Non-stiff anti-amphiphysin syndrome: clinical manifestations and outcome after immunotherapy.

Abstract

Amphiphysin antibody causes paraneoplastic stiff-person syndrome and can also result in a variety of neurological manifestations. Here, we investigated the clinical spectrum of 20 patients with non-stiff anti-amphiphysin syndrome and their responses to immunotherapy. The most common neurological manifestation was limbic encephalitis (n=10), followed by dysautonomia (n=9), and cerebellar dysfunction (n=6). Cancer was detected in only seven patients. Intravenous immunoglobulin or steroid treatment was effective in most patients, but three improved only after rituximab treatment. Our study suggests that anti-amphiphysin syndrome can manifest as non-stiff encephalomyelitis and is only partially associated with cancer. Active immunotherapy, including rituximab, would be beneficial.

• Authors: Jangsup Moon, Soon-Tae Lee, Jung-Won Shin, Jung-Ick Byun, Jung-Ah Lim, Yong-Won Shin, Tae-Joon Kim, Keon-Joo Lee, Kyung-Il Park, Keun-Hwa Jung, Ki-Young Jung, Sang Kun Lee, Kon Chu
• Journal: Journal of neuroimmunology (J Neuroimmunol) Vol. 274 Issue 1-2 Pg. 209-14 (Sep 15 2014) ISSN: 1872-8421 [Electronic] Netherlands
• PMID: 25087755 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2014 Elsevier B.V. All rights reserved.

Chemical References

• Antibodies, Monoclonal, Murine-Derived
• Autoantibodies
• Immunoglobulins, Intravenous
• Immunologic Factors
• Nerve Tissue Proteins
• amphiphysin
• Rituximab

Topics

• Adult
• Aged
• Aged, 80 and over
• Antibodies, Monoclonal, Murine-Derived (therapeutic use)
• Autoantibodies (blood, immunology)
• Cerebellar Diseases (drug therapy, etiology, immunology)
• Encephalomyelitis (drug therapy, etiology, immunology)
• Female
• Humans
• Immunoglobulins, Intravenous (therapeutic use)
• Immunologic Factors (therapeutic use)
• Limbic Encephalitis (drug therapy, etiology, immunology)
• Male
• Middle Aged
• Neoplasms (complications)
• Nerve Tissue Proteins (immunology)
• Primary Dysautonomias (drug therapy, etiology, immunology)
• Rituximab
• Stiff-Person Syndrome (drug therapy, etiology, immunology)
• Treatment Outcome