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儿童发作性睡病中的猝倒特征。

Cataplexy features in childhood narcolepsy.

作者信息

Serra Leonardo, Montagna Pasquale, Mignot Emmanuel, Lugaresi Elio, Plazzi Giuseppe

机构信息

Sleep Disorders Center, Department of Neurological Sciences, University of Bologna, Italy.

出版信息

Mov Disord. 2008 Apr 30;23(6):858-65. doi: 10.1002/mds.21965.

DOI:10.1002/mds.21965
PMID:18307264
Abstract

Cataplexy, the hallmark of narcolepsy, has been well characterized in adults but not in children. This study systematically used structured clinical assessments and video-recordings (49 episodes in eight cases) to evaluate cataplexy in 23 patients diagnosed before the age of 18 years. Forty-three percent of patients had falls as part of their attacks. During cataplexy knees, head, and jaw were the most frequently compromised body segments; eyelids, arms, and trunk being less commonly involved. More rarely, blurred vision, slurred speech, irregular breathing, or a sudden loss of smiling mimics were reported. One-third of the sample presented with a previously unrecognized description of cataplexy that we coined "cataplectic facies," consisting of a state of semipermanent eyelid and jaw weakness, on which partial or complete cataplectic attacks were superimposed. The usual triggering emotions, such as laughter, joking, or anger, were not always present, especially when close to an abrupt onset, hampering diagnosis. Video-recordings of cataplectic attacks may be useful to document the attack, allowing a comparison with archived presentations.

摘要

猝倒症是发作性睡病的标志性症状,在成年人中已有充分的特征描述,但在儿童中却并非如此。本研究系统地采用结构化临床评估和视频记录(8例患者共49次发作),对23例18岁前确诊的患者的猝倒症进行评估。43%的患者发作时伴有跌倒。在猝倒发作期间,膝盖、头部和下巴是最常受累的身体部位;眼睑、手臂和躯干较少受累。更罕见的是,有视力模糊、言语不清、呼吸不规则或突然失去微笑表情的报告。三分之一的样本表现出一种以前未被认识到的猝倒症表现,我们将其称为“猝倒面容”,表现为半永久性的眼睑和下巴无力状态,并叠加部分或完全的猝倒发作。通常引发发作的情绪,如大笑、开玩笑或愤怒,并不总是出现,尤其是在发作接近突然起病时,这会妨碍诊断。猝倒发作的视频记录可能有助于记录发作情况,以便与存档的表现进行比较。

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