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抗二肽基肽酶样蛋白6(DPPX)自身免疫性脑炎伴严重多灶性肌张力障碍

Anti-dipeptidyl-peptidase-like protein-6 (DPPX) autoimmune encephalitis associated with severe multifocal dystonia.

作者信息

Roddy Eric, Gentry Erik, Holiday Victoria, Robertson David, Hedera Peter

机构信息

University of Louisville School of Medicine, Department of Neurology, USA.

Norton Neuroscience Institute, Department of Neurology, University of Louisville, 220, Abraham Flexner Way, Suite 606, USA.

出版信息

Clin Park Relat Disord. 2025 Apr 2;12:100320. doi: 10.1016/j.prdoa.2025.100320. eCollection 2025.

Abstract

Anti-DPPX encephalitis is a rare form of autoimmune encephalitis characterized antibodies against a subunit of Kv4.2 potassium channels. Characteristic clinical features include cognitive dysfunction, parasomnias, psychosis, and seizures. Motor symptoms typically include myoclonus, tremor, and midline ataxia. DPPX encephalitis presenting as new-onset focal dystonia has not been previously described.

摘要

抗二肽基肽酶样蛋白 6(DPPX)脑炎是一种罕见的自身免疫性脑炎,其特征是存在针对 Kv4.2 钾通道亚基的抗体。典型的临床特征包括认知功能障碍、异态睡眠、精神病和癫痫发作。运动症状通常包括肌阵挛、震颤和中线共济失调。此前尚未有以新发局灶性肌张力障碍为表现的 DPPX 脑炎的相关报道。

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