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J Neuroimmunol. 2014 Sep 15;274(1-2):209-14. doi: 10.1016/j.jneuroim.2014.07.011. Epub 2014 Jul 23.

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

Author information

1
Department of Neurology, Laboratory for Neurotherapeutics, Comprehensive Epilepsy Center, Biomedical Research Institute, Seoul National University Hospital, Seoul, South Korea; Program in Neuroscience, Seoul National University College of Medicine, Seoul, South Korea.
2
Department of Neurology, Seoul Paik Hospital, Inje University College of Medicine, Seoul, South Korea.
3
Department of Neurology, Laboratory for Neurotherapeutics, Comprehensive Epilepsy Center, Biomedical Research Institute, Seoul National University Hospital, Seoul, South Korea; Program in Neuroscience, Seoul National University College of Medicine, Seoul, South Korea. Electronic address: stemcell.snu@gmail.com.

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.

KEYWORDS:

Anti-amphiphysin antibody; Immunotherapy; Paraneoplastic neurological syndrome; Stiff-person syndrome; rituximab

PMID:
25087755
DOI:
10.1016/j.jneuroim.2014.07.011
[Indexed for MEDLINE]
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