title: 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.affiliation: 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.
authors.forename: Jangsup
authors.initials: J
authors.lastname: Moon
authors.affiliation: 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.
authors.forename: Soon-Tae
authors.initials: ST
authors.lastname: Lee
authors.affiliation: 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.
authors.forename: Jung-Won
authors.initials: JW
authors.lastname: Shin
authors.affiliation: 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.
authors.forename: Jung-Ick
authors.initials: JI
authors.lastname: Byun
authors.affiliation: 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.
authors.forename: Jung-Ah
authors.initials: JA
authors.lastname: Lim
authors.affiliation: 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.
authors.forename: Yong-Won
authors.initials: YW
authors.lastname: Shin
authors.affiliation: 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.
authors.forename: Tae-Joon
authors.initials: TJ
authors.lastname: Kim
authors.affiliation: 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.
authors.forename: Keon-Joo
authors.initials: KJ
authors.lastname: Lee
authors.affiliation: Department of Neurology, Seoul Paik Hospital, Inje University College of Medicine, Seoul, South Korea.
authors.forename: Kyung-Il
authors.initials: KI
authors.lastname: Park
authors.affiliation: 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.
authors.forename: Keun-Hwa
authors.initials: KH
authors.lastname: Jung
authors.affiliation: 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.
authors.forename: Ki-Young
authors.initials: KY
authors.lastname: Jung
authors.affiliation: 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.
authors.forename: Sang Kun
authors.initials: SK
authors.lastname: Lee
authors.affiliation: 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.
authors.forename: Kon
authors.initials: K
authors.lastname: Chu
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D002526:Cerebellar Diseases / Q000188:drug therapy / Q000209:etiology / Q000276:immunology; D004679:Encephalomyelitis / Q000188:drug therapy / Q000209:etiology / Q000276:immunology; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D020363:Limbic Encephalitis / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000150:complications; D009419:Nerve Tissue Proteins / Q000276:immunology*; D054969:Primary Dysautonomias / Q000188:drug therapy / Q000209:etiology / Q000276:immunology; D000069283:Rituximab; D016750:Stiff-Person Syndrome / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology*; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D009419:Nerve Tissue Proteins; C075637:amphiphysin; D000069283:Rituximab
keywords: Anti-amphiphysin antibody; Immunotherapy; Paraneoplastic neurological syndrome; Stiff-person syndrome; rituximab