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Narcolepsy

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https://www.readbyqxmd.com/read/28728625/parental-fitness-questioned-on-the-grounds-of-narcolepsy-presentation-of-two-cases
#1
Laura Barbero, Annamaria Govi, Fabio Pizza, Giuseppe Plazzi, Francesca Ingravallo
We report two cases of fathers whose parental fitness was questioned during divorce and custody litigation because of narcolepsy type 2 and type 1, respectively. These cases highlighted both the existence of a narcolepsy-related stigma and the need to involve sleep experts in custody assessments when concerns about the parental fitness are related to a sleep disorder, expanding the field of interest of the growing "sleep forensics."
July 14, 2017: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
https://www.readbyqxmd.com/read/28726523/investigational-therapies-for-the-treatment-of-narcolepsy
#2
Stefano de Biase, Annacarmen Nilo, Gian Luigi Gigli, Mariarosaria Valente
Narcolepsy is a chronic sleep disorder characterized by a pentad of excessive daytime sleepiness (EDS), cataplexy, sleep paralysis, hypnagogic/hypnopompic hallucinations, and disturbed nocturnal sleep. While non-pharmacological treatments are sometimes helpful, more than 90% of narcoleptic patients require a pharmacological treatment. Areas covered: The present review is based on an extensive Internet and PubMed search from 1994 to 2017. It is focused on drugs currently in development for the treatment of narcolepsy...
July 20, 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/28724928/on-wakefulness-fluctuations-as-a-source-of-bold-functional-connectivity-dynamics
#3
Ariel Haimovici, Enzo Tagliazucchi, Pablo Balenzuela, Helmut Laufs
Human brain dynamics and functional connectivity fluctuate over a range of temporal scales in coordination with internal states and environmental demands. However, the neurobiological significance and consequences of functional connectivity dynamics during rest have not yet been established. We show that the coarse-grained clustering of whole-brain dynamic connectivity measured with magnetic resonance imaging reveals discrete patterns (dynamic connectivity states) associated with wakefulness and sleep. We validate this using EEG in healthy subjects and patients with narcolepsy and by matching our results with previous findings in a large collaborative database...
July 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28707076/correlation-of-changes-in-patient-reported-quality-of-life-with-physician-rated-global-impression-of-change-in-patients-with-narcolepsy-participating-in-a-clinical-trial-of-sodium-oxybate-a-post-hoc-analysis
#4
Richard K Bogan, Jed Black, Todd Swick, Mortimer Mamelak, Ruzica Kovacevic-Ristanovic, Kathleen F Villa, Fannie Mori, Jacques Montplaisir
INTRODUCTION: Narcolepsy patients report lower health-related quality of life (HRQoL) than the general population, as measured by the Short Form-36 Health Survey (SF-36). This analysis evaluated whether changes in SF-36 correlated with physician-rated Clinical Global Impression of Change (CGI-C). METHODS: Data were from 209 of 228 narcolepsy patients participating in an 8-week clinical trial of sodium oxybate. Changes from baseline for SF-36 subscales (Physical Functioning, Role Physical, Bodily Pain, General Health, Vitality, Social Functioning, Role Emotional, and Mental Health) and the summary scores were evaluated for correlation with CGI-C overall and by treatment group...
July 13, 2017: Neurology and Therapy
https://www.readbyqxmd.com/read/28691719/narcolepsy-type-1-and-hypersomnia-associated-with-a-psychiatric-disorder-show-different-slow-wave-activity-dynamics
#5
Ewa Walacik-Ufnal, Anna Justyna Piotrowska, Dorota Wołyńczyk-Gmaj, Piotr Januszko, Bartłomiej Gmaj, Marcin Ufnal, Marek Kabat, Marcin Wojnar
The aim of the study was to compare electrophysiological parameters of night sleep in narcolepsy type 1 and hypersomnia associated with a psychiatric disorder. Fortyfour patients: 15 with narcolepsy type 1, 14 with hypersomnia associated with a psychiatric disorder and 15 age- and sex-matched controls participated in the study. The study subjects filled in the Athens Insomnia Scale (AIS) and the Beck Depression Inventory (BDI). The severity of daytime sleepiness was quantified subjectively using the Epworth Sleepiness Scale (ESS) and the Stanford Sleepiness Scale (SSS), and objectively using the Multiple Sleep Latency Test (MSLT)...
2017: Acta Neurobiologiae Experimentalis
https://www.readbyqxmd.com/read/28684017/postnatal-treatment-of-women-with-narcolepsy-who-choose-to-breastfeed-time-for-a-reappraisal
#6
EDITORIAL
Todd J Swick
No abstract text is available yet for this article.
May 29, 2017: Sleep Medicine
https://www.readbyqxmd.com/read/28680929/orexins-and-the-cardiovascular-events-of-awakening
#7
REVIEW
Alessandro Silvani
This brief review aims to provide an updated account of the cardiovascular events of awakening, proposing a testable conceptual framework that links these events with the neural control of sleep and the autonomic nervous system, with focus on the hypothalamic orexin (hypocretin) neurons. Awakening from non-rapid-eye-movement sleep entails coordinated changes in brain and cardiovascular activity: the neural "flip-flop" switch that governs state transitions becomes biased toward the ascending arousal systems, arterial blood pressure and heart rate rise toward waking values, and distal skin temperature falls...
2017: Temperature: Multidisciplinary Biomedical Journal
https://www.readbyqxmd.com/read/28677318/the-cost-utility-of-sodium-oxybate-as-narcolepsy-treatment
#8
K Bolin, P Berling, P Wasling, H Meinild, J Kjellberg, P Jennum
AIMS AND OBJECTIVES: Based on class-I studies, sodium oxybate is regarded as a first-line treatment for both EDS and cataplexy. The cost-effectiveness of sodium oxybate is largely unknown, though. In this study, we estimate the cost-effectiveness of sodium oxybate as treatment for patients with narcolepsy as compared to standard treatment, by calculating incremental cost-effectiveness ratios (cost per quality-adjusted life year, QALY) for patients in a Swedish setting. MATERIALS AND METHODS: Calculations were performed using a Markov model with a 10-year time horizon...
July 5, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/28676060/the-development-of-a-health-related-quality-of-life-instrument-for-young-people-with-narcolepsy-narqol-21
#9
John E Chaplin, Attila Szakács, Tove Hallböök, Niklas Darin
BACKGROUND: Narcolepsy is a lifelong sleep disorder with a prevalence of between 0.03% and 0.06% and onset at around puberty. It is associated with psychiatric comorbidities and cognitive difficulties. No valid and reliable condition-specific health-related quality-of-life (HrQoL) instrument has been developed for this population. METHODS: A questionnaire based on four mixed-gender age-defined focus group discussions and a patient panel analysis was administered to young people with narcolepsy and a control group...
July 4, 2017: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/28675258/narcolepsy-cataplexy-and-psychosis-a-case-study
#10
F Canellas-Dols, C Delgado, C Arango-Lopez, R Peraita-Adrados
AIMS: To report a challenging patient a girl who developed narcolepsyy with cataplexy (NT1) and a psychosis during adolescence. To discuss diagnostic and therapeutic challenges of the comorbid cases. CASE REPORT: A 14-year-old girl was referred to Sleep and Epilepsy Unit for excessive daytime sleepiness, impaired nocturnal sleep, binge eating and weight gain, over the last year. After being diagnosed with a NT1 the patient was treated with modafinil and sodium oxybate...
July 16, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28668666/ghb-levels-in-breast-milk-of-women-with-narcolepsy-with-cataplexy-treated-with-sodium-oxybate
#11
Emily C Barker, Michelle Puchowicz, John Letterio, Kristine Higgins, Katherine M Sharkey
OBJECTIVE: To determine GHB levels in breast milk of women taking sodium oxybate (Xyrem) for treatment of narcolepsy and cataplexy. METHODS: Two women with narcolepsy and cataplexy treated with sodium oxybate before pregnancy collected breast milk for analysis of GHB concentration after resuming sodium oxybate postpartum. One woman collected samples across two consecutive nights (doses: 3.0 gm and 4.5 gm twice per night) five months after delivering her first child; the other collected samples on three separate days (doses: 2...
June 1, 2017: Sleep Medicine
https://www.readbyqxmd.com/read/28666745/the-clinical-spectrum-of-childhood-narcolepsy
#12
REVIEW
Emanuela Postiglione, Elena Antelmi, Fabio Pizza, Michel Lecendreux, Yves Dauvilliers, Giuseppe Plazzi
Narcolepsy type 1 is a life-long, severe, multifaceted disease often arising in childhood or adolescence. Beyond the classical symptoms (excessive daytime sleepiness, cataplexy, hallucinations, sleep paralysis and nocturnal fragmented sleep), metabolic, endocrinological, psychiatric and psychosocial aspects must be considered. Despite the increased awareness after H1N1 pandemic influenza and vaccination, narcolepsy is still misdiagnosed and unrecognized. The peculiar presentation of symptoms in narcoleptic children could in part explain the misdiagnoses...
May 8, 2017: Sleep Medicine Reviews
https://www.readbyqxmd.com/read/28658402/is-there-a-relationship-between-narcolepsy-multiple-sclerosis-and-hla-dqb1-06-02
#13
Paulo José Lorenzoni, Lineu Cesar Werneck, Ana Christina de Souza Crippa, Alessandra Zanatta, Cláudia S Kamoi Kay, Carlos Eduardo S Silvado, Rosana Herminia Scola
We studied multiple sclerosis (MS) patients with the HLA-DQB1*06:02 allele and compared them with MS patients who did not carry the HLA-DQB1*06:02 allele. We analyzed clinical and neurophysiological criteria for narcolepsy in six MS patients with HLA-DQB1*06:02, compared with 12 MS patients who were HLA-DQB1*06:02 non-carriers. Only two patients with HLA-DQB1*06:02 allele scored higher than 10 on the Epworth Sleepiness Scale. Polysomnography recording parameters and the multiple sleep latency test showed an absence of narcolepsy in the study group...
June 2017: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/28656632/serum-brain-derived-neurotrophic-factor-bdnf-in-sleep-disordered-patients-relation-to-sleep-stage-n3-and-rapid-eye-movement-rem-sleep-across-diagnostic-entities
#14
Michael Deuschle, Michael Schredl, Christian Wisch, Claudia Schilling, Maria Gilles, Olga Geisel, Rainer Hellweg
Experimental and clinical evidence suggests an association between neuroplasticity, brain-derived neurotrophic factor and sleep. We aimed at testing the hypotheses that brain-derived neurotrophic factor is associated with specific aspects of sleep architecture or sleep stages in patients with sleep disorders. We included 35 patients with primary insomnia, 31 patients with restless legs syndrome, 17 patients with idiopathic hypersomnia, 10 patients with narcolepsy and 37 healthy controls. Morning serum brain-derived neurotrophic factor concentrations were measured in patients and controls...
June 28, 2017: Journal of Sleep Research
https://www.readbyqxmd.com/read/28651750/reduced-orexin-immunoreactivity-in-perry-syndrome-and-multiple-system-atrophy
#15
Takayasu Mishima, Koji Kasanuki, Shunsuke Koga, Monica Castanedes-Casey, Zbigniew K Wszolek, Yoshio Tsuboi, Dennis W Dickson
INTRODUCTION: Orexin is a neuropeptide that plays a key role in maintaining a state of arousal, and possibly associates with sleep apnea syndrome (SAS). Reduced orexin immunoreactivity has been reported in various neurologic conditions such as narcolepsy, Alzheimer's disease, Lewy body disease and multiple system atrophy (MSA); however, there has been no report investigating orexin in Perry syndrome, a rare hereditary neurodegenerative disease characterized by four clinical cardinal signs (parkinsonism, depression/apathy, weight loss, and central hypoventilation)...
June 12, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28631980/orexin-research-patent-news-from-2016
#16
Christoph Boss, Catherine Roch
The orexin system consists of two G-protein-coupled receptors, orexin 1 and orexin 2 and two endogenous ligands, orexin A and orexin B . It is evolutionarily highly conserved. It is involved in the promotion of wakefulness as well as in anxiety and addictive disorders. In addition, its activation via the Ox1 receptor triggers apoptosis in several cancer cell lines. Dual orexin receptor antagonists are successfully used to treat primary insomnia. The major open questions are now related to the clinical validation of Ox1 selective antagonists...
June 28, 2017: Expert Opinion on Therapeutic Patents
https://www.readbyqxmd.com/read/28630298/hypocretin-orexin-is-critical-in-sustaining-theta-gamma-rich-waking-behaviors-that-drive-sleep-need
#17
Anne Vassalli, Paul Franken
Hcrt gene inactivation in mice leads to behavioral state instability, abnormal transitions to paradoxical sleep, and cataplexy, hallmarks of narcolepsy. Sleep homeostasis is, however, considered unimpaired in patients and narcoleptic mice. We find that whereas Hcrt(ko/ko) mice respond to 6-h sleep deprivation (SD) with a slow-wave sleep (SWS) EEG δ (1.0 to 4.0 Hz) power rebound like WT littermates, spontaneous waking fails to induce a δ power reflecting prior waking duration. This correlates with impaired θ (6...
July 3, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28619187/safety-and-efficacy-of-long-term-use-of-sodium-oxybate-for-narcolepsy-with-cataplexy-in-routine-clinical-practice
#18
Panagis Drakatos, Dimosthenis Lykouras, Grainne D'Ancona, Sean Higgins, Nadia Gildeh, Raluca Macavei, Ivana Rosenzweig, Joerg Steier, Adrian J Williams, Rexford Muza, Brian D Kent, Guy Leschziner
BACKGROUND: Sodium oxybate is licensed in Europe for the treatment of narcolepsy with cataplexy in adults. The aim of this study was to assess the efficacy and safety of sodium oxybate in clinical practice in patients with narcolepsy and cataplexy refractory to other treatments. MATERIALS AND METHODS: This was a retrospective single centre study including patients with severe narcolepsy with cataplexy refractory to other treatments, who were initiated on sodium oxybate between 2009 and 2015...
July 2017: Sleep Medicine
https://www.readbyqxmd.com/read/28619176/evaluation-of-the-effect-of-jzp-110-in-patients-with-narcolepsy-assessed-using-the-maintenance-of-wakefulness-test-censored-to-20-minutes
#19
Chad Ruoff, Richard K Bogan, Helen Emsellem, Neil Feldman, Alan Lankford, Gary Bream, Moise Khayrallah, Yuan Lu, Lawrence P Carter, Jed Black
OBJECTIVE: To evaluate the effects of JZP-110 on the Maintenance of Wakefulness Test (MWT) with data censored to include only the first 20 min of a 40-min MWT. METHODS: In a 4-week, placebo-controlled crossover design (Study 201; N = 33) and a 12-week parallel-group design (Study 202; N = 93), JZP-110 was evaluated in narcolepsy patients using changes from baseline in the 40-min MWT as the primary endpoint. Effect sizes based on the change from baseline in mean MWT sleep latency were calculated using 20-min censored MWT data and compared to 40-min MWT data...
July 2017: Sleep Medicine
https://www.readbyqxmd.com/read/28619175/sodium-oxybate-treatment-in-narcolepsy-and-its-effect-on-muscle-tone
#20
Geert Mayer, Andrea Rodenbeck, Karl Kesper
AIMS: To estimate the effect of the compound sodium oxybate (SO) on chin muscle tone in sleep, a re-analysis of the results of the international multicenter study SXB-15 was performed, applying a validated semi-automatic analysis of muscle tone. This analysis distinguishes short (<0.5 s) and long (>0.5 s) muscle activity indices per hour (SMI, LMI) in 116 patients with narcolepsy-cataplexy. While stable stimulant medication was permitted, tricyclics and SSRIs were withdrawn. Polysomnographies were performed at baseline (V5), four weeks after titration of SO to 4...
July 2017: Sleep Medicine
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