目的:Kleine-Levin综合征(KLS)是一种罕见的睡眠障碍,其特征是反复发作的严重嗜睡,并伴有不同程度的认知障碍。感知异常,冷漠,行为障碍。其中一些症状,嗜睡,强迫性进食和增加的性欲可能被它们的对立面所取代或与它们交替。值得注意的是,这些“非典型症状”从未被发现,也没有与相应的典型症状进行比较。此外,KLS在男性中比在女性中更常见,但从未进行过比较男性和女性的诱发因素和症状的频率。
方法:为了揭示KLS尚未研究的这些方面,使用预先设计的模板来提取诱发因素和症状,在475例KLS病例报告中,包括364名男性和111名女性。
结果:男性(67.31%)比女性(49.55%)更频繁地记录了沉淀因素。94.32%的病例中出现了高发的反复发作,复发性失眠占1.05%,嗜睡和失眠交替占4.63%。67.37%的病例存在认知障碍,6.95%的病例不存在认知障碍。38.32%的病例存在二重化/感知改变,1.68%的病例不存在。44.63%的病例存在严重的冷漠。59.58%的病例存在强迫性进食,13.26%不存在,9.05%的人被厌食症取代,强迫进食和厌食交替占5.68%,强迫进食和无强迫进食交替占8.42%。33.68%的病例存在性欲增加,在22.74%中缺席,取而代之的是1.47%的性欲下降,2.95%的性欲增加和性欲没有增加的交替。45.05%的病例存在奇怪行为。71.58%的病例有精神病特征,2.95%不存在。最后,诱发因素和睡眠障碍的百分比,冷漠,性障碍,烦躁/易怒,男性高于女性。
结论:嗜睡的对立面频率,强迫性进食和增加性欲似乎相当重要。此外,对男性和女性的诱发因素和症状的系统比较表明,两性之间的差异有限。
OBJECTIVE: Kleine-Levin syndrome (KLS) is a rare sleep disorder characterized by recurrent episodes of severe hypersomnolence in association with various degrees of cognitive impairment, perceptive abnormalities, apathy, behavioral disturbances. Some of these symptoms, hypersomnolence, compulsive eating and increased sexual drive may be replaced by their opposites or alternate with them. Remarkably enough, these « atypical symptoms » have never been enlighted nor compared in frequency with corresponding typical symptoms. Besides, KLS is more frequent in males than in females but no review has ever compared the frequency of precipitating factors and symptoms in males and females.
METHODS: To uncover these as yet uninvestigated aspects of KLS, a predesigned template was used to extract precipitating factors and symptoms, in 475 case reports of KLS, comprising 364 males and 111 females.
RESULTS: Precipitating factors were more frequently recorded in males (67.31 %) than in females (49.55 %). Recurrent episodes of hypersomnolencee were present in 94.32 % of cases, recurrent insomnia in 1.05 % and alternation of hypersomnolence and insomnia in 4.63 %. Cognitive impairment was present in 67.37 % of cases and absent in 6.95 %. Derealization/altered perception was present in 38.32 % of cases and absent in 1.68 %. Severe apathy was present in 44.63 % of cases. Compulsive eating was present in 59.58 % of cases, absent in 13.26 %, replaced by anorexia in 9.05 %, alternation of compulsive eating and anorexia in 5.68 % and alternation of compulsive eating and no compulsive eating in 8.42 %. Increased sexual drive was present in 33.68 % of cases, absent in 22.74 %, replaced by decreased sexual drive in 1.47 %, alternation of increased sexual drive and no increased sexual drive in 2.95 %. Odd behaviors were present in 45.05 % of cases. Psychiatric features were present in 71.58 % of cases, absent in 2.95 %. Finally, the percentages of precipitating factors and of sleep disorder, apathy, sexual disorder, irritability/agressivity, were higher in males than in females.
CONCLUSIONS: The frequency of the opposites of hypersomnolence, compulsive eating and increased sexual drive appears to be quite significant. In addition, a systematic comparison of precipitating factors and symptoms in males and females has shown limited differences between sexes.