contrasted with those of 16 patients with multifocal motor neuropathy (MMN). Eighty-two percent of MADSAM neuropathy patients had elevated protein concentrations in the cerebrospinal fluid, compared with 9% of the MMN patients (P < 0.001). No MADSAM neuropathy patient had elevated anti-GM, antibody titers, compared with 56% of MMN patients (P < 0.01). In contrast to the subtle abnormalities described for MMN, MADSAM neuropathy patients had prominent demyelination on sensory nerve biopsies. Response to intravenous immunoglobulin treatment was similar in both groups (P = 1.0). Multifocal motor neuropathy patients typically do not respond to prednisone, but 3 of 6 MADSAM neuropathy patients improved with prednisone. MADSAM neuropathy more closely resembles chronic inflammatory demyelinating polyneuropathy and probably represents an asymmetrical variant. Given their different clinical patterns and responses to treatment, it is important to distinguish between MADSAM neuropathy and MMN.
AB - We report 11 patients with multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy, defined clinically by a multifocal pattern of motor and sensory loss, with nerve conduction studies showing conduction block and other features of demyelination. The clinical, laboratory, and histological features of these patients were contrasted with those of 16 patients with multifocal motor neuropathy (MMN). Eighty-two percent of MADSAM neuropathy patients had elevated protein concentrations in the cerebrospinal fluid, compared with 9% of the MMN patients (P < 0.001). No MADSAM neuropathy patient had elevated anti-GM, antibody titers, compared with 56% of MMN patients (P < 0.01). In contrast to the subtle abnormalities described for MMN, MADSAM neuropathy patients had prominent demyelination on sensory nerve biopsies. Response to intravenous immunoglobulin treatment was similar in both groups (P = 1.0). Multifocal motor neuropathy patients typically do not respond to prednisone, but 3 of 6 MADSAM neuropathy patients improved with prednisone. MADSAM neuropathy more closely resembles chronic inflammatory demyelinating polyneuropathy and probably represents an asymmetrical variant. Given their different clinical patterns and responses to treatment, it is important to distinguish between MADSAM neuropathy and MMN.
KW - Chronic inflammatory demyelinating polyneuropathy
KW - Conduction block
KW - Lewis-Sumner syndrome
KW - Mononeuropathy multiplex
KW - Multifocal motor neuropathy
UR - http://www.scopus.com/inward/record.url?scp=0032959118&partnerID=8YFLogxK
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U2 - 10.1002/(SICI)1097-4598(199905)22:5<560::AID-MUS2>3.0.CO;2-Q
DO - 10.1002/(SICI)1097-4598(199905)22:5<560::AID-MUS2>3.0.CO;2-Q
M3 - Article
C2 - 10331353
AN - SCOPUS:0032959118
SN - 0148-639X
VL - 22
SP - 560
EP - 566
JO - Muscle and Nerve
JF - Muscle and Nerve
IS - 5
ER -