RESUMEN
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.
RESUMEN
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.
RESUMEN
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.
RESUMEN
A case of Kleine Levin Syndrome is presented. Episodic course with spontaneous remission of each episode and characteristic features of hypersomnia, hyperphagia, disinhibited behavior. affective features like irritability and cognitive disturbance made the diagnosis of Kleine Levin syndrome in our patient.
RESUMEN
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.
Asunto(s)
Adolescente , Femenino , Humanos , Masculino , Amantadina , Amnesia , Despersonalización , Trastornos de Somnolencia Excesiva , Hiperfagia , Síndrome de Kleine-LevinRESUMEN
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.