Kleine-Levin syndrome: a systematic study of 108 patients.

OBJECTIVE: Kleine-Levin syndrome is a rare disorder characterized by relapsing-remitting episodes of hypersomnia, cognitive disturbances, and behavioral disturbances, such as hyperphagia and hypersexuality. METHODS: We collected detailed clinical data and blood samples on 108 patients, 79 parent pairs, and 108 matched control subjects. We measured biological markers and typed human leukocyte antigen genes DR and DQ. RESULTS: Novel predisposing factors were identified including increased birth and developmental problems (odds ratio, 6.5). Jewish heritage was overrepresented, and five multiplex families were identified. Human leukocyte antigen typing was unremarkable. Patients were 78% male (mean age at onset, 15.7 +/- 6.0 years), averaged 19 episodes of 13 days, and were incapacitated 8 months over 14 years. The disease course was longer in men, in patients with hypersexuality, and when onset was after age 20. During episodes, all patients had hypersomnia, cognitive impairment, and derealization; 66% had megaphagia; 53% reported hypersexuality (principally men); and 53% reported a depressed mood (predominantly women). Patients were remarkably similar to control subjects between episodes regarding sleep, mood, and eating attitude, but had increased body mass index. We found marginal efficacy for amantadine and mood stabilizers, but found no increased family history for neuropsychiatric disorders. INTERPRETATION: The similarity of the clinical and demographic features across studies strongly suggests that Kleine-Levin syndrome is a genuine disease entity. Familial clustering and increased prevalence in the Jewish population support a role for a major genetic susceptibility factor. Considering the inefficacy of available treatments, we propose that disease management should primarily be supportive and educational.

Kleine-Levin syndrome and psychosis: observation from an unusual case.

OBJECTIVE: This study evaluated the possible pathologic relation between Kleine-Levin syndrome (KLS) and mood disorders. BACKGROUND: A 28-year-old man with a remote history of KLS had the sudden onset of a manic episode with psychotic features after the end of hypersomnolence. METHOD: The patient received an extensive laboratory examination, including single photon emission computed tomography and magnetic resonance imaging. RESULTS: Single photon emission computed tomography showed decreased tracer perfusion in the basal ganglion, hypothalamus, and right frontotemporal region. Magnetic resonance imaging revealed a cystic lesion in the pineal region. CONCLUSIONS: Hypothalamic dysfunction has been described in KLS and mood disorders, but pineal gland dysfunction has been mentioned only rarely. The clinical and neuroimaging findings suggest the need for further study of KLS.

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

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