Spinocerebellar Ataxia Type 31 Exacerbated by Anti-amino Terminal of Alpha-enolase Autoantibodies

Satoshi Zeniya; Nobuo Sanjo; Hiroya Kuwahara; Kinya Ishikawa; Miwa Higashi; Akiko Matsunaga; Makoto Yoneda; Hidehiro Mizusawa; Takanori Yokota

doi:10.2169/internalmedicine.8883-21

CASE REPORTS

Spinocerebellar Ataxia Type 31 Exacerbated by Anti-amino Terminal of Alpha-enolase Autoantibodies

Satoshi Zeniya, Nobuo Sanjo, Hiroya Kuwahara, Kinya Ishikawa, Miwa Higashi, Akiko Matsunaga, Makoto Yoneda, Hidehiro Mizusawa, Takanori Yokota

Author information

Satoshi Zeniya
Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Japan
Nobuo Sanjo
Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Japan
Hiroya Kuwahara
Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Japan
Kinya Ishikawa
The Center for Personalized Medicine for Healthy Aging, Tokyo Medical and Dental University, Japan
Miwa Higashi
Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Japan
Akiko Matsunaga
Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
Makoto Yoneda
Faculty of Nursing and Social Welfare Sciences, Fukui Prefectural University, Japan
Hidehiro Mizusawa
National Center Hospital, National Center of Neurology and Psychiatry, Japan
Takanori Yokota
Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Japan

Keywords: spinocerebellar ataxia, hashimoto's encephalopathy, anti-amino terminal of alpha-enolase antibody, autoimmune cerebellar ataxia, immune-mediated cerebellar ataxia

JOURNAL OPEN ACCESS

2022 Volume 61 Issue 18 Pages 2793-2796

DOI https://doi.org/10.2169/internalmedicine.8883-21

Details

Abstract

We herein report a 61-year-old woman who was genetically diagnosed with spinocerebellar ataxia type 31 whose symptoms were modified by anti-amino terminal of alpha-enolase (NAE) antibodies, known as a biomarker of Hashimoto's encephalopathy (HE), and ultimately responded to immunotherapy. The relative titers of anti-NAE antibodies increased when her cerebellar ataxia showed acute deterioration and decreased after immunotherapy. This is the first report of cerebellar ataxia associated with genetic spinocerebellar ataxia with concomitant cerebellar type HE. Physicians should be mindful of measuring anti-NAE antibodies to prevent overlooking patients with genetic spinocerebellar ataxia with treatable simultaneous ataxic diseases.

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