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Kleine-levin syndrome

Y Hu, J Y Wang, X S Dong, J Li, H Yan, P P Wang, L Zhao, X Z Zhang, F Han
Objective: To analyze the clinical features of Kleine-Levin syndrome (KLS) patients. Methods: Clinical data of 44 patients with KLS of the Sleep Center of Peking University People's Hospital from January 2002 to July 2013 were systematically reviewed. The predisposing factors and clinical presentations were summarized, and compared with the data from a Western KLS study with a large subjects number. Nocturnal polysomnography (PSG) and multiple sleep latency test (MSLT) were conducted during relapse and remission period, respectively...
April 25, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Natan Gadoth, Arie Oksenberg
Since 1962, when Critchley and Hoffman coined the term Kleine-Levin Syndrome (KLS) for the triad of hypersomnia, excessive eating and "often abnormal behavior" which they have observed in 11 adolescent boys, the number of patients recognized with this rare syndrome expanded, the spectrum of the clinical presentation, disease course, prognosis, gender specificity and the presence of familial cases were established. However, in spite of the progress made in neuroscience, the search for the cause, neuroanatomy, pathophysiology and drug treatment of KLS is still ongoing...
April 20, 2017: Brain & Development
A Duat-Rodriguez, I Martinez-Albadalejo, I Perez-Sebastian, V Cantarin-Extremera, A Hedrera-Fernandez, J J Garcia-Penas
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)...
April 1, 2017: Revista de Neurologia
Clément Drouet, Olivier Morel, Antoine Verger, Eric Guedj, Hatem Boulahdour
We report the case of a 14-year-old girl with clinically diagnosed Kleine-Levin syndrome. Electroencephalogram and brain MRI were unremarkable. An FDG PET/CT scan was acquired during a symptomatic episode, while she was asleep, and another PET scan was obtained when she was asymptomatic, awake, and quiet. Comparison of both examinations demonstrated a bilateral activation of thalami, caudate nuclei, and lenticular nuclei. These findings may be linked to the implication of thalamostriatal structures in the regulation of sleep and wakefulness...
May 2017: Clinical Nuclear Medicine
Olufunke Afolabi-Brown, Thornton B A Mason
Kleine-Levin syndrome (KLS) is a rare disorder characterized by discrete episodes of hypersomnia associated with cognitive and behavioural abnormalities, as well as normal alertness and function between episodes. The prevalence of KLS may be underestimated as it is often misdiagnosed and managed as another sleep disorder, neurological disorder or psychiatric condition. KLS is more typically seen in adolescence than at other ages, and is more common in males than in females. There are currently neither standard biomarkers nor specific imaging study findings, making the diagnosis of KLS a challenge...
December 23, 2016: Paediatric Respiratory Reviews
Elisa Evangelista, Lucie Barateau, Yves Dauvilliers
Excessive sleepiness is a common problem, defined by a complaint of excessive daytime sleepiness almost daily with an inability to stay awake and alert dosing periods at sleep, with episodes of irresistible sleep need or drowsiness or non-intentional sleep, or by a night's sleep time overly extended often associated with sleep inertia. This sleepiness is variable in terms of phenotype and severity to be specified by the out-patient clinic. It is considered to be chronic beyond three months and often responsible for significant functional impairment of school and professional performance, of the accidents and cardiovascular risk...
June 2016: La Revue du Praticien
S Pillen, N L E Vandenbussche, R Fronczek, J van Duijn, G J Lammers, S Overeem
BACKGROUND: Kleine Levin Syndrome (KLS) is a rare disease with periodic hypersomnia as its main feature. Hyperphagia and hypersexuality are also described as classical symptoms, although quite recently it has become clear that the full triad is absent in the majority of patients. CASE DESCRIPTION: A 14-year-old boy developed KLS after a period of flu-like symptoms. Over the course of three years he suffered from seven one-week episodes of extreme hypersomnia (sleeping 18 hours a day), depersonalisation, apathy, anxiety, paranoia, confusion, hallucinations and uninhibited sexual behaviour...
2016: Nederlands Tijdschrift Voor Geneeskunde
Yan-Wen Luo, Huan Yu, Lu-Hua Yuan, Guo-Xing Zhu
BACKGROUND: Kleine-Levin syndrome (KLS) is a rare sleep disorder characterized by recurrent episodes of hypersomnia. Polysomnographic (PSG) researches of KLS have been reported only in few publications in the past decades. This study aimed to investigate the characteristics of PSG of KLS. METHODS: This study, which was conducted from March 2010 to July 2014, included seven patients diagnosed with KLS in the Sleep and Wake Disorder Center of Huashan Hospital, Fudan University (Shanghai, China)...
July 5, 2016: Chinese Medical Journal
Alexander D Nesbitt, Guy D Leschziner
Recurrent hypersomnia, or Kleine-Levin syndrome, is rare and frequently causes substantial diagnostic anxiety and delay. Patients often undergo multiple investigations to rule out other causes of encephalopathy. The treatment options are unsatisfactory. Migraine with brainstem aura has not previously been widely considered in the medical literature as a differential diagnosis. We describe two patients referred to a tertiary sleep neurology service with a putative diagnosis of Kleine-Levin syndrome. Each described attacks of hypersomnia with elements of migraine with brainstem aura, in addition to having a history of migraine with aura...
October 2016: Practical Neurology
Quang Tuan Remy Nguyen, Elisabeth Groos, Laurène Leclair-Visonneau, Christelle Monaca-Charley, Tom Rico, Neal Farber, Emmanuel Mignot, Isabelle Arnulf
STUDY OBJECTIVES: Kleine-Levin syndrome (KLS) is a rare, mostly sporadic disorder, characterized by intermittent episodes of hypersomnia plus cognitive and behavior disorders. Although its cause is unknown, multiplex families have been described. We contrasted the clinical and biological features of familial versus sporadic KLS. METHODS: Two samples of patients with KLS from the United States and France (n = 260) were studied using clinical interviews and human leukocyte antigen (HLA) genotyping...
August 1, 2016: Sleep
Oussama Habra, Raphaël Heinzer, Jose Haba-Rubio, Andrea O Rossetti
STUDY OBJECTIVE: Kleine-Levin syndrome (KLS) is a rare disease of unknown etiology, the diagnosis of which can be challenging. We aimed to estimate KLS prevalence in French-speaking Switzerland, and assess differences with mimicking conditions. METHODS: In this cross-sectional study, KLS patients were identified through a population-based approach, including at our hospital and contacting all sleep-certified facilities and neurologists in French-speaking Switzerland...
August 15, 2016: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
Maria Engström, Anne-Marie Landtblom, Thomas Karlsson
Previous studies have indicated involvement of the thalamus and the pons in Kleine-Levin syndrome. In the present study, functional connectivity of the thalamus and the pons was investigated in asymptomatic patients with Kleine-Levin syndrome and healthy controls. Twelve patients and 14 healthy controls were investigated by functional magnetic resonance imaging during rest. Resting state images were analysed using seed regions of interest in the thalamus and the pons. The results showed significantly lower functional connectivity between the pons and the frontal eye field in persons with Kleine-Levin syndrome compared with healthy controls...
December 2016: Journal of Sleep Research
Marcio M de Oliveira, Cristiane Conti, Gilmar F Prado
BACKGROUND: This is an updated version of the original Cochrane review, published in 2009, Issue 2.Kleine-Levin syndrome (KLS) is a rare disorder that mainly affects adolescent men. It is characterised by recurrent episodes of hypersomnia, usually accompanied by hyperphagia, cognitive and mood disturbances, abnormal behaviour, such as hypersexuality, and signs of dysautonomia.In 1990, the diagnostic criteria for Kleine-Levin syndrome were modified in the International Classification of Sleep Disorders, where KLS was defined as a syndrome comprised of recurring episodes of undue sleepiness lasting some days, which may or may not be associated with hyperphagia and abnormal behaviour...
May 6, 2016: Cochrane Database of Systematic Reviews
Mitchell G Miglis, Christian Guilleminault
Kleine-Levin syndrome is a rare recurrent hypersomnia associated with symptoms of behavioral and cognitive impairment. This article reviews common presenting symptoms, differential diagnosis, diagnostic workup, and potential treatment options. Current updates on functional imaging studies and long-term neuropsychological studies are reviewed.
June 2016: Current Neurology and Neuroscience Reports
Oliver Sum-Ping, Christian Guilleminault
Kleine-Levin Syndrome [KLS] is often under-recognized and also misdiagnosed. When suspicion for KLS is raised, a thorough clinical evaluation should be performed, including detailed history from family members and a neurologic and psychiatric examination. Additional studies may include PSG, EEG, neuroimaging, as well as serological and CSF studies to rule out alternative diagnoses as clinically indicated. After arriving at a diagnosis of KLS, the foundation of care is supportive. Patients and their families should be provided with education about the disease...
June 2016: Current Treatment Options in Neurology
Berthe Assi, Constance Yapo-Ehounoud, Mohamed Ben Allaoui Baby, Evelyne Aka-Diarra, Muriel Amon-Tanoh, Christian Tanoh
The Kleine-Levin syndrome is a rare pathology characterized by recurrent episodes of hypersomnia associated with behavioral and cognitive disorders with, among others, hyperphagia and hypersexuality. The disease mainly affects young males. A few studies mention cases that occurred in Africa, especially in Côte d'Ivoire. In this paper, we report the very first two cases observed in the Neurology Department of the University Hospital of Cocody. The diagnosis was clinical, based on the recurrence of hypersomnia, cognitive and behavioral disorders during the periods of hypersomnia, and the return of patients to normal state between episodes...
2016: Case Reports in Neurological Medicine
Jing Yu Wang, Fang Han, Song X Dong, Jing Li, Pei An, Xiao Zhe Zhang, Yuan Chang, Long Zhao, Xue Li Zhang, Ya Nan Liu, Han Yan, Qing Hua Li, Yan Hu, Chang Jun Lv, Zhan Cheng Gao, Kingman P Strohl
STUDY OBJECTIVES: Kleine-Levin syndrome (KLS) is a rare disorder of relapsing sleepiness. The hypothesis was that the syndrome is related to a change in the vigilance peptide orexin A. METHODS: From 2002 to 2013, 57 patients with relapsing hypersomnolence were clinically assessed in a referral academic center in Beijing, China, and 44 (28 males and 16 females; mean age 18.3 ± 8.9 y (mean ± standard deviation, range 9-57 y) were determined to have clinical and behavioral criteria consistent with KLS...
April 1, 2016: Sleep
Jacqueline M Arnone, Richard P Conti
Kleine-Levin syndrome (KLS) is a neuropsychiatric sleep disorder primarily affecting adolescent males. Onset is insidious, idiopathic, and hastened by neurological incident or infection. Typically, the initial onset occurs during the teen years or after the second decade, although cases have been documented in early childhood, adulthood, and senescence. KLS is marked by unexpected debilitating, yet reversible, episodic hypersomnia, with varying recurrence rates; cognitive and behavioral impairment; compulsive eating; and feelings of derealization, hypersexuality, apathy, and depressed mood...
March 2016: Journal of Psychosocial Nursing and Mental Health Services
Jacob Feigal, Chen Lin, George Barrio, Peter G Kranz, Xavier Preud׳Homme
No abstract text is available yet for this article.
May 2016: Psychosomatics
Saad M Al Suwayri
OBJECTIVES: To highlight the occurrence of familial cases and addresses, whether familial Kleine-Levine syndrome (KLS) presents the same spectrum of disease, as that seen in sporadic KLS.   METHODS: Between September and December 2014, reports of familial cases of KLS were identified by searching the Library of Congress, PubMed, and Web of Science databases restricted to the English language, with no restriction on date of publication. All cases were reviewed to identify familial cases consistent with current diagnostic criteria for sporadic KLS...
January 2016: Saudi Medical Journal
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