Department of Neurology, Gui de Chauliac Hospital, Montpellier, France.
Institute for Neurosciences of Montpellier INM, Montpellier University, INSERM, Montpellier, France.
Sleep Med. 2024 Sep;121:135-143. doi: 10.1016/j.sleep.2024.06.003. Epub 2024 Jun 14.
OBJECTIVE/BACKGROUND: 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.
PATIENTS/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.
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.
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.
目的/背景：克莱恩-莱文综合征（KLS）是一种罕见的睡眠障碍，其特征为反复发作的严重嗜睡，并伴有不同程度的认知障碍、知觉异常、淡漠、行为障碍。这些症状中的一些，如嗜睡、强迫性进食和性欲增加，可能会被其对立面所取代，或者与它们交替出现。值得注意的是，这些“非典型症状”从未被阐明过，也从未与相应的典型症状的频率进行过比较。此外，KLS 在男性中比在女性中更为常见，但尚无研究比较过男性和女性中诱发因素和症状的频率。
患者/方法：为了揭示 KLS 尚未被研究的这些方面，我们使用预先设计的模板，从 364 例男性和 111 例女性的 475 例 KLS 病例报告中提取了诱发因素和症状。
诱发因素在男性（67.31%）中比女性（49.55%）更常见。反复发作的嗜睡在 94.32%的病例中存在，反复发作的失眠在 1.05%的病例中存在，嗜睡和失眠交替存在在 4.63%的病例中存在。认知障碍在 67.37%的病例中存在，在 6.95%的病例中不存在。现实感丧失/知觉改变在 38.32%的病例中存在，在 1.68%的病例中不存在。严重淡漠在 44.63%的病例中存在。强迫性进食在 59.58%的病例中存在，在 13.26%的病例中不存在，在 9.05%的病例中被厌食症取代，在 5.68%的病例中交替出现厌食症和强迫性进食，在 8.42%的病例中交替出现强迫性进食和无强迫性进食。性欲增加在 33.68%的病例中存在，在 22.74%的病例中不存在，在 1.47%的病例中被性欲减退取代，在 2.95%的病例中交替出现性欲增加和无性欲增加。异常行为在 45.05%的病例中存在。精神特征在 71.58%的病例中存在，在 2.95%的病例中不存在。最后，男性中睡眠障碍、淡漠、性障碍、易怒/攻击性的诱发因素和症状的百分比高于女性。
嗜睡、强迫性进食和性欲增加的对立面的频率似乎相当显著。此外，对男性和女性的诱发因素和症状进行系统比较显示，两性之间的差异有限。
