General Anesthetic Management of a Patient With Kleine-Levin Syndrome.

Kleine-Levin syndrome (KLS) is a rare sleep disorder characterized by periodic hypersomnia and behavioral or cognitive disturbances. Although prolonged emergence from general anesthesia and postoperative hypersomnia may occur in a patient with KLS, there is little information about the safe anesthetic management of these patients. We describe the case of a 22-year-old female previously diagnosed with KLS who was scheduled to have her third molars extracted under general anesthesia. Because the patient had symptoms of periodic hypersomnia and hyperphagia, the surgery was scheduled during a KLS crisis interval. General anesthesia was induced with propofol, remifentanil, and rocuronium, and maintained with desflurane and remifentanil. To prevent overuse of anesthetic agents, an electroencephalogram (EEG)-based depth of anesthesia monitor (SedLine; Masimo Corporation) was used intraoperatively. A neuromuscular monitor was also used to carefully titrate use of a neuromuscular blocking agent. After surgery, sugammadex was administered, and the patient quickly emerged within 10 minutes, as also confirmed by the EEG monitor. She had no KLS recurrence postoperatively. When anesthetizing patients with KLS, an EEG-based depth of anesthesia monitor and neuromuscular monitor may be warranted to ensure complete emergence from general anesthesia. In addition, elective surgery should be planned during crises intervals.

An unusual occurrence of Kleine-Levin syndrome in a man with refractory immune thrombocytopenic purpura: a case report.

INTRODUCTION: Kleine-Levin syndrome is an extremely rare neurological entity characterized by recurrent episodes of hypersomnia which are sometimes associated with compulsive hyperphagia and behavioral changes. Autoimmunity has recently been proposed as a factor contributing to its pathogenesis. Immune thrombocytopenic purpura is a relatively common autoimmune disease showing a lot of complexity and uncertainty regarding its treatment regimens and its refractory nature in some cases. CASE PRESENTATION: A 32-year-old Persian White man visited his private hematologist complaining of recent episodes of epistaxis and appearance of petechial lesions 24 hours after receiving a meningococcal vaccine. He had a history of immune thrombocytopenic purpura 13 years before his presentation. Based on his history and laboratory findings, his condition was diagnosed as a relapse of immune thrombocytopenic purpura and was managed accordingly. He did not respond to first-line corticosteroid regimens and later developed neurological symptoms as recurrent episodes of hypersomnia and hyperphagia. After a complete clinical and paraclinical evaluation and ruling out other possible conditions, he was given a diagnosis of Kleine-Levin syndrome. He was followed up for his immune thrombocytopenic purpura and received different treatment regimens none of which were adequately successful except intravenous immunoglobulin that was only temporarily effective. He has had 4 documented self-limited episodes of Kleine-Levin syndrome since his initial presentation. CONCLUSIONS: Immune thrombocytopenic purpura may be associated with meningococcal vaccination in adulthood. Responses to treatment in immune thrombocytopenic purpura vary among patients. Our patient only had a transient acceptable response to intravenous immunoglobulin while all other options failed to improve his platelet count. Concurrence of immune thrombocytopenic purpura and Kleine-Levin syndrome supports the role of autoimmunity as the proposed pathophysiological mechanism of Kleine-Levin syndrome.

Sarcoidosis: a rare cause of Kleine-Levine-Critchley syndrome.

Hypothalamic sarcoidosis is a rare entity that can alter the hypothalamic-pituitary axis and induce various combinations of endocrine changes. We present a case of neurosarcoidosis with uncommon features of hypersomnolense and hyperphagia. Current strategies to increase awareness and prevention of the harmful effects of obesity require clinicians to be cognizant of potential disorders that produce these features The mechanism, differential diagnosis and therapeutic options of this organic etiology are reviewed.

Kleine-Levin syndrome: interface between neurology and psychiatry

We report the first episode of Kleine-Levin (KLS) syndrome in a 17-year-old male. The illness onset, clinical features, neuropsychological evaluation and polysomnographic recording are described. Typical symptoms hypersomnia, hyperphagia and sexual disinhibition were observed besides behavioral disturbances, polysonographic and neuropsychological alterations. Behavioral disturbances similar to a manic episode including psychotic symptoms were relevant. The pharmacologic treatment included lithium, methylphenidate and risperidone. The introduction of risperidone aimed the control of psychotic symptoms and the persistent manifestations of hypersexuality after sleepness control and to the best of our knowledge there are no other report regarding risperidone use for KLS in the literature.

Relatamos o primeiro episódio de síndrome de Kleine-Levin (SKL) num adolescente de 17 anos. São descritos o modo de aparecimento da doença, achados clínicos, avaliação neuropsicológica e polissonografia. Foram observados sintomas típicos como hipersonolência, hiperfagia e desinibição sexual, e outras alterações comportamentais, polissonográficas e neuropsicológicas. As alterações comportamentais assemelhadas a um episódio de mania, incluindo sintomas psicóticos, foram relevantes. O tratamento farmacológico incluiu lítio, metilfenidato e risperidona. A introdução de risperidona teve por objetivo o controle dos sintomas psicóticos e das persistentes manifestações da hipersexualidade, que permaneceram após diminuição da sonolência; não temos conhecimento de relatos anteriores, na literatura, de seu uso no tratamento da SKL.

Kleine-Levin syndrome.

Kleine-Levin Syndrome (KLS) is a rare disorder of uncertain etiology, characterized by recurring episodes of undue sleepiness lasting for days. We report a case of young female presenting with episodes of undue sleep along with hypersexuality and excessive food intake, who improved significantly on lithium and valproate. KLS should be considered in young patients, who present with episodes of undue somnolence.

[Klein-Levin syndrome: a neurological disease with psychiatric symptoms]. / Le syndrome de Kleine-Levin: une affection neurologique à symptomatologie psychiatrique.

Kleine-Levin syndrome belongs to the recurrent hypersomnias group. It is a rare and benignant disease, occurring in young men 10 to 25 years old. The diagnosis is first clinical and the hypersomniac episodes, joined to psychiatric symptoms, are irregularly recurrent during few years. Diagnosis is uneasy during the first episode and in the attenuated forms... 500 cases have been described all around the world but it's highly likely that many patients haven't been listed. This syndrome, just like Gélineau disease, stands in the group of primary pathological hypersomnias. In a clinical point of view, the cardinal and constant symptom is hypersomnia. Psychiatric symptoms can be irregularly joined: megaphagia, sexual behavioural disorders, thymic disorders, personality modifications. The clinical examination is poor and aspecific. During an hypersomniac episode, a polygraphic recording during 24 or 48 hours will give diagnosis informations (fragmented and unstable sleep, reduction in stages 3 and 4 of non-REM sleep, reduction in REM sleep latency) and a biological and radiological evaluation will be necessary to exclude organic etiology (tumoral progres, infectious disease...). In a therapeutic point of view, prescription of psychostimulant drugs is recommended during fits and some treatments are used in a preventive way (lithium and carbamazepine).

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

Kleine-Levin syndrome: clinical course, polysomnography and multiple sleep latency test: case report

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; all-night 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.

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