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1型发作性睡病迟发型罕见病例。

Rare Case of Late-Onset Narcolepsy Type 1.

作者信息

Kovalská Petra, Dostálová Simona, Machová Hana, Nytrová Petra, Maurovich Horvat Eszter, Šonka Karel

机构信息

Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital in Prague, Prague, Czechia.

Inspamed, Ltd., Institute of Sleep Medicine, Prague, Czechia.

出版信息

Case Rep Neurol. 2020 Nov 12;12(3):428-432. doi: 10.1159/000510633. eCollection 2020 Sep-Dec.

Abstract

A 69-year-old male developed symptoms typical of the diagnosis of narcolepsy type 1 without any previous triggering events. First, daytime sleepiness occurred, soon followed by cataplexy. Nocturnal polysomnography revealed rapid eye movement (REM) sleep behavior disorder, a apnea-hypopnea index of 25.8 events/h, and no sleep-onset REM. Multiple Sleep Latency Test showed a mean sleep latency of 2.1 min and REM sleep in 3 tests. HLA DQB1*06:02 was positive and hypocretin-1 in cerebrospinal fluid unmeasurable. A treatment with 50 mg clomipramine controlled the cataplexy; excessive daytime sleepiness was sufficiently managed by repeated naps. The administration of 0.25 mg of clonazepam subjectively improved REM sleep behavior disorder. Bilevel Positive Airway Pressure improved the apnea-hypopnea index without important influence on sleepiness. Our unique case demonstrates that even elderly subjects can develop narcolepsy type 1.

摘要

一名69岁男性在没有任何先前诱发事件的情况下出现了1型发作性睡病的典型症状。首先出现日间嗜睡,随后很快出现猝倒。夜间多导睡眠图显示快速眼动(REM)睡眠行为障碍、呼吸暂停低通气指数为25.8次/小时,且无睡眠起始REM。多次睡眠潜伏期试验显示平均睡眠潜伏期为2.1分钟,在3次试验中出现REM睡眠。人类白细胞抗原DQB1*06:02呈阳性,脑脊液中下丘脑分泌素-1无法测出。50毫克氯米帕明治疗控制了猝倒;通过反复小睡充分管理了日间过度嗜睡。服用0.25毫克氯硝西泮主观上改善了REM睡眠行为障碍。双水平气道正压通气改善了呼吸暂停低通气指数,对嗜睡没有重要影响。我们这个独特的病例表明,即使是老年患者也可能患1型发作性睡病。

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