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

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

doi:10.1016/j.jneuroim.2014.07.011

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

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

Authors

Affiliations

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: [email protected].

PMID: 25087755
DOI: 10.1016/j.jneuroim.2014.07.011

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.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

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

Substances

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