Multifocal acquired demyelinating sensory and motor neuropathy: The Lewis-Sumner syndrome

David S. Saperstein, Anthony A. Amato, Gil I. Wolfe, Jonathan S. Katz, Sharon P. Nations, Carlayne E. Jackson, Wilson W. Bryan, Dennis K. Burns, Richard J. Barohn

Research output: Contribution to journalArticle

214 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)560-566
Number of pages7
JournalMuscle and Nerve
Volume22
Issue number5
DOIs
StatePublished - 1999

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Demyelinating Diseases
Prednisone
Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Intravenous Immunoglobulins
Neural Conduction
Cerebrospinal Fluid
Anti-Idiotypic Antibodies
Biopsy
Therapeutics
Proteins

Keywords

  • Chronic inflammatory demyelinating polyneuropathy
  • Conduction block
  • Lewis-Sumner syndrome
  • Mononeuropathy multiplex
  • Multifocal motor neuropathy

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Multifocal acquired demyelinating sensory and motor neuropathy : The Lewis-Sumner syndrome. / 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.

In: Muscle and Nerve, Vol. 22, No. 5, 1999, p. 560-566.

Research output: Contribution to journalArticle

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. / Multifocal acquired demyelinating sensory and motor neuropathy : The Lewis-Sumner syndrome. In: Muscle and Nerve. 1999 ; Vol. 22, No. 5. pp. 560-566.
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