Stiff-man syndrome in a woman with breast cancer: an uncommon central nervous system paraneoplastic syndrome.
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.
Rosin
L
L
Department of Neurology, University of Heidelberg, Germany.
DeCamilli
P
P
Butler
M
M
Solimena
M
M
Schmitt
H P
HP
Morgenthaler
N
N
Meinck
H M
HM
D000818:Animals; D001323:Autoantibodies / Q000097:blood / Q000302:isolation & purification; D015153:Blotting, Western; D001943:Breast Neoplasms / Q000150:complications*; D018270:Carcinoma, Ductal, Breast / Q000150:complications*; D003937:Diagnosis, Differential; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology; D006801:Humans; D007124:Immunoenzyme Techniques; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000276:immunology; D010257:Paraneoplastic Syndromes / Q000150:complications* / Q000276:immunology; D011233:Precipitin Tests; D051381:Rats; D016750:Stiff-Person Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology
D001323:Autoantibodies; D009419:Nerve Tissue Proteins; C075637:amphiphysin; D005968:Glutamate Decarboxylase