Autoantibodies to glutamic acid, decarboxylase in a patient with stiff-man syndrome, epilepsy, and Type I diabetes mellitus

M. Solimena, F. Folli, S. Denis-Donini, G. C. Comi, G. Pozza, P. De Camilli, A. M. Vicari

Research output: Contribution to journalArticlepeer-review

Abstract

Stiff-man syndrome is a rare disorder of the central nervous system consisting of progressive, fluctuating muscle rigidity with painful spasms. It is occasionally associated with endocrine disorders, including insulin-dependent diabetes, and with epilepsy. We investigated the possible existence of autoimmunity against the nervous system in a patient with stiff-man syndrome associated with epilepsy and Type I diabetes mellitus. Levels of IgG, which had an oligoclonal pattern, were elevated in the cerebrospinal fluid. The serum and the cerebrospinal fluid produced an identical, intense staining of all gray-matter regions when used to stain brain sections according to an indirect light-microscopical immunocytochemical procedure. The staining patterns were identical to those produced by antibodies to glutamic acid decarboxylase (the enzyme responsible for the synthesis of gamma-aminobutyric acid). A band comigrating with glutamic acid decarboxylase in sodium dodecyl sulfate-polyacrylamide gels appeared to be the only nervous-tissue antigen recognized by cerebrospinal fluid antibodies, and the predominant antigen recognized by serum antibodies. These findings support the idea that an impairment of neuronal pathways that operate through gamma-aminobutyric acid is involved in the pathogenesis of stiff-man syndrome, and they raise the possibility of an autoimmune pathogenesis.

Original languageEnglish
Pages (from-to)1012-1020
Number of pages9
JournalNew England Journal of Medicine
Volume318
Issue number16
Publication statusPublished - 1988

ASJC Scopus subject areas

  • Medicine(all)

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