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Multifocal acquired demyelinating sensory and motor neuropathy: The Lewis–Sumner syndrome
Author(s) -
Saperstein David S.,
Amato Anthony A.,
Wolfe Gil I.,
Katz Jonathan S.,
Nations Sharon P.,
Jackson Carlayne E.,
Bryan Wilson W.,
Burns Dennis K.,
Barohn Richard J.
Publication year - 1999
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/(sici)1097-4598(199905)22:5<560::aid-mus2>3.0.co;2-q
Subject(s) - multifocal motor neuropathy , mismatch negativity , medicine , chronic inflammatory demyelinating polyneuropathy , sensory system , polyneuropathy , pathology , neuroscience , antibody , immunology , psychology , electroencephalography , psychiatry
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 1 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. © 1999 John Wiley & Sons, Inc. Muscle Nerve 22: 560–566, 1999