Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM)-like Symptoms Associated with Anti-ganglionic Acetylcholine Receptor Antibodies
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- Kitazaki Yuki
- Department of Neurology, Fukui-ken Saiseikai Hospital, Japan Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
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- Ikawa Masamichi
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan Department of Advanced Medicine for Community Healthcare, Faculty of Medical Sciences, University of Fukui, Japan
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- Kishitani Toru
- Department of Neurology, Fukui-ken Saiseikai Hospital, Japan Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
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- Kamisawa Tomoko
- Department of Neurology, Fukui-ken Saiseikai Hospital, Japan Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
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- Nakane Shunya
- Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Japan
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- Nakamoto Yasunari
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
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- Hamano Tadanori
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan Department of Aging and Dementia, Faculty of Medical Sciences, University of Fukui, Japan
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抄録
<p>This report describes a 59-year-old woman who presented with progressive encephalomyelitis with rigidity and myoclonus (PERM)-like symptoms and severe dysautonomia, including orthostatic hypotension, sinus bradycardia, dysuria, and prolonged constipation. Her neurological symptoms improved after immunotherapy, but the dysautonomia persisted. Anti-ganglionic acetylcholine receptor (gAChR) α3 subunit antibodies, which are frequently identified in patients with autoimmune autonomic ganglionopathy, were detected in the pre-treatment serum. The central distribution of the nicotinic acetylcholine receptors, a target of anti-gAChR antibodies, and immunotherapeutic efficacy observed in this case indicate that anti-gAChR α3 subunit antibodies are associated with the PERM-like features accompanied by autonomic manifestations. </p>
収録刊行物
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- Internal Medicine
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Internal Medicine 60 (14), 2307-2313, 2021-07-15
一般社団法人 日本内科学会
