Autoantibodies to Glutamic Acid Decarboxylase in a Patient with Stiff-Man Syndrome, Epilepsy, and Type I Diabetes Mellitus
- 21 April 1988
- journal article
- case report
- Published by Massachusetts Medical Society in The New England Journal of Medicine
- Vol. 318 (16), 1012-1020
- https://doi.org/10.1056/nejm198804213181602
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.Keywords
This publication has 43 references indexed in Scilit:
- Long-standing "stiff-man" syndrome: a particular form of disseminated inflammatory CNS disease?Journal of Neurology, Neurosurgery & Psychiatry, 1987
- Type I Diabetes MellitusThe New England Journal of Medicine, 1986
- Resolution of Stiff-Man Syndrome with Cortisol Replacement in a Patient with Deficiencies of ACTH, Growth Hormone, and ProlactinThe New England Journal of Medicine, 1984
- Specific lysis of GABAergic synaptosomes by an antiserum to glutamate decarboxylaseFEBS Letters, 1983
- Immunohistochemical Studies of the GABA System in the PancreasNeuroendocrinology, 1983
- “Western Blotting”: Electrophoretic transfer of proteins from sodium dodecyl sulfate-polyacrylamide gels to unmodified nitrocellulose and radiographic detection with antibody and radioiodinated protein AAnalytical Biochemistry, 1981
- BRAIN-TYPICAL CREATINEKINASE IN THE SERUM OF NEWBORN INFANTS WITH PERINATAL BRAIN DAMAGEActa Paediatrica, 1978
- Sleep Studies and Therapeutic Trial with L-Dopa in a Case of Stiffman SyndromeEuropean Neurology, 1973
- Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4Nature, 1970
- The stiff‐man syndrome: Report of a caseArthritis & Rheumatism, 1960