Objective

Cataplexy is a pathognomonic symptom of narcolepsy type 1. This study was conducted to clarify the clinical characteristics of cataplexy by staging, and to further analyse the correlations of clinical features and cataplectic stages in patients with narcolepsy type 1 (NT1).

Methods

We experimentally triggered patients with NT1 into cataplexy while under video-polysomnography (v-PSG) monitoring in the sleep lab. The most serious cataplectic attack from each patient was analysed. Each cataplectic episode was segmented into four stages according to the v-PSG. Correlations were analysed between cataplectic stages in pairs, and between cataplectic stages and other clinical features.

Results

We observed 81 cataplectic episodes in 21 patients with diverse triggers, including humorous or exciting videos, tickling, recalling horrible memories and exercising. Nine patients (43%) went through complete cataplectic attacks while the others experienced partial attacks. Four cataplectic stages (ie, triggering, resisting, atonic, and recovering) were identified according to clinical and electromyograms characteristics. Resisting stage is predominant (56.4%) in cataplexy, while atonic stage is most related with the total duration of cataplexy. The Epworth Sleepiness Scale score (ESS) has a positive correlation with the total duration of cataplexy. Both duration of cataplexy and ESS score are negatively correlated with disease course. However, medication history seems have no influence on either cataplexy duration or ESS score.

Conclusion

Four-stage segmentation shows the dynamic process of the cataplectic attack, which is different from the traditional classification of complete or partial cataplexy. Resisting stage is necessary for every cataplexy and might reflect the compensation mechanism, while atonic stage may be omitted in some patients. The severity of narcolepsy reduces with the extension of natural course regardless of medication history.

中文翻译：

---

发作性睡病1型的重症发作的临床特征

客观的

猝倒性发作是发作性睡病1型的病理症状。本研究通过分期阐明猝倒性发作的临床特征，并进一步分析了发作性睡病1型（NT1）患者的临床特征与猝发性分期的相关性。

方法

我们在睡眠实验室接受视频多导睡眠图（v-PSG）监控的同时，通过实验触发了NT1患者进入白内障。分析了每个患者最严重的折返性发作。根据v-PSG，每个重发发作可分为四个阶段。分析成对的折返期之间，折返期与其他临床特征之间的相关性。

结果

我们在21位患者中观察到81次折返性发作，这些发作有不同的触发因素，包括幽默或激动人心的视频，发痒，令人难忘的回忆和锻炼。9名患者（43％）经历了完全的折返性发作，而其他患者则经历了部分发作。根据临床和肌电图特征，确定了四个折返阶段（即触发，抵抗，失调和恢复）。瘫痪中抵抗阶段占主导地位（56.4％），而无张力阶段则与瘫痪的总持续时间最相关。Epworth嗜睡量表评分（ESS）与瘫痪的总持续时间呈正相关。瘫痪持续时间和ESS评分均与病程呈负相关。但是，用药史似乎对瘫痪持续时间或ESS评分均无影响。

结论

四阶段分割显示了折返式攻击的动态过程，这与传统的完全或部分瘫痪分类不同。抵抗阶段对于每个脑瘫都必不可少，并且可能反映了补偿机制，而在某些患者中可以忽略无力阶段。发作发作的严重程度随自然病程的延长而降低，而与服药史无关。