Usefulness of brain SPECT imaging in the study of recurrent hypersomnia: Kleine-Levin syndrome
Publication date: Available online 11 June 2019Source: Neurología (English Edition)Author(s): D. Ramírez Ocaña, E. Espinosa Muñoz, C. Puentes Zarzuela (Source: Neurologia)
Source: Neurologia - June 12, 2019 Category: Neurology Source Type: research

Excessive daytime sleepiness in narcolepsy and central nervous system hypersomnias.
CONCLUSIONS: Different CNS hypersomnias present with specific features of EDS are useful to guide the clinician to apply and interpret appropriate neurophysiological investigations. PMID: 31154621 [PubMed - as supplied by publisher] (Source: Sleep and Breathing)
Source: Sleep and Breathing - May 31, 2019 Category: Sleep Medicine Authors: Pizza F, Filardi M, Moresco M, Antelmi E, Vandi S, Neccia G, Mazzoni A, Plazzi G Tags: Sleep Breath Source Type: research

The neurophysiological basis of excessive daytime sleepiness: suggestions of an altered state of consciousness.
Abstract Excessive daytime sleepiness (EDS) is characterized by difficulty staying awake during daytime, though additional features may be present. EDS is a significant problem for clinical and non-clinical populations, being associated with a range of negative outcomes that also represent a burden for society. Extreme EDS is associated with sleep disorders, most notably the central hypersomnias such as narcolepsy, Kleine-Levin syndrome, and idiopathic hypersomnia (IH). Although investigation of these conditions indicates that EDS results from diminished sleep quality, the underlying cause for this impairm...
Source: Sleep and Breathing - May 27, 2019 Category: Sleep Medicine Authors: Hitchcott PK, Menicucci D, Frumento S, Zaccaro A, Gemignani A Tags: Sleep Breath Source Type: research

New-Onset Psychosis in a Child with Suspected Kleine –Levin Syndrome
Journal of Child and Adolescent Psychopharmacology,Volume 29, Issue 2, Page 158-161, March 2019. (Source: Journal of Child and Adolescent Psychopharmacology)
Source: Journal of Child and Adolescent Psychopharmacology - March 6, 2019 Category: Pediatrics Authors: Timothy Rice Jasmine Tatum Natasha Juneja Barbara J. Coffey Source Type: research

An Atypical Case of Kleine-Levin Syndrome.
Authors: Edaki O, Khawaja IS, Schenck CH PMID: 30664337 [PubMed - in process] (Source: The Primary Care Companion for CNS Disorders)
Source: The Primary Care Companion for CNS Disorders - January 23, 2019 Category: Primary Care Tags: Prim Care Companion CNS Disord Source Type: research

Late diagnosis of a rare urea cycle disorder mimicking Kleine-Levin syndrome
We describe a young man with a late-onset urea cycle disorder who initially presented with phenomenology akin to KLS. (Source: Neurology Clinical Practice)
Source: Neurology Clinical Practice - December 10, 2018 Category: Neurology Authors: Bozinov, N., Han, M., Lau, W., Santini, V. Tags: MRI, All Clinical Neurology, Metabolic disease (inherited) Case Source Type: research

Neuroimaging in the Kleine-Levin Syndrome
AbstractPurpose of ReviewThe purpose was to review the most recent literature on neuroimaging in the Kleine-Levin syndrome (KLS). We aimed to investigate if frontotemporal and thalamic dysfunction are key KLS signatures, and if recent research indicates other brain networks of interest that elucidate KLS symptomatology and aetiology.Recent FindingsIn a comprehensive literature search, we found 12 original articles published 2013 –2018. Most studies report deviations related to cerebral perfusion, glucose metabolism, or blood-oxygen-level-dependent responses in frontotemporal areas and/or the thalamus. Studies also report...
Source: Current Neurology and Neuroscience Reports - July 21, 2018 Category: Neuroscience Source Type: research

Kleine ‐Levin syndrome is associated with LMOD3 variants
Journal of Sleep Research, EarlyView. (Source: Journal of Sleep Research)
Source: Journal of Sleep Research - June 20, 2018 Category: Neurology Authors: Saad M. Al Shareef , Sulman Basit , Sha Li , Corinne Pfister , Sylvain Pradervand , Michel Lecendreux , Geert Mayer , Yves Dauvilliers , Vincenzo Salpietro , Henry Houlden , Ahmed S. BaHammam , Mehdi Tafti Source Type: research

Kleine-Levin syndrome is associated with LMOD3 variants.
Abstract Kleine-Levin syndrome (KLS) is a rare periodic hypersomnia with associated behavioural abnormalities but with often favourable prognosis. There is excess risk of KLS in first-degree relatives, suggesting a strong genetic contribution. So far, no mutation is identified in KLS and comprehensive genetic analysis of affected individuals is lacking. Here we performed whole genome single-nucleotide polymorphism (SNP) genotyping and exome sequencing in a large family with seven affected members. The identified gene with a mutation was resequenced in 38 sporadic KLS patients and the expression of the gene...
Source: Journal of Sleep Research - June 19, 2018 Category: Sleep Medicine Authors: Al Shareef SM, Basit S, Li S, Pfister C, Pradervand S, Lecendreux M, Mayer G, Dauvilliers Y, Salpietro V, Houlden H, BaHammam AS, Tafti M Tags: J Sleep Res Source Type: research

Fluctuating CSF hypocretin-1 levels in mild brain trauma-induced Kleine-Levin syndrome
Kleine-Levin syndrome (KLS) is a rare sleep disorder, mainly affecting adolescent boys, and is characterized by recurrent hypersomnia combined with cognitive, mood, and/or behavioral disturbances [1]. It typically follows various precipitating factors such as infection, alcohol-drinking, and mental and physical stress [1]. Although the precise pathophysiology of KLS remains unclear, several functional neuroimaging studies have shown hypoperfusion in the hypothalamus, thalamus, caudate nucleus, and associative cortical areas during symptomatic and/or asymptomatic periods in patients with KLS [2]. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - May 17, 2018 Category: Neurology Authors: Maho Usuda, Minori Kodaira, Yuka Ogawa, Takashi Kanbayashi, Shin Yanagisawa, Yoshiki Sekijima Tags: Letter to the Editor Source Type: research

Emerging psychiatric disorders in Kleine ‐Levin syndrome
Journal of Sleep Research, EarlyView. (Source: Journal of Sleep Research)
Source: Journal of Sleep Research - April 14, 2018 Category: Neurology Authors: Elisabeth Groos , Charlotte Chaumereuil , Mathilde Flamand , Agnes Brion , Hubert Bourdin , Vanessa Slimani , Michel Lecendreux , Isabelle Arnulf Source Type: research

Emerging psychiatric disorders in Kleine-Levin syndrome.
Abstract In Kleine-Levin syndrome (KLS), episodes of hypersomnia and cognitive, psychiatric and behavioural disturbances alternate with asymptomatic periods in adolescents. We evaluated whether psychiatric disorders would emerge during asymptomatic periods in a naturalistic, uncontrolled clinical cohort. Patients with primary KLS underwent psychiatric interviews at diagnosis and every year for 1-10 years, leading to diagnosis of former and present comorbid psychiatric disorders. Among the 115 patients (65.2% male and aged 16.1 ± 4.8 years at KLS onset), 19 (16.5%) had a history of psychiatric disorder...
Source: Journal of Sleep Research - April 14, 2018 Category: Sleep Medicine Authors: Groos E, Chaumereuil C, Flamand M, Brion A, Bourdin H, Slimani V, Lecendreux M, Arnulf I Tags: J Sleep Res Source Type: research

Kleine-Levin syndrome: the contribution of PET scan and hydrogen breath test for small intestinal bacterial overgrowth
A 13 year-old boy started to present recurrent episodes, lasting from 3 to 12 days, characterized by prolonged sleep time, megaphagia, derealization, mood depression, rudeness and hypersexual behaviour. Episodes occurred after infective diseases, moderate alcohol assumption or sleep deprivation, and were often heralded by terrifying visual hallucinations. Physical examination, blood tests, lumbar puncture, EEG, brain CT scan and MRI were normal. The patient arrived at our lab at the age of 15, during a relapse. (Source: Sleep Medicine)
Source: Sleep Medicine - April 5, 2018 Category: Sleep Medicine Authors: Andrea Melpignano, Giulia Milioli, Anna Bertel è, Anna Elisabetta Vaudano, Caterina Ghetti, Liborio Parrino, Livia Ruffini Source Type: research

Kleine-Levin syndrome: A neuropsychiatric disorder.
Authors: Arnulf I, Groos E, Dodet P Abstract Kleine-Levin syndrome (KLS) is a rare, relapsing-remitting disease that affects mostly adolescents. It is characterized by episodes lasting from 1 to several weeks, and comprises neurological (hypersomnia, confusion, slowness, amnesia) and neuropsychiatric symptoms (derealization and apathy). Some psychiatric symptoms (megaphagia, hypersexuality, anxiety, depressed mood, hallucinations, delusions) arise during episodes, albeit less frequently, while patients are normal between episodes. However, sudden severe (>18h/day of sleep) and recurrent hypersomnia help...
Source: Revue Neurologique - April 4, 2018 Category: Neurology Tags: Rev Neurol (Paris) Source Type: research