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

ABSTRACT 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.

17 Reads
  • Source
    • "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. "
  • [Show abstract] [Hide abstract]
    ABSTRACT: The use of a bi-directional DC-DC converter in motor drives devoted to EVs allows a suitable control of both motoring and regenerative braking operations. In particular, during motoring operations of a battery-fed DC motor drive, a DC-DC converter is to be used to adjust the motor current in order to follow the torque reference signal. On the other hand, a bi-directional arrangement of the converter is needed for the reversal of the power flow, in order to recover the vehicle kinetic energy in the battery by means of motor drive regenerative braking operations. This paper deals with the study and comparison of two bi-directional buck-boost converter topologies. Each of them allows stepping the battery voltage level either up or down, according to motor drive modes of operation. For each converter topology computer simulations of modes of operation are presented together with experimental test results
    Applied Power Electronics Conference and Exposition, 1998. APEC '98. Conference Proceedings 1998., Thirteenth Annual; 03/1998
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Background Kleine-Levin syndrome is a rare sleep disorder of unknown etiology. It is characterized by intermittent periods of excessive sleepiness, cognitive disturbances and behavioral abnormalities. Nine cases of familial Kleine-Levin syndrome have been identified, but there are no reported cases describing twins that are affected by the syndrome. Case presentation We report the cases of 16-year-old monozygotic twin boys who both suffered from Kleine-Levin syndrome. In both cases, the onset of the first episode was preceded by an influenza infection. During symptomatic periods they slept for the entire day except for meals and bathroom visits. Actimetry recordings revealed that during symptomatic periods, daily activity was lower than that of asymptomatic periods, on the other hand, activity during the night was significantly higher in symptomatic periods than asymptomatic periods. Polysomnography (PSG) data during symptomatic periods revealed a decrease in sleep efficiency. Human leukocyte antigen (HLA) typing revealed no DQB1*02 loci. They were administered lithium carbonate but the beneficial effect was limited. Conclusions Our observations suggest that Kleine-Levin syndrome may be due to genetic and autoimmune processes, although etiologic relationship to specific HLA type remains controversial.
    BMC Neurology 05/2012; 12(1):31. DOI:10.1186/1471-2377-12-31 · 2.04 Impact Factor
Show more