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 disabl ing shoulder subluxation and wrist ankylosis, in association with brea st cancer. Immunologic investigations disclosed autoimmunity directed against not only glutamic acid decarboxylase but also amphiphysin, a 1 28-kd protein located in the presynaptic compartment of neurons. The p atient improved after surgery and corticosteroid treatment and has bee n stable for nearly 4 years on only anti-estrogenics. The triad of sti ff-man syndrome, breast cancer, and autoantibodies against amphiphysin identifies a new autoimmune paraneoplastic syndrome of the CNS.