Kleine-levin syndrome: clinical course, polysomnography and multiple sleep latency test. Case report

Abstract

-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; allnight 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.KEY WORDS: Kleine-Levin syndrome, recurrent hypersomnia, sleep disorder, sleep, adolescent, polysomnography, Epworth Sleepiness Scale.Síndrome de Kleine-Levin: aspectos clínicos, polissonografia e teste das latências múltiplas do sono. Relato de caso.RESUMO -Relata-se o caso de paciente com síndrome de Kleine-Levin, de 17 anos de idade, sexo feminino, com períodos de hipersônia recorrente, severa e crônica. O início do quadro foi aos 15 anos de idade. Os períodos de hipersônia acompanhavam-se de hiperfagia, duravam cerca de 5 dias e repetiam-se a intervalos de 28 a 60 dias. A severidade da hipersônia impedia a participação em atividades escolares. Entre os períodos de hipersônia, a paciente encontrava-se assintomática, EEG, tomografia computadorizada cerebral, ressonância nuclear magnética cerebral, polissonografia, Teste das Latências Múltiplas do Sono (TLMS) e Escala de Sonolência Epworth (ESE), foram normais. Durante período com hipersônia, a polissonografia evidenciou latência do sono e latência REM reduzidas. TLMS teve latência do sono, em média, de 1,8 min; presença de um período REM em no segundo subteste; o índice da ESE foi acentuadamente elevado. PALAVRAS-CHAVE: síndrome de Kleine-Levin, hipersônia recorrente, distúrbio do sono, sono, adolescente, polissonografia, escala Epworth de sonolência.Kleine-Levin syndrome (KLS) is characterized by recurrent hypersomnia, usually starting in the second decade of life, and is associated with hyperphagia 1-4 . This report describes an adolescent female with severe and chronic KLS, focusing on clinical aspects, polysomnography, Multiple Sleep Latency Test (MSLT) 5 and the Epworth Sleepiness Scale (ESS) 6,7 .

A Polysomnography Study of Kleine–Levin Syndrome in a Single Center

“…Therefore, no specificity of SOREM events was detected in KLS patients, and these episodes cannot be a diagnostic criterion for KLS. [ 2 8 ]…”

“…[ 1 ] Although no population-based studies reporting on KLS prevalence are available, it is generally estimated that there are only 1.5–2.0 cases per million people. [ 2 3 4 ]…”

“…Some evidence exists that the duration slow-wave sleep periods decreases during the first half of the episodes, and the length of rapid eye movement (REM) sleep periods is diminished in the second half. [ 2 6 7 ] The sleep efficiency is usually lower in patients with KLS than in healthy people. The multiple sleep latency test (MSLT) revealed no difference in mean sleep latency (MSL) and the number of sleep-onset REM sleep (SOREM) between the episodes and interictal periods.…”

Kleine–Levin syndrome: a systematic review of 186 cases in the literature

“…The types of stroke reported were a multi-infarct dementia (Drake, 1987), a thalamic ischaemic stroke (McGilchrist et al, 1993) and traumatic haemorrhages of the right hemisphere (Chiu et al, 1989;Kostic et al, 1998;Pelin et al, 2004). The genetic diseases were heterogeneous and included a case of mosaicism with Robert's syndrome, phocomelia, mild mental retardation, optic atrophy, bilateral facial palsy (Hasegawa et al, 1998), a case with Prader-Willi syndrome (Gau et al, 1996), an unidentified disease with mental retardation and bilateral pyramidal syndrome (Livrea et al, 1977), another complex case of consanguinity, mental retardation, an ectodermal disorder (incontinentia pigmenti), acanthosis nigricans, and hereditary exostosis (Reimao and Shimizu, 1998) and developmental Asperger's disease in two patients, one with cortical dysplasia and retinitis pigmentosa (Berthier et al, 1992). As for the three patients with infectious encephalitis of unknown origin, one had an acute viral meningo-encephalitis and high CSF lymphocyte counts (Merriam, 1986) while another had a meningo-encephalitis with neurological sequels, including left hypo-sensitivity, central facial palsy, concentric loss of visual fields and bilateral facial spasms (Persson et al, 1969).…”

Kleine-Levin syndrome: clues to aetiology

“…A number of abnormal sleep patterns have been described in KLS [ 19 , 20 ], but polysomnographic studies have most often been normal [ 21 – 23 ]. A reduction in slow wave sleep early in attacks with a return to normal despite persistent symptoms and, conversely, normal rapid eye movement (REM) sleep early in an attack but decreased REM sleep as the attack progresses, have been described [ 24 ].…”