Neuropathology and binding studies in anti-amphiphysin-associated stiff-person syndrome.
The authors report a 71-year-old woman with amphiphysin-associated paraneoplastic stiff-person syndrome, opsoclonus, and encephalopathy. The patient's symptoms temporarily responded to plasmapheresis in parallel with a decline of serum anti-amphiphysin antibody titers. Later, the encephalopathy progressed rapidly and the patient died. Binding studies and the detection of autoantibodies in the patient's CNS as well as the treatment response suggest a pathogenic role of the anti-amphiphysin antibodies.
Wessig
C
C
Department of Neurology, University of Würzburg, Germany.
Klein
R
R
Schneider
M F
MF
Toyka
K V
KV
Naumann
M
M
Sommer
C
C
D000368:Aged; D000818:Animals; D000937:Antigen-Antibody Reactions; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology*; D020274:Autoimmune Diseases of the Nervous System / Q000209:etiology / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D001943:Breast Neoplasms / Q000150:complications / Q000276:immunology; D018270:Carcinoma, Ductal, Breast / Q000150:complications / Q000276:immunology; D003072:Cognition Disorders / Q000188:drug therapy / Q000209:etiology / Q000276:immunology / Q000473:pathology; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D005911:Gliosis / Q000209:etiology / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D009419:Nerve Tissue Proteins / Q000276:immunology*; D015835:Ocular Motility Disorders / Q000188:drug therapy / Q000209:etiology / Q000276:immunology / Q000473:pathology; D010257:Paraneoplastic Syndromes / Q000209:etiology / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D010956:Plasmapheresis; D011149:Pons / Q000473:pathology*; D051381:Rats; D013116:Spinal Cord / Q000473:pathology*; D016750:Stiff-Person Syndrome / Q000209:etiology / Q000276:immunology* / Q000473:pathology / Q000628:therapy
D001323:Autoantibodies; D001324:Autoantigens; D007166:Immunosuppressive Agents; D009419:Nerve Tissue Proteins; C075637:amphiphysin; D003520:Cyclophosphamide