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

作者: Jangsup Moon , Soon-Tae Lee , Jung-Won Shin , Jung-Ick Byun , Jung-Ah Lim

DOI: 10.1016/J.JNEUROIM.2014.07.011

关键词:

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

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