Stiff-man syndrome: possible autoimmune etiology targeted against GABA-ergic cells.

作者: H. Feistner , E. Kirches , H. Wittig , K. Dietzmann , M. Warich-Kirches

DOI:

关键词: Autoimmune diseaseGlutamate decarboxylaseRenshaw cellAutoantibodyPancreasInternal medicineEndocrinologyMedicineCerebellumAtrophyCerebellar cortex

摘要: We report the case of a female patient, who died at age 66 years. Besides an insulin-dependent diabetes mellitus (IDDM) she had developed clinical symptoms stiff-man-syndrome (SMS) and harbored autoantibodies against glutamate-decarboxylase (GAD) in blood liquor. GAD catalyzes biosynthesis inhibitory neurotransmitter gamma-aminobutyric acid (GABA). The autopsy revealed typical alterations observed including incomplete fibrosis pancreatic Langerhans islets. A decrease GABA-ergic cells cerebellar cortex was observed, size reduction Renshaw spinal cord. Furthermore, dilution series polyclonal GABA antibody delivered reduced immunofluorescence cerebellum. In skeletal muscle neurogenic atrophy observed. As described literature, decayed following clonazepam administration. suggest that this autoantibodies, dramatic loss GAD-expressing cells, or secretory neurons, supports hypothesis SMS may be autoimmune disease directed cells. we neuronal hypersensitivity cord level caused by atrophic

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