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NEUROLOGY 1998;50:94-98
© 1998 American Academy of Neurology

Stiff-man syndrome in a woman with breast cancer

An uncommon central nervous system paraneoplastic syndrome

L. Rosin, MD, P. DeCamilli, MD, M. Butler, MD, M. Solimena, MD, H.-P. Schmitt, MD, N. Morgenthaler, MD and H.-M. Meinck, MD

From the Department of Neurology (Drs. Rosin and Meinck), University of Heidelberg, Heidelberg, Germany; Department of Cell Biology and Howard Hughes Medical Institute (Drs. DeCamilli and Butler) and Department of Internal Medicine, Section of Endocrinology (Dr. Solimena), Yale University, New Haven, CT; Department of Neuropathology (Dr. Schmitt), University of Heidelberg, Heidelberg, Germany; and Department of Medicine III (Dr. Morgenthaler), University of Leipzig, Leipzig, Germany.

Address correspondence and reprint requests to Prof. Dr. Hans-Michael Meinck, Neurologische Universitätsklinik, Im Neuenheimerfeld 400, D 69120 Heidelberg, Germany.

We report a patient who developed stiff-man syndrome, including disabling shoulder subluxation and wrist ankylosis, in association with breast cancer. Immunologic investigations disclosed autoimmunity directed against not only glutamic acid decarboxylase but also amphiphysin, a 128-kd protein located in the presynaptic compartment of neurons. The patient improved after surgery and corticosteroid treatment and has been stable for nearly 4 years on only anti-estrogenics. The triad of stiff-man syndrome, breast cancer, and autoantibodies against amphiphysin identifies a new autoimmune paraneoplastic syndrome of the CNS.

Supported by the Volkswagenstiftung and the Heidelberg Medical Research Council.

Received July 30, 1996. Accepted in final form July 22, 1997.




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