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NEUROLOGY 1998;51:250-252
© 1998 American Academy of Neurology

Atypical stiff-person syndrome with spinal MRI findings, amphiphysin autoantibodies, and immunosuppression

K. Schmierer, MD, J. M. Valdueza, MD, A. Bender, MD, P. DeCamilli, MD, C. David, MD, M. Solimena, MD and R. Zschenderlein, MD

From the Department of Neurology (Drs. Schmierer, Valdueza, and Zschenderlein) and the Institute of Radiology, Neuroradiology Unit (Dr. Bender), Charité University Hospital, Berlin, Germany; and the Departments of Medicine (Dr. Solimena), Cell Biology, and Howard Hughes Medical Institute (Drs. DeCamilli and David), Yale University School of Medicine, New Haven, CT.

Address correspondence and reprint requests to Dr. Klaus Schmierer, Charité University Hospital, Department of Neurology, Schumannstrasse 20/21, 10098 Berlin, Germany.

In an atypical case of stiff-person syndrome (SPS), spinal T2-weighted MRI revealed a hyperintense lesion extending from C2 to C7 that corresponded with the clinical symptoms and signs. CSF showed lymphocytic pleocytosis and oligoclonal bands. Amphiphysin autoantibodies were detected In serum and CSF; however, unlike other reported cases, no malignancy occurred during a 3-year observation period. Methylprednisolone and cyclophosphamide pulse therapy led to a marked reduction of symptoms.

Received December 1, 1997. Accepted in final form April 3, 1998.




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