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Spinocerebellar Ataxia Type 31 Exacerbated by Anti-amino Terminal of Alpha-enolase Autoantibodies |
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| Authors: | Satoshi Zeniya Nobuo Sanjo Hiroya Kuwahara Kinya Ishikawa Miwa Higashi Akiko Matsunaga Makoto Yoneda Hidehiro Mizusawa Takanori Yokota |
| Affiliation: | 1.Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Japan; 2.The Center for Personalized Medicine for Healthy Aging, Tokyo Medical and Dental University, Japan; 3.Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan; 4.Faculty of Nursing and Social Welfare Sciences, Fukui Prefectural University, Japan; 5.National Center Hospital, National Center of Neurology and Psychiatry, Japan |
| 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. |
| Keywords: | spinocerebellar ataxia hashimoto''s encephalopathy anti-amino terminal of alpha-enolase antibody autoimmune cerebellar ataxia immune-mediated cerebellar ataxia |