Stiff-person syndrome associated with cerebellar ataxia and high glutamic acid decarboxylase antibody titer.
Glutamic acid decarboxylase (GAD) is the main target of humoral autoimmunity in patients with insulin-dependent diabetes mellitus (IDDM) and stiff-person syndrome. We reviewed the case of a 46-year-old woman who had cerebellar ataxia before getting stiff-person syndrome and IDDM with high anti-GAD autoantibody titers. This was a rare case in which there were both the clinical symptoms of stiff-person syndrome and cerebellar ataxia. In western blot analysis her serum reacted with 65-kDa proteins from rat cerebellum, cerebral cortex, and spinal cord. Autoantibodies to GAD may cause functional impairment of gamma-aminobutyric acid (GABA) neurons in the spinal cord as well as in the cerebellum.
Kono
S
S
First Department of Medicine, Hamamatsu University School of Medicine.
Miyajima
H
H
Sugimoto
M
M
Suzuki
Y
Y
Takahashi
Y
Y
Hishida
A
A
D001323:Autoantibodies / Q000097:blood*; D015153:Blotting, Western; D002524:Cerebellar Ataxia / Q000150:complications* / Q000175:diagnosis / Q000201:enzymology / Q000276:immunology; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000201:enzymology / Q000276:immunology; D004576:Electromyography; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D016750:Stiff-Person Syndrome / Q000150:complications* / Q000175:diagnosis / Q000201:enzymology / Q000276:immunology
D001323:Autoantibodies; D005968:Glutamate Decarboxylase