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.

Síndrome de Kleine-Levin. Reporte de un caso / Kleine-Levin Syndrome. A Case Report

El síndrome de Kleine-Levin es un trastorno raro, caracterizado por episodios recurrentes de hipersomnia, alteraciones del apetito, disfunción cognitiva y conducta desinhibida; se estima que afecta entre una y cinco personas por millón de habitantes y es cuatro veces más frecuente en el sexo masculino. Se presenta el caso de un paciente masculino, de 18 años de edad, evaluado en la consulta de trastornos del sueño, en Villa Clara, por haber sufrido en cinco ocasiones de hipersomnia, de siete a diez días de duración, separados por tres o cuatro meses. Durante estos periodos se mostró irritable, apático y con anorexia. Fuera del cuadro clínico de hipersomnia el comportamiento es normal. El paciente cumple los criterios de la 3ra Clasificación Internacional de los Trastornos del sueño para el diagnóstico del síndrome de Kleine-Levin. Se decidió la presentación del caso por lo infrecuente de este padecimiento, cuyo diagnóstico se hace a los 15 meses de su comienzo y porque no existen referencias bibliográficas previas en Cuba.

Kleine-Levin syndrome is a rare disorder characterized by recurrent episodes of hypersomnia, appetite disturbance, cognitive dysfunction and uninhibited behavior. It is estimated to affect one to five per million people and is four times more common in males. We present the case of an 18-year-old patient treated at the sleep disorders consultation in Villa Clara due to five episodes of hypersomnia lasting seven to ten days, separated by three or four months. During these periods he was irritable, apathetic and anorexic. When not suffering from hypersomnia, his behavior is normal. The patient meets the criteria of the 3rd International Classification of Sleep Disorders for the diagnosis of the Kleine-Levin syndrome. This case is presented given the rarity of this condition; the diagnosis was made 15 months after its onset and there are no previous references in Cuba.

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...

História natural da síndrome de Kleine-Levin: revisão e discussão / Natural history of the Kleine-Levin syndrome: review and discussion

Neste artigo, os autores fazem um levantamento da história natural da síndrome de Kleine-Levin mediante a revisão e discussão críticas da literatura científica publicada até a data sobre o assunto. Prestam uma atenção especial aos correlatos psiquiátricos desta síndrome neuropsiquiátrica, focando os dilemas de diagnóstico deles decorrentes. Discutem as dificuldades e equacionam o perfil que os conhecimentos do estado da arte permitem delinear para a história natural da síndrome Kleine-Levin.

In this article, we provide a picture of the so-called natural history of the Kline-Levin syndrome by means of a critical review and discussion of the scientific literature published so far about this topic. We focused special attention on the psychiatric correlates of this neuropsychiatric syndrome, addressing the diagnostic dilemmas raised from them. We also discuss the difficulties and show the profile that the knowledge about the state of the art in this area allows us to outline for the natural history of this syndrome.

Síndrome de Kleine-Levin: caso clínico e dificuldades de diagnóstico / Kleine-Levin syndrome: clinical case and diagnosis difficulties

Os autores descrevem a síndrome de Kleine-Levin e referem as dificuldades de seu diagnóstico. Apresentam um caso clínico de um rapaz de 16 anos de idade, que tiveram a ocasião de diagnosticar e de acompanhar e que, embora manifestando a trilogia clássica (hipersônia, hiperfagia e hipersexualidade), é ilustrativo dessas dificuldades sob várias formas clínicas. Discutem as causas e as conseqüências colocadas pelas dificuldades de diagnóstico, prestando uma atenção especial aos aspectos de diagnóstico diferencial.

The authors describe the Kline-Levin Syndrome (KLS) and point out the difficulties regarding its diagnosis. A clinical case of a 16-year-old boy diagnosed and followed by the authors is presented. Although having the classic trilogy (hypersomnolence; hyperphagia; hypersexuality), this is an illustrative case of such difficulties under several distinct clinical forms. Causes and consequences raised by diagnostic difficulties are discussed, with special attention to aspects concerning differential diagnosis.