Hipersomnias / Hypersomnia

Las hipersomnias son un grupo de trastornos caracterizados por una somnolencia excesiva durante al menos 1 mes, evidenciada tanto por episodios prolongados de sueño como por episodios de sueño diurno que se producen prácticamente cada día. Se dividen en primarios o centrales, (Hipersomnia idiopática, Narcolepsia y Síndrome de Klein-Levin) y secundarios (Privación del sueño crónica en niños). La somnolencia excesiva debe ser de su ciente gravedad como para provocar alteraciones clínicas significativas o deterioro social, escolar, laboral o de otras áreas importantes de la actividad del individuo; no aparece en el transcurso de otro trastorno del sueño o de otro trastorno mental ni se debe a los efectos fisiológicos directos de una sustancia o de una enfermedad médica. La somnolencia excesiva diurna (SDE) es una manifestación común, se presenta con una frecuencia variable; del 11% en niños hasta el 52,8% en adolescentes. La predominancia es igual en la narcolepsia con o sin cataplejía y en el Síndrome de Kleine-Levin. Su diagnóstico adecuado se basa en la historia clínica y estudios de polisomnografía. Y el tratamiento, ayudará al paciente a mejorar en sus actividades y a elevar su autoestima. La fisiopatología no es clara y su tratamiento va enfocado a disminuir el sueño diurno con fármacos como el Modafinil, Claritromicina o simpaticomiméticos y terapias de apoyo.

Abstract Hypersomnias are a group of disorders characterized by excessive drowsiness for at least 1 month, evidenced by both prolonged episodes of sleep and episodes of daytime sleep that occur almost every day. They are divided into primary or central, (idiopathic hypersomnia, Narcolepsy and Klein-Levin Syndrome) and secondary (Deprivation of chronic sleep in children). Excessive drowsiness should be of sufficient severity to cause significant cant clinical alterations or social, school, work or other important areas of the individual's activity; which does not appear in the course of another sleep disorder or other mental disorder, nor is it due to the direct physiological effects of substances or medical illness. Excessive daytime sleepiness (EDS) is a common manifestation; it occurs with a variable frequency; From 11% in children to 52.8% in adolescents. The predominance is the same in Narcolepsy with or without cataplexy and in Kleine-Levin Syndrome. The adequate diagnosis is based on clinical history and studies of polysomnography. The treatment will help the patient to improve their activities and raise their self-esteem. The pathophysiology is not clear and the treatment is focused on decreasing daytime sleep with drugs such as Modafinil, Clarithromycin or sympathomimetics and supportive therapies.

A Polysomnography Study of Kleine-Levin Syndrome in a Single Center / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Kleine-Levin syndrome (KLS) is a rare sleep disorder characterized by recurrent episodes of hypersomnia. Polysomnographic (PSG) researches of KLS have been reported only in few publications in the past decades. This study aimed to investigate the characteristics of PSG of KLS.</p><p><b>METHODS</b>This study, which was conducted from March 2010 to July 2014, included seven patients diagnosed with KLS in the Sleep and Wake Disorder Center of Huashan Hospital, Fudan University (Shanghai, China). PSG and multiple sleep latency tests (MSLT) were performed during their episodes and the results were evaluated.</p><p><b>RESULTS</b>Five of the seven patients were males. The mean age at KLS onset was 15.6 ± 3.6 years. The number of episodes ranged from 2 to 7. The duration of episodes lasted from 4 to 11 days. The sleep architecture and proportion were normal in most of the patients. The average value of mean sleep latency was 6.9 ± 4.1 min. No sleep-onset rapid eye movement (SOREM) was detected in three of the patients, whereas one patient experienced one period of SOREM, and such episodes occurred twice in other two patients.</p><p><b>CONCLUSIONS</b>We found that sleep architecture and proportion were normal in most KLS patients. However, the results of PSG and MSLT had no specificity for KLS patients.</p>

Síndrome de Kleine-Levin / Kleine-Levin Syndrome

El síndrome de Kleine-Levin es un trastorno raro manifestado por alteración del sueño y cambios psiquiátricos que pueden precipitarse por diversos factores, entre los cuales se han descrito infecciones y trauma. Los síntomas más comunes son hipersomnia, alteraciones cognitivas (incluyendo un sentimiento específico de desrealización), trastornos alimentarios, hipersexualidad, compulsiones y depresión. Presentamos el caso de un paciente de 16 años, cuya evolución y presentación cíclica fue la clave para reconocerlo. Hacemos una revisión de los diagnósticos diferenciales, estudios, diagnóstico y manejo...

Kleine-Levin syndrome is a rare disorder characterized by sleep pattern alterations and psychiatric symptoms which may be precipitated by various factors, among which are infections and trauma. The most common symptoms are: hypersomnia, cognitive alterations (including a specific derealization sensation), eating disorders, hypersexuality, compulsions and depression. We present the case of a 16-year old male patient, in whom disease progression and cyclical episodes were the clue to diagnosing this syndrome. We also review differential diagnosis, existing trials, and disease diagnosis and management...

Síndrome de Kleine-Levin: caso clínico y revisión del tema / Kleine-Levin Syndrome: clinical case and revision

Episodic hypersomnia, compulsive excessive eating and erotic behaviour, with schizophreniclike mental symptoms are the hallmarks of the rare Kleine–Levin syndrome. Many patients may not necessarily fulfill minimum criteria described for diagnosis. We report a 19 years young man with incomplete presentation the Kleine–Levin syndrome and briefly reviewed the most relevantaspects of this disorder, its epidemiology, clinical symptoms and complementary diagnostic examinations. Known therapeutic options and prognosis are also discussed.

[Kleine-Levin syndrome]

Kleine-levin syndrome is a rare disorder with symptoms that include periodic, sudden-onset episodes of hypersomnia, cognitive and behavioral disturbances [96%], changes and eating disturbances [80%], hypersexuality [43%]. This syndrome happened mostly in men [68%] and occurred sporadically worldwide. The median age of onset is adolescent. It was precipitated most frequently by infections [38.2%]. The prognosis is generally benign, with almost complete remission with normal cognitive and social functions after the episodes. The aim of this case report is to present a typical case of Kleine-Levin, and to illustrate problems in differential diagnosis, history, course, and therapeutic, neuropsychological sequela after the episode of the illness. Further research in the natural history of Klein-Levin syndrome is needed. Lithium and carbamazepine or other antiepileptics had a higher reported response rate [41%] for stopping relapses. It is important that the diagnosis is suspected early, especially in adolescent males who present with recurrent episodes of somnolence, increased appetite, and abnormal behavior

Kleine-Levin syndrome: interface between neurology and psychiatry

We report the first episode of Kleine-Levin (KLS) syndrome in a 17-year-old male. The illness onset, clinical features, neuropsychological evaluation and polysomnographic recording are described. Typical symptoms hypersomnia, hyperphagia and sexual disinhibition were observed besides behavioral disturbances, polysonographic and neuropsychological alterations. Behavioral disturbances similar to a manic episode including psychotic symptoms were relevant. The pharmacologic treatment included lithium, methylphenidate and risperidone. The introduction of risperidone aimed the control of psychotic symptoms and the persistent manifestations of hypersexuality after sleepness control and to the best of our knowledge there are no other report regarding risperidone use for KLS in the literature.

Relatamos o primeiro episódio de síndrome de Kleine-Levin (SKL) num adolescente de 17 anos. São descritos o modo de aparecimento da doença, achados clínicos, avaliação neuropsicológica e polissonografia. Foram observados sintomas típicos como hipersonolência, hiperfagia e desinibição sexual, e outras alterações comportamentais, polissonográficas e neuropsicológicas. As alterações comportamentais assemelhadas a um episódio de mania, incluindo sintomas psicóticos, foram relevantes. O tratamento farmacológico incluiu lítio, metilfenidato e risperidona. A introdução de risperidona teve por objetivo o controle dos sintomas psicóticos e das persistentes manifestações da hipersexualidade, que permaneceram após diminuição da sonolência; não temos conhecimento de relatos anteriores, na literatura, de seu uso no tratamento da SKL.

Kleine-Levin syndrome and encephalitis.

Kleine-Levin Syndrome is characterized by hypersomnolence, hyperphagia and sexual disinhibition. The article reported a case of 10-year-old boy with a two-week history of altered sensorium, irrelevant talks, markedly increasing appetite and tendency to sleep most of the times. Immediately preceding to it the child had been an episode of enteric fever confirmed by the serological tests.

A Female Case of Kleine-Levin Syndrome Treated with Amantadine / 신경정신의학

Kleine-Levin syndrome is a rare disorder which usually affects adolescent males and is characterized by periodic hypersomnia, hyperphagia and abnormal behavior. This is an unexplained clinical syndrome for which several etiologies have been entertained with no standard treatment is yet available. A 18-year old woman began suffering from recurrent hypersomnia, hyperphagia, and behavioral disturbances such as irritability, derealization, and amnesia. She was normal between the episodes and diagnosed as Kleine-Levin syndrome. In the course of about two years she had 11 episodes and the mean interval between the episodes was 52.8+/-16.7 days. After application of amantadine, there were two mild episodes and then she had no episodes for more than 6 months. This case suggests the possible role of amantadine in the treatment of Kleine-Levin syndrome.

Neuropsychological sequelae in Kleine-Levin syndrome: case report

Kleine-Levin syndrome is characterized by periodic hypersomnia, hyperphagia, sexual disinhibitions and behavioral disturbances. The prognosis is generally benign, with normal cognitive and social functions after the episodes. We describe a typical case of Kleine-Levin syndrome associated with apparent academic decline, neuropsychological sequelae and personality alterations after the second episode of the illness. Further research in the natural history of Kleine-Levin syndrome is needed, for example, to determine whether early intervention would improve long-term prognosis.

Kleine-Levin Syndrome: Two Cases

Kleine-Levin syndrome (KLS) is characterized by recurring episodes of hypersomnia, megaphagia, and abnormal behavior. We report two cases of KLS. Two boys, aged 18 (case 1) and 17 (case 2), had recurrent episodes of hyper-somnolence with compulsive eating or drinking and hypersexuality for several years. HLA-DR typing was HLA-DR3 and 13 in case 1 and HLA-DR4 and 10 in case 2. Case 1 showed hypersomnia with early onset of REM sleep on MSLT and frequent frontal intermittent rhythmic delta activity on EEG. Both cases showed no abnormalities on brain MRI. HLA-DR typing facilitates differentiation between KLS and narcolepsy by the absence of HLA-DR2.

A Case of Kleine-Levin Syndrome

Kleine-Levin syndrome (KLS) is a rare disorder and is diagnosed by recurrent episodes of hypersomnia, hyperphagia, and neurobehavioral dysfunctions. We present a case of a male 19 year-old with seven episodes of the above symptoms. All episodes occurred after a respiratory infectious illness and spontaneously resolved after a few days or a few weeks. A polysomnography showed prolonged sleep latency and decreased REM sleep (14.6%). A multiple sleep latency test revealed a slightly short mean sleep latency (8min 7sec) and three sleep-onset REM episodes in a series of four sleep latency tests across a one day period. The electrophysiological features of previously reported were reviewed.

Síndrome de Kleine-Levin: Revisäo Bibliográfica / Kleine-Levin Syndrome: Bibliographic review

A Síndrome de Kleine-Levin é caracterizada pela tríade: hipersônica, megafagia e hipersexualidade. Jovens do sexo masculino säo mais comumente afetados. A patogênese da doença é desconhecida. Seu diagnóstico é baseado em dados clínicos. Näo há dados específicos aos exames laboratoriais. O tratamento é baseado em estimulantes centrais, antidepressivos, lítio e antagonistas da serotonina

Kleine-Levin syndrome: clinical course, polysomnography and multiple sleep latency test: case report

A case of Kleine-Levin syndrome, with chronic severe periodic hypersomnia is described in a 17-year-old female. The first episode started when she was 15 years old. The episodes were characterized by periodic hypersomnia accompanied by hyperphagia, lasting 5 days, and repeating at 28 to 60 day intervals. The severity of hypersomnia prevented her from attending school activities. Outside the hypersomnia periods, she was asymptomatic. EEG, brain computerized tomography and brain nuclear magnetic resonance were normal; all-night polysomnography, Multiple Sleep Latency Test (MSLT) and Epworth Sleepiness Scale (ESS) were within normal limits. During the period of hypersomnolence, polysomnography showed short sleep latency and short REM latency. MSLT mean sleep latency was 1.8 min; and REM period was present in one subtest; the ESS was markedly elevated.

Sleep disorders in school-age children.

This article on school age children reviews relevant issues in sleep physiology, the classification of sleep disorders, their clinical and laboratory assessment, some common sleep disorders, the sleep-epilepsy relationship, as well as the impact of daytime sleepiness on higher cortical functions.

Síndrome de Kleine-Levin associada a presença de anticorpos antifosfolipídios / Kleine-Levin synsdrome associated to antiphospholipid antibodies

Descriçäo de um caso de síndrome de Kleine-Levin, manifestado por hipersonia predominante, em um adolescente de 13 anos, acompanhado ambulatorialmente há vários anos por apresentar fenômeno de Raynaud, presença de anticorpos antifosfolipídios e FAN positivo. O caso ilustra a discussäo de uma doença bastante rara, com ênfase nos aspectos importantes de epidemiologia, diagnóstico e terapêutica

Resultados da escala de sonolencia Epworth em 480 alunos de graduacao da Faculdade de Medicina da Universidade de Sao Paulo / Epworth sleepiness scale outcome in 480 Brazilian medical students

Este estudo relata os resultados da aplicacao da Escala de Sonolencia de Epworth (ESE) em 480 alunos da FMUSP, o resultado da correlacao do valor obtido da ESE com o numero declarado de horas de sono por noite e a comparacao com um estudo semelhante com alunos de medicina da Australia. A ESE e um questionario auto-aplicado de 8 itens usado para se avaliar sonolencia subjetiva em adultos. Os valores da ESE variam de 0 ate o maximo de 24 pontos. Os questionarios foram aplicados no final de 1994. Total de respostas = 480 sendo que 293 masculino, 185 feminino. Idade media = 20,55 + ou - 2,15 (DP). Etnia branca = 3346; amarela = 96; negra = 3; outros = 10; nao declarados = 25...

Kleine-levin syndrome case report

A case of Kleine- Levin syndrome is reported in a 28-year-old man, which started at the age of 19 years, with typical features of the syndrome, i.e., excessive sleepiness for 1-2 weeks, hyperphagia and irritability which recurred about twice annually. The patient was treated with Lithium which apparently had beneficial effects on the course of the illness