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克莱恩-莱文综合征的病因发病机制与治疗

Kleine-Levin syndrome ethiopathogenesis and treatment.

作者信息

Smolík P, Roth B

机构信息

Psychiatric Research Laboratory, Charles University Medical Faculty, Prague.

出版信息

Acta Univ Carol Med Monogr. 1988;128:5-94.

PMID:3078227
Abstract

The complex of the symptoms of psychic disorders and of the disorders of sleep, appetite, and food intake often forms the basis of the clinical picture of a mental disease. However, it is only rarely conceived in a complex manner as a set of physiologically interdependent functions. A remarkable proof of the interdependence of these functions is their complex disorder, the Kleine-Levin syndrome. The first descriptions of the symptoms of the Kleine-Levin syndrome can be found in the studies of several authors published as early as at the turn of the century. In 1942, the syndrome was designated by Critchley and Hoffmann after Willi Kleine and Max Levin, who defined it precisely in 1925 and 1929. The syndrome of periodic hypersomnia, megaphagia, and psychic disorders, originally described only in young males, was later found in females as well; the original very strict criteria were gradually broadened and complemented to some extent. At present, the most commonly accepted criterion for the diagnosis of the Kleine-Levin syndrome is the existence of the combined sleep disorder (hypersomnia or insomnia lasting from days to weeks), food intake disorders (megaphagia or anorexia), and various psychic abnormalities accompanying or following the attacks of the affection. We term the syndrome typical if the sleep disorder appears in the form of hypersomnia, food disorder in the form of megaphagia, and if psychic abnormalities are clearly expressed. On the other hand, we term the syndrome atypical if one of the main symptoms is opposite. The incomplete syndrome consists of only two main symptoms. The attacks of the affection set on mostly suddenly, lasting from several days to several weeks, ending suddenly again. The interparoxysmal periods last from several days to several months, sometimes even to several years. The etiopathogenesis of the affection is still unknown. A number of reports indicate a disorder of the diencephalon, perhaps only of the hypothalamus. The pathological-anatomical findings following the death of persons suffering from the disorders of sleep and food intake and from psychic abnormalities mostly reveal lesions in the region of the third brain ventricle. The development of the typical syndrome is benign, however, and morphological studies are not available. The typical Kleine-Levin syndrome can hardly escape the attention of clinicians owing to the richness and clarity of symptoms. The atypical or discretely expressed forms, however, often remain unrecognized even after a detailed medical examination and may lead to diagnostic uncertainty.(ABSTRACT TRUNCATED AT 400 WORDS)

摘要

精神障碍症状与睡眠、食欲及食物摄入紊乱的复合体,常常构成精神疾病临床症状的基础。然而,人们很少将其视为一组生理上相互依存的功能进行综合考量。这些功能相互依存的一个显著证据,就是它们的复杂紊乱——克莱因-莱文综合征。早在世纪之交,就有几位作者发表了关于克莱因-莱文综合征症状的研究,最早可追溯到这些研究中对该综合征症状的首次描述。1942年,克里奇利和霍夫曼以威利·克莱因和马克斯·莱文的名字为该综合征命名,他们在1925年和1929年对其进行了精确界定。这种周期性嗜睡、贪食及精神障碍综合征,最初仅在年轻男性中被描述,后来在女性中也有发现;最初非常严格的标准在一定程度上逐渐放宽并得到补充。目前,诊断克莱因-莱文综合征最普遍接受的标准是存在合并的睡眠障碍(持续数天至数周的嗜睡或失眠)、食物摄入紊乱(贪食或厌食)以及发作时或发作后伴随的各种精神异常。如果睡眠障碍以嗜睡形式出现,食物紊乱以贪食形式出现,且精神异常明显,我们就称该综合征为典型的。另一方面,如果主要症状之一相反,我们就称该综合征为非典型的。不完全综合征仅由两种主要症状组成。病情发作大多突然开始,持续数天至数周,然后又突然结束。发作间期持续数天至数月,有时甚至数年。该病症的病因仍不清楚。一些报告表明是间脑功能紊乱,可能仅涉及下丘脑。患有睡眠和食物摄入紊乱以及精神异常的患者死亡后的病理解剖结果大多显示第三脑室区域有病变。然而,典型综合征的发展是良性的,且没有形态学研究资料。典型的克莱因-莱文综合征因其症状丰富且明显,很难不引起临床医生的注意。然而,非典型或表现不明显的形式,即使经过详细的医学检查,也常常未被识别,可能导致诊断不确定。

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