Furthermore, several new drugs have become available.
Since 2006, there have been considerable developments in our understanding of both the aetiopathology and clinical characteristics in narcolepsy.
A recent paper addressed the limitations of the current International Classification of Sleep Disorders 3 (ISCD-3) diagnostic criteria and made suggestions for future improvements. Criteria for diagnosing narcolepsy into types 1 and 2 (NT1 and NT2) were revised in 2014 by the American Academy of Sleep Medicine. Treatment guidelines for narcolepsy were first published in Europe in 2006 and in the United States in 2007. Current treatments include non-pharmacological and pharmacological approaches. As a result, quality of life measures of both patients and their families are significantly reduced. Narcolepsy potentially affects every aspect of daily life with considerable personal, social and economic consequences. Some recent studies indicate that narcolepsy may be less frequent. Worldwide prevalence estimates suggest that approximately 25–50 persons out of 100,000 are affected. Although the precise aetiology of narcolepsy is unknown, most evidence suggests it is usually a sporadic acquired immune-mediated condition that develops in people who are genetically predisposed. Whereas narcolepsy with typical cataplexy (type 1 narcolepsy, NT1) is considered a distinct entity, associated with hypocretin deficiency, narcolepsy without cataplexy (type 2 narcolepsy, NT2) is less clearly defined and when diagnosed following the current diagnostic criteria is a heterogeneous disorder. Excessive daytime sleepiness (EDS) is usually the most troublesome feature although significantly fragmented and disturbed nighttime sleep (DNS) is common with phenomena including sleep paralysis and hallucinatory experiences around sleep–wake transitions (hypnagogic and hypnopompic hallucinations). Narcolepsy is a disabling hypothalamic disorder that presents with a variety of sleep–wake and other symptoms. INTRODUCTION: BACKGROUND AND SCOPE Clinical need for a guideline The management of narcolepsy involves non-pharmacological and pharmacological approaches with an increasing number of symptomatic treatment options for adults and children that have been studied in some detail. Treatment choices should be tailored to each patient’s symptoms, comorbidities, tolerance and risk of potential drug interactions. The main recommendations can be summarized as follows: (i) excessive daytime sleepiness in adults-scheduled naps, modafinil, pitolisant, sodium oxybate (SXB), solriamfetol (all strong), methylphenidate, amphetamine derivates (both weak) (ii) cataplexy in adults-SXB, venlafaxine, clomipramine (all strong) and pitolisant (weak) (iii) excessive daytime sleepiness in children-scheduled naps, SXB (both strong), modafinil, methylphenidate, pitolisant, amphetamine derivates (all weak) (iv) cataplexy in children-SXB (strong), antidepressants (weak). ResultsĪ total of 10,247 references were evaluated, 308 studies were assessed and 155 finally included. Following a systematic review of the literature (performed in Fall 2018 and updated in July 2020) recommendations were developed according to the GRADE approach. According to the EAN recommendations, 10 relevant clinical questions were formulated in PICO format. The European Academy of Neurology (EAN), European Sleep Research Society (ESRS) and European Narcolepsy Network (EU-NN) nominated a task force of 18 narcolepsy specialists. This paper aims to provide evidence-based guidelines for the management of narcolepsy in both adults and children. We accept referrals from doctors (GPs, paediatricians and child psychiatrists).Narcolepsy is an uncommon hypothalamic disorder of presumed autoimmune origin that usually requires lifelong treatment. NIHR Wellcome Trust Clinical Research FacilityĪs well as offering a clinical service we train health professionals about childhood sleep disorders.University of Southampton: Dr Cathy Hill.We have an active research programme focusing on a number of different sleep disorders as well as treatment trials. narcolepsy type 1 (with cataplexy) and narcolepsy type 2 (without cataplexy).parasomnias, including sleep walking and night terrors.We diagnose and treat a wide range of childhood sleep disorders including: We work with children aged between one and 18 with chronic sleep disorders, who have already been seen by their GP or at another hospital. We're a specialist centre for children's sleep medicine, one of only a few in the UK.
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