Abstract
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.
Publication types
-
Case Reports
-
Research Support, Non-U.S. Gov't
MeSH terms
-
Animals
-
Autoantibodies / blood
-
Autoantibodies / isolation & purification
-
Blotting, Western
-
Breast Neoplasms / complications*
-
Carcinoma, Ductal, Breast / complications*
-
Diagnosis, Differential
-
Female
-
Glutamate Decarboxylase / immunology
-
Humans
-
Immunoenzyme Techniques
-
Middle Aged
-
Nerve Tissue Proteins / immunology
-
Paraneoplastic Syndromes / complications*
-
Paraneoplastic Syndromes / immunology
-
Precipitin Tests
-
Rats
-
Stiff-Person Syndrome / diagnosis
-
Stiff-Person Syndrome / etiology*
-
Stiff-Person Syndrome / immunology
Substances
-
Autoantibodies
-
Nerve Tissue Proteins
-
amphiphysin
-
Glutamate Decarboxylase