Neuropathology and binding studies in anti-amphiphysin-associated stiff-person syndrome

Neurology. 2003 Jul 22;61(2):195-8. doi: 10.1212/01.wnl.0000073143.53337.dd.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Animals
  • Antigen-Antibody Reactions
  • Autoantibodies / blood
  • Autoantibodies / immunology*
  • Autoantigens / immunology*
  • Autoimmune Diseases of the Nervous System / etiology
  • Autoimmune Diseases of the Nervous System / immunology*
  • Autoimmune Diseases of the Nervous System / pathology
  • Autoimmune Diseases of the Nervous System / therapy
  • Breast Neoplasms / complications
  • Breast Neoplasms / immunology
  • Carcinoma, Ductal, Breast / complications
  • Carcinoma, Ductal, Breast / immunology
  • Cognition Disorders / drug therapy
  • Cognition Disorders / etiology
  • Cognition Disorders / immunology
  • Cognition Disorders / pathology
  • Combined Modality Therapy
  • Cyclophosphamide / therapeutic use
  • Disease Progression
  • Fatal Outcome
  • Female
  • Gliosis / etiology
  • Gliosis / pathology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Magnetic Resonance Imaging
  • Nerve Tissue Proteins / immunology*
  • Ocular Motility Disorders / drug therapy
  • Ocular Motility Disorders / etiology
  • Ocular Motility Disorders / immunology
  • Ocular Motility Disorders / pathology
  • Paraneoplastic Syndromes / etiology
  • Paraneoplastic Syndromes / immunology*
  • Paraneoplastic Syndromes / pathology
  • Paraneoplastic Syndromes / therapy
  • Plasmapheresis
  • Pons / pathology*
  • Rats
  • Spinal Cord / pathology*
  • Stiff-Person Syndrome / etiology
  • Stiff-Person Syndrome / immunology*
  • Stiff-Person Syndrome / pathology
  • Stiff-Person Syndrome / therapy

Substances

  • Autoantibodies
  • Autoantigens
  • Immunosuppressive Agents
  • Nerve Tissue Proteins
  • amphiphysin
  • Cyclophosphamide