Familial Recurrent Hypersomnia: Two Siblings with Kleine-Levin Syndrome and Menstrual-Related Hypersomnia

Department of Neurology, Hospital del Mar, Barcelona, Spain.
Journal of child neurology (Impact Factor: 1.72). 11/2010; 25(11):1408-10. DOI: 10.1177/0883073810366599
Source: PubMed


Kleine-Levin syndrome and menstrual-related hypersomnia are rare idiopathic sleep disorders occurring primarily in adolescence. They are characterized by intermittent periods of excessive sleepiness, cognitive disturbances, and behavioral abnormalities. In both, the etiology remains unknown but autoinmune, hormonal, infectious, and inflammatory mechanisms have been proposed. The authors describe, for the first time, the association of Kleine-Levin syndrome and menstrual-related hypersomnia in 2 adolescent siblings who shared the human leukocyte antigen (HLA) loci DQB1*0501. The same haplotype has been associated with sleepwalking and with rapid eye movement (REM) sleep behavior disorder. This gender differences in the manifestation of a probably genetic influenced sleep disorder suggests that hormonal mechanisms could be implicated in the phenotypical expression of this sleep disorder. The male sibling with Kleine-Levin syndrome was easily controlled with carbamazepine in low doses, but his sister could be only efficaciously treated with oral contraceptives.

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    • "The cause of KLS is not established, but autoimmune and genetic factors are suspected [4] [5] [6]. Infections, fever, trauma, 1984-0063/& 2015 Brazilian Association of Sleep. "
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    ABSTRACT: Introduction: Recurrent hypersomnia (RH) is a rare disorder without established treatment. Methods: We report 2 RH medication-responsive cases with typical characteristics of Kleine-Levin syndrome (KLS). Case-reports: A 10 y.o. girl and a 14 y.o. boy presented with sudden sleepiness for 3-9 days (every 2-3 weeks). Physical examination, brain images and blood tests were normal. Polysomnographic findings were heterogenous, including disrupted sleep architecture. MSLTs revealed 2-3 SOREMPs and short sleep latency. Carbamazepine rendered girl׳s sleep normalization, while risperidone normalized boy׳s sleep cycles. Conclusions: Facing the absence of clinical trials in RH, reports of responsive cases are the available therapeutic evidence.
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    ABSTRACT: The first descriptions of patients with recurrent episodes of sleepiness and pathologic hunger date back to Kleine in Germany [1], Lewis [2] and Levin [3] in the USA. In 1936, Levin rewrote his original case report, and made for the first time specific mention of “a syndrome of periodic somnolence and morbid hunger” as a new entity in pathology [4]. Six years later, Critchley and Hoffman reported two more cases of periodic somnolence and morbid hunger and coined the term Kleine–Levin syndrome (KLS) [5]. In 1962, Critchley published an article referred to as “Periodic hypersomnia and megaphagia in adolescent males” in which he collected 15 genuine instances from the literature and 11 cases of his own, and proposed the definition of “a syndrome composed of recurring episodes of undue sleepiness lasting some days, associated with an inordinate intake of food, and often with abnormal behavior” [6]. From this time on, new case reports have been published. However, in 1960, Alfandary published four cases of hypnolepsie des adolescents which matched KLS except for the absence of compulsive eating [7], and in 1968, Bonkalo suggested the term “forme fruste” of KLS for a patient whose food intake was rated from “poor” to “good” but did not show compulsive eating [8]. In 2005 the second edition of the International Classification of Sleep Disorders introduced the term “recurrent hypersomnia” (including KLS and menstrual-related hypersomnia (MRH)) with the following diagnostic criteria [9]. The patient experiences recurrent episodes of excessive sleepiness of 2 days to 4 weeks’ duration. Episodes recur at least once a year. The patient has normal alertness, cognitive functioning and behavior between attacks. The hypersomnia is not better explained by another sleep disorder, medical or neurological disorder, mental disorder, medication use, or substance use disorder. Diagnostic criteria are not provided for KLS and MRH, but it is indicated that “a diagnostic of KLS should be reserved for cases in which recurrent episodes of hypersomnia are clearly associated with behavioral abnormalities. These may include binge eating; hypersexuality; abnormal behavior such as irritability, aggression, and odd behavior; and cognitive abnormalities such as feeling of unreality, confusion, and hallucinations.” This is a more comprehensive definition of KLS, moving away from Critchley’s definition, as binge eating is not compulsory for the diagnosis.
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