Introduction: The Kleine-Levin syndrome is a rare disease of unknown origin characterized by recurrent and self-limited episodes of hypersomnia that are also accompanied by a cognitive and behavioral dysfunction. Patients present normal sleeping and behavior patterns between episodes.
Case reports: We present three patients who are 14 years old: two boys and one girl. They started having the episodes after a predisposing factor (vaccine, influenza B and menstruation). During the episode they presented hypersomnolence and while wakefulness they were bradipsychic, in motor restlessness and with emotional liability. They also presented a tendency towards crying and claimed the presence of relatives constantly. The episodes lasted between 10 and 15 days and they appeared monthly, being asymptomatic between episodes. All three patients were attended initially by pediatricians, diagnosed and treated as autoimmune encephalitis. Only one of our cases had the three typical symptoms of hypersomnia, hyperfagia and hypersexuality. However, none of the three had been asked initially and the family only referred to it after the directed anamnesis.
Conclusions: The Kleine-Levin syndrome presents neurologic symptoms initially more frequently than psychiatric ones. Hypersomnia and behavioural disturbances during wakefulness, bradypsychia, apatheia and emotional liability make us suspect that it could be an encephalitis process. We should be aware of this entity if we face a patient with recurrent encephalitis of unknown origin.
Title: Sindrome de Kleine-Levin: diagnostico diferencial en los sindromes encefaliticos recurrentes del adolescente.
Introduccion. El sindrome de Kleine-Levin es una enfermedad rara de causa desconocida que se caracteriza por episodios recurrentes autolimitados de hipersomnia acompañados de alteracion cognitiva y conductual. Entre los episodios, los pacientes tienen un patron de sueño y cognitivo normal. Casos clinicos. Se presentan tres pacientes de 14 años, dos chicos y una chica. Comenzaron tras un desencadenante (vacuna, una infeccion respiratoria por influenza B; en el caso de la chica, coincidian con la menstruacion). En el episodio agudo mostraban tendencia al sueño y en vigilia destacaba bradipsiquia, inquietud motora y gran labilidad emocional, con tendencia al llanto y necesidad de la presencia de los familiares. Presentaron una duracion aproximada de 10-15 dias y periodicidad mensual, y se mostraron asintomaticos entre los episodios. Los tres pacientes fueron valorados por pediatras, diagnosticados y tratados de encefalitis autoinmune. Solo uno cumplia la triada tipica de hipersomnia, hiperfagia e hipersexualidad, pero ninguno de los tres datos se habia recogido en la historia clinica inicial y la familia solo lo referia tras una anamnesis dirigida. Conclusiones. En el sindrome de Kleine-Levin, los sintomas neurologicos durante el cuadro agudo son aun mas frecuentes que los psiquiatricos. La tendencia al sueño y el hecho de que durante la vigilia no esten asintomaticos y se muestren lentos, apaticos, labiles e irascibles, situa en primer lugar la sospecha de sindrome encefalitico. Debemos tener presente esta entidad en encefalitis recurrentes de etiologia no filiada.