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: Based on 339 cases this review identifies, quantifies and compares 4 clinical forms of recurrent hypersomnia (1) Kleine-Levin syndrome (KLS) (239 cases), (2) Kleine-Levin syndrome without compulsive eating (KLS WOCE) (54 cases), (3) Menstrual related hypersomnia (MRH) (18 cases) and Recurrent hypersomnia with comorbidity (RHC) (28 cases). A second part of the review considers the main current issues on recurrent hypersomnia: the predisposing factors, including a window on family cases; the pathophysiology based on clinical patterns, neuroimaging data, neuropathological examinations and cerebrospinal fluid (CSF) hypocretin-1 measurements; the issues of recurrence and of a possible disruption of the circadian timing system; the relationships between recurrent hypersomnia and mood disorders; and a note on the atypical Kleine-Levin syndrome. The main outcomes of this study are a clear nosologic distinction of the different forms of recurrent hypersomnia, the finding that the prevalence of familial cases of KLS is in the same range as in narcolepsy, the suggestion of the possible involvement of a large set of cortical and subcortical structures in recurrent hypersomnia and some clues in favour of a relationship between recurrent hypersomnia and mood disorders.
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