Cerebellar Ataxia With Anti–Glutamic Acid Decarboxylase Antibodies

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Abstract
GLUTAMIC acid decarboxylase (GAD) is a major enzyme of the nervous system that catalyzes the conversion of glutamate to γ-aminobutyric acid (GABA). This enzyme, also expressed by pancreatic beta cells, has been identified as a dominant and essential autoantigen in the development of insulin-dependent (type 1) diabetes mellitus (IDDM).1 Anti-GAD autoantibodies (GAD-Ab) are present in up to 80% of patients with newly diagnosed IDDM and can be detected many years before the clinical onset of the disease.2 High levels of GAD-Ab are found in the serum and cerebrospinal fluid (CSF) of at least 60% of patients with the stiff-man syndrome, a rare disorder of the central nervous system characterized by progressive muscle rigidity with superimposed painful spasms.3,4 Patients with stiff-man syndrome who have GAD-Ab usually also have IDDM and other organ-specific autoimmune manifestations, suggesting that the stiff-man syndrome may have an autoimmune-mediated pathogenesis.5 The GAD-Ab of patients with stiff-man syndrome are in higher titer and have a different epitope specificity than those of patients with IDDM, suggesting that the 2 diseases may have different autoimmune mechanisms.6,7

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