Richard Y, Le Galudec M, Saint-André S, Planche P, Genestet S, Lazartigues A
Jeune équipe Ethique, Professionnalisation, Santé (JE 2535), université de Bretagne occidentale, UFR médecine et sciences de la santé, Service hospitalo-universitaire de psychiatrie de l'enfant et de l'adolescent, hôpital de Bohars, CHU de Brest, France.
Encephale. 2010 Feb;36(1):28-32. doi: 10.1016/j.encep.2008.11.003. Epub 2009 Apr 2.
The purpose of this article is to report an original clinical case whose symptoms suggest a very peculiar pathology, because of its rarity, symptomatic expression and unclear etiopathogenesis: the Kleine-Levin Syndrome (KLS). During the regression of tonsillitis concomitant with an emotional shock, the 15-year-old patient exhibited a dramatic change in behaviour, at odds with his previous state, and accompanied by hypersomnia and confusion, megaphagia, irritability, hypersexuality and mood disorders. We observed a spontaneous and total regression of the symptoms after 12 days, except for the incomplete amnesia that proved to be persistent. Four months later, further to an ethylic drunkenness, the patient presented with a new and similar episode. The patient benefited from no medicinal treatment, even in the course of hypersomnia episodes and asymptomatic periods. After a clinical presentation of this patient, we will consider this case study from a more psychopathological angle by questioning the existence of a facilitating psychological profile. The discovery of an IQ equal to 86 from the scores of WISC-IV, and the identification of constructive visual difficulties made us suspect neurological disorders, but these abnormalities were not found during the completion of the Rey Complex Figure Test. The personality profile issued from the scores at the MMPI-A assessment was ranked as barely significant (type 2-4): indeed, it showed nothing specific to this patient. Literature data show that most of the patients presenting with a KLS have been seen by a psychiatrist at the time of the disease and diagnosed as suffering from hysteria, or schizophrenia, or bipolar disorders... Because of diagnostic wanderings, some patients have, hence, received inappropriate treatments. One should pay close attention to this very rare syndrome, on the border between neurology and psychiatry, since its diagnosis is essentially based on clinical features, and carefully think about the implementation of a medicinal treatment. This unique case seems unable to support our working hypothesis about the identification of a particular psychological profile in the KLS, but the question of an underlying fragility is still worth considering. We personally think that, even though links between the KLS and bipolar disorders have been suggested, this disease has to be considered as a separate entity.
本文旨在报告一例原始临床病例，因其罕见性、症状表现及病因发病机制不明，其症状提示一种非常特殊的病理学情况：克莱恩-莱文综合征（KLS）。在扁桃体炎消退过程中，伴随一次情绪冲击，这位15岁的患者行为发生了戏剧性变化，与他之前的状态不符，同时伴有嗜睡、意识模糊、贪食、易怒、性欲亢进和情绪障碍。我们观察到12天后症状自发且完全消退，除了不完全遗忘症持续存在。四个月后，在一次酒精中毒后，患者出现了新的类似发作。即使在嗜睡发作期和无症状期，该患者也未接受药物治疗。在介绍该患者的临床表现后，我们将从更精神病理学的角度考虑这个案例研究，质疑是否存在一种促发心理特征。从韦氏儿童智力量表第四版（WISC-IV）得分发现智商等于86，以及识别出建设性视觉困难，使我们怀疑存在神经障碍，但在完成雷氏复杂图形测验时未发现这些异常。明尼苏达多相人格调查表青少年版（MMPI-A）评估得分得出的人格特征被评为几乎无显著意义（2-4型）：实际上，它没有显示出该患者的任何特异性。文献数据表明，大多数患有KLS 的患者在患病时被精神科医生诊治，并被诊断为患有癔症、精神分裂症或双相情感障碍……由于诊断的徘徊不定，一些患者因此接受了不恰当的治疗。鉴于其诊断主要基于临床特征，人们应该密切关注这种处于神经学和精神病学边界的非常罕见的综合征，并仔细考虑药物治疗的实施。这个独特的案例似乎无法支持我们关于在KLS中识别特定心理特征的工作假设，但潜在脆弱性的问题仍然值得考虑。我们个人认为，尽管有人提出KLS与双相情感障碍之间存在联系，但这种疾病必须被视为一个独立的实体。
