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Pavla Peřinová, Eva Feketeová, David Kemlink, Petra Kovalská, Karolína Chlebušová, Jiří Nepožitek, Veronika Ibarburu, Eva Králíková, Soňa Nevšímalová, Karel Šonka
Narcolepsy-cataplexy (NC) is a chronic neurological disease with suggested autoimmune etiopathogenesis. Nicotine stimulates central nervous system and smoking increases the risk of autoimmune diseases. Assessment of smoking habits and its correlation to clinical parameters among 87 adult NC patients (38 male, 49 female) included night polysomnography and multiple sleep latency test. In our sample, 43.7% NC patients were regular smokers, and 19.5% former smokers compared to 22.2%, and 12.6%, respectively, in the general population...
2016: Prague Medical Report
Petra Kovalská, David Kemlink, Soňa Nevšímalová, Eszter Maurovich Horvat, Eva Jarolímová, Eva Topinková, Karel Šonka
OBJECTIVE: Narcolepsy with cataplexy (NC) is a chronic disabling disease; however, there are insufficient data on older NC subjects. METHODS: A cross-sectional evaluation on health and social status, including intensity and progression of NC symptoms, was performed on 42 NC patients (age 71.9 years ± 7.5) and 46 age-and-sex-matched controls (age 72.2 years ± 7.0). RESULTS: A greater proportion of patients than controls suffered from hypertension and type 2 diabetes...
August 24, 2016: Sleep Medicine
Régis Lopez, Lucie Barateau, Elisa Evangelista, Sofiene Chenini, Philippe Robert, Isabelle Jaussent, Yves Dauvilliers
STUDY OBJECTIVES: To follow the temporal changes of cerebrospinal fluid biomarker levels in narcoleptic patients with unexpected hypocretin level at referral. METHODS: From 2007 to 2015, 170 HLA DQB1*06:02-positive patients with primary narcolepsy and definite (n=155, 95 males, 36 children) or atypical cataplexy (n=15, 4 males, 3 children) were referred to our center. Cerebrospinal hypocretin deficiency was found in 95.5% and 20% of patients with definitive and atypical cataplexy, respectively...
September 9, 2016: Sleep
Raphaël Bernard-Valnet, Lidia Yshii, Clémence Quériault, Xuan-Hung Nguyen, Sébastien Arthaud, Magda Rodrigues, Astrid Canivet, Anne-Laure Morel, Arthur Matthys, Jan Bauer, Béatrice Pignolet, Yves Dauvilliers, Christelle Peyron, Roland S Liblau
Narcolepsy with cataplexy is a rare and severe sleep disorder caused by the destruction of orexinergic neurons in the lateral hypothalamus. The genetic and environmental factors associated with narcolepsy, together with serologic data, collectively point to an autoimmune origin. The current animal models of narcolepsy, based on either disruption of the orexinergic neurotransmission or neurons, do not allow study of the potential autoimmune etiology. Here, we sought to generate a mouse model that allows deciphering of the immune mechanisms leading to orexin(+) neuron loss and narcolepsy development...
September 27, 2016: Proceedings of the National Academy of Sciences of the United States of America
Michel Maitre, Christian Klein, Ayikoe G Mensah-Nyagan
Gamma-hydroxybutyrate (GHB or Xyrem(R)) is frequently used in humans for several clinical indications, including anesthesia, narcolepsy/cataplexy, and alcohol-withdrawal symptoms. Pharmacological effects induced in the brain by therapeutic doses of Xyrem(R) are generally GABAergic-dependent. These effects allow sedation, stress/anxiety reduction, deep sleep induction, decrease of neuroinflammation, and neuroprotection. Furthermore, Xyrem(R) promotes the expression of pivotal genes reducing toxic proteinopathies, as demonstrated in laboratory animal models...
2016: Alzheimer's Research & Therapy
Kartini Gadroen, Sabine M J M Straus, Alexandra Pacurariu, Daniel Weibel, Xavier Kurz, Miriam C J M Sturkenboom
OBJECTIVE: This study aims to describe the frequency and quality of spontaneous narcolepsy case reports following administration of pandemic influenza vaccine as captured in the Eudravigilance database. METHODS: We conducted a retrospective descriptive study of spontaneous Individual Case Safety Reports (ICSRs), reporting narcolepsy following administration of pandemic influenza vaccine as received by Eudravigilance until July 2014. De-duplication was carried out by Eudravigilance...
September 22, 2016: Vaccine
Christopher A Kosky, Anastasios Bonakis, Arthee Yogendran, Gihan Hettiarachchi, Paul I Dargan, Adrian J Williams
STUDY OBJECTIVES: Drugs and psychoactive substances can cause sleepiness and when undetected, may lead to over diagnosis of central hypersomnias. We performed urine drug testing using gas chromatography-mass spectrometry in adults undergoing multiple sleep latency testing (MSLT) for a suspected central hypersomnia. We examined how the drug test results modified the treating physician's diagnosis. METHODS: One hundred eighty-six consecutive patients with a suspected central hypersomnia who underwent clinical assessment, MSLT and urine drug testing by gas chromatography-mass spectrometry were retrospectively studied...
August 22, 2016: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
Ayşe Kacar Bayram, Hüseyin Per, Sevda Ismailoğullari, Mehmet Canpolat, Hakan Gumus, Murat Aksu
Objective Narcolepsy is a chronic sleep disorder characterized by excessive daytime sleepiness, cataplexy, hypnagogic and/or hypnopompic hallucinations, and sleep paralysis. It is one of the most important causes of excessive daytime sleepiness in the pediatric population. The aim of this study is to present the clinical and laboratory findings, and treatment results of pediatric patients with narcolepsy. Materials and Methods We studied five unrelated consecutive children with narcolepsy, focusing on clinical and laboratory features, the therapy and outcome over the 33-month follow-up period...
August 26, 2016: Neuropediatrics
Tomi Sarkanen, Reija Alén, Markku Partinen
INTRODUCTION: Narcolepsy type 1 is an organic sleep disorder caused by the destruction of hypocretin producing neurons in hypothalamus. In addition to daytime sleepiness, the spectrum and severity of symptoms are very variable. Psychiatric comorbidity and phenomena resembling psychotic symptoms are also common. Current treatment options for narcolepsy are symptomatic but there are few case reports of positive effect of immunotherapy. We report a very severely affected young boy treated with rituximab (RXB)...
September 2016: Neurologist
Richard E Rosch, Michael Farquhar, Paul Gringras, Deb K Pal
Narcolepsy with cataplexy is a rare, but important differential diagnosis for daytime sleepiness and atonic paroxysms in an adolescent. A recent increase in incidence in the pediatric age group probably linked to the use of the Pandemrix influenza vaccine in 2009, has increased awareness that different environmental factors can "trigger" narcolepsy with cataplexy in a genetically susceptible population. Here, we describe the case of a 13-year-old boy with narcolepsy following yellow fever vaccination. He carries the HLA DQB1*0602 haplotype strongly associated with narcolepsy and cataplexy...
2016: Frontiers in Neurology
Lucie Barateau, Régis Lopez, Yves Dauvilliers
Narcolepsy type 1 (NT1) and type 2 (NT2) are two rare neurological diseases, classified as central disorders of hypersomnolence. The pathophysiology of NT1 is well known; it is caused by the selective destruction of hypocretin (Hcrt) neurons, by a highly suspected autoimmune process. On the contrary, little is known about NT2 etiology, sharing with NT1 somnolence and signs of dysregulation of rapid eye movement (REM) sleep, but not cataplexy. Management strategies are rather codified, at least in adults, with a lifelong treatment required in NT1, whereas no pharmacological study focused only on NT2 patients, with sometimes spontaneous improvement or disappearance of their symptoms...
October 2016: Current Treatment Options in Neurology
Joseph Andrew Berkowski, Anita Valanju Shelgikar
Central disorders of hypersomnolence are rare conditions with a poorly understood pathophysiology, making the identification and management challenging for sleep clinicians. Clinical history is essential for ruling out secondary causes of hypersomnolence and distinguishing among diagnoses. Current diagnostic criteria rely heavily on the polysomnogram and multiple sleep latency test. The current focus of treatment of hypersomnolence is on drugs that promote alertness. Additionally, in the case of narcolepsy type 1, medication management addresses control of cataplexy, the hallmark symptom of this disorder...
September 2016: Sleep Medicine Clinics
Yves Dauvilliers, Régis Lopez
Central hypersomnias include narcolepsy type 1, type 2 and idiopathic hypersomnia with daytime sleepiness excessive in the foreground of the clinical symptoms. Despite major advances in our understanding of the mechanisms of the narcolepsy type 1 with a low level of hypocretin-1 in cerebrospinal fluid, its current management is only symptomatic. The current management is also only symptomatic for type 2 narcolepsy and idiopathic hypersomnia with an unknown pathophysiology. Treatment options may vary from a single drug targeting several symptoms or several drugs treating a specific symptom...
June 2016: La Revue du Praticien
Yves Dauvilliers, Régis Lopez
Narcolepsy with cataplexy or narcolepsy type 1 in a rare, disabling sleep disorder, with a prevalence of 20 to 30 per 100,000. Its onset peaks in the second decade. The main features are excessive daytime sleepiness and cataplexy or sudden less of muscle tone triggered by emotional situations. Other less consistent symptoms include hypnagogic hallucinations, sleep paralysis, disturbed nighttime sleep, and weight gain. Narcolepsy with cataplexy remains a clinical diagnosis but nighttime and daytime polysomnography (multiple sleep latency tests) are useful to document mean sleep latency below 8 min and at least two sleep-onset REM periods...
June 2016: La Revue du Praticien
Marek Susta, Veronika Nemcova, Gustav Bizik, Karel Sonka
Reported brain abnormalities in anatomy and function in patients with narcolepsy with cataplexy led to a project based on qualitative electroencephalography examination and analysis in an attempt to find a narcolepsy with cataplexy-specific brain-derived pattern, or a sequence of brain locations involved in processing humorous stimuli. Laughter is the trigger of cataplexy in these patients, and the difference between patients and healthy controls during the laughter should therefore be notable. Twenty-six adult patients (14 male, 12 female) suffering from narcolepsy with cataplexy and 10 healthy controls (five male, five female) were examined...
August 15, 2016: Journal of Sleep Research
Blanka Klimova, Petra Maresova, Michal Novotny, Kamil Kuca
BACKGROUND: Narcolepsy is an incurable neurological disorder when the brain is not able to regulate a sleep and wakefulness cycle correctly. The affected person suddenly falls asleep during the day or he/she suffers from excessive day sleepiness. In addition, people may also suffer from cataplexy, hypnagogic hallucinations, sleep paralysis, and disturbed nighttime sleep. OBJECTIVE: The purpose of this review study is to provide the latest information on both clinical and socioeconomic issues in the field of narcolepsy treatment and emphasize its benefits and limitations...
July 31, 2016: Mini Reviews in Medicinal Chemistry
Yohei Sagawa, Masatoshi Sato, Noriaki Sakai, Sachiko Chikahisa, Shintaro Chiba, Takashi Maruyama, Junki Yamamoto, Seiji Nishino
Prostaglandin (PG)D2 is an endogenous sleep substance, and a series of animal studies reported that PGD2 or PGD2 receptor (DP1) agonists promote sleep, while DP1 antagonists promote wakefulness. This suggests the possibility of use of PG DP1 antagonists as wake-promoting compounds. We therefore evaluated the wake-promoting effects of ONO-4127Na, a DP1 antagonist, in a mouse model of narcolepsy (i.e., orexin/ataxin-3 transgenic mice) and compared those to effects of modafinil. ONO-4127Na perfused in the basal forebrain (BF) area potently promoted wakefulness in both wild type and narcoleptic mice, and the wake-promoting effects of ONO-4127Na at 2...
November 2016: Neuropharmacology
Mercedes Pineda, Eugen Mengel, Helena Jahnová, Bénédicte Héron, Jackie Imrie, Charles M Lourenço, Vanessa van der Linden, Parvaneh Karimzadeh, Vassili Valayannopoulos, Pavel Jesina, Juan V Torres, Stefan A Kolb
BACKGROUND: Niemann-Pick disease Type C (NP-C) is difficult to diagnose due to heterogeneous and nonspecific clinical presentation. The NP-C Suspicion Index (SI) was developed to identify patients with a high likelihood of NP-C; however, it was less reliable in patients aged <4 years. METHODS: An early-onset NP-C SI was constructed following retrospective chart review of symptom presentation in 200 patients from nine centres comprised of 106 NP-C cases, 31 non-cases and 63 controls...
2016: BMC Pediatrics
Yahiya Y Syed
Pitolisant (Wakix™) is an inverse agonist of the histamine H3 receptor that is being developed by Bioproject. Oral pitolisant is approved in the EU for the treatment of narcolepsy with or without cataplexy in adults. Pitolisant has received a Temporary Authorization of Use in France for this indication in case of treatment failure, intolerance or contraindication to currently available treatment. Pitolisant has orphan drug designation in the EU and the USA. In the pivotal HARMONY I trial, pitolisant significantly decreased excessive daytime sleepiness versus placebo in adults with narcolepsy with or without cataplexy (primary endpoint)...
September 2016: Drugs
Hildegard Hidalgo, Ulf Kallweit, Johannes Mathis, Claudio L Bassetti
Narcolepsy with cataplexy (NC) is a chronic neurological disorder thought to result from an altered immune response based on a genetic predisposition coupled with environmental factors. We here present four cases of NC following a tick-borne encephalitis (TBE) vaccination with FSME Immun. Our findings implicate TBE vaccination as a potential additional environmental factor for the development of NC and add additional evidence for an immunological mechanism in the pathogenesis of the disease.
July 1, 2016: Sleep
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