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Neurology 2003;61:195-198
© 2003 American Academy of Neurology

Neuropathology and binding studies in anti-amphiphysin-associated stiff-person syndrome

C. Wessig, MD, R. Klein, MD, M.F. Schneider, MD, K. V. Toyka, MD, M. Naumann, MD and C. Sommer, MD

From the Departments of Neurology (Drs. Wessig, Schneider, Toyka, Naumann, and Sommer) and Neuropathology (Dr. Klein), University of Würzburg, Germany.

Address correspondence and reprint requests to Dr. Claudia Sommer, Neurologische Universitätsklinik, Josef-Schneider-Str. 11, D-97080 Würzburg, Germany; e-mail: sommer{at}mail.uni-wuerzburg.de

The authors report a 71-year-old woman with amphiphysin-associated paraneoplastic stiff-person syndrome, opsoclonus, and encephalopathy. The patient’s symptoms temporarily responded to plasmapheresis in parallel with a decline of serum anti-amphiphysin antibody titers. Later, the encephalopathy progressed rapidly and the patient died. Binding studies and the detection of autoantibodies in the patient’s CNS as well as the treatment response suggest a pathogenic role of the anti-amphiphysin antibodies.




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