Indeed, pharmacological symptom management per se may be insufficient to allow for normal everyday life, so behavioral therapies and interventions remain crucial. Patients also report additional non-sleep related aspects (i.e., decreased sexual activity or memory complaints) that negatively impact on disease perception and quality of life, and patients’ unmet needs could benefit from new multidisciplinary management strategies. Narcolepsy has a severe impact on patients’ quality of life, with impairment in performance (e.g., at school, at work, at home) and psychosocial difficulties. NT1 is also associated with significant medical comorbidities, such as obesity, precocious puberty (in the childhood onset form), and cardiovascular problems, as well as with psychological consequences all to psychiatric diseases. On the other hand, the pathophysiology of NT2 is still unclear, and within this diagnosis, some subjects may be in a prodromic phase of NT1 (with subsequent appearance of cataplexy). Indeed, NT1 is strongly associated with the human leukocyte antigen (HLA) DQB1*0602 allele and other genetic features pointing to a specific configuration of the immune system, and environmental risk factors, such as upper airway infections, may act as triggers leading to the destruction of hypothalamic hypocretin-producing neurons. The etiology of narcolepsy is still unknown, but recent studies have suggested an autoimmune hypothesis. NT1 is considered more frequent than NT2, both in adults and in children, although real prevalence rates are missing and both disorders may be largely underestimated. Narcolepsy, as a rare disease, has a prevalence that varies from geographical regions ranging from 0.02 to 0.06% in Europe and the USA, to 0.16–0.18% in Japan. Narcolepsy onset usually occurs between 10 and 20 years of age, although the diagnosis is mostly established with a substantial delay (mean of 10–15 years). The hypocretin-producing cell destruction contributes to most of the symptoms of narcolepsy, including EDS with sleep onset REM periods (SOREMPs), and other symptoms reflecting exaggerated REM sleep pressure such as cataplexy, sleep paralysis, hypnagogic/hypnopompic hallucinations, and REM sleep behavior disorder (RBD), all disclosing an intrinsic overlap between REM sleep and other states. This neurotransmitter also influences serotonin, histamine, dopamine, acetylcholine, GABA, and glutamate release. Hypocretin-1 contributes to several functions, including circadian rhythm regulation, arousal and appetite control, as well as mood and behavior modulation. Cataplexy, defined as brief episodes of muscle atonia evoked by strong, mainly positive, emotions, is the pathognomonic symptom of NT1, while NT2 does not present with strictly defined cataplexy. Narcolepsy core symptoms are excessive daytime sleepiness (EDS), cataplexy, sleep paralyses, hallucinations, and disrupted nocturnal sleep including frequent parasomnias. NT1 is biologically marked by low cerebrospinal fluid hypocretin-1 levels (CSF hcrt-1), caused by the selective loss of hypothalamic neurons producing hypocretins, while NT2 is associated with normal levels of CSF hcrt-1. Narcolepsy is categorized in narcolepsy type 1 (NT1) and in narcolepsy type 2 (NT2). Narcolepsy is a severe, chronic, and rare disorder, classified by the International Classification of Sleep Disorders Third Edition (ICSD-III) within the central disorders of hypersomnolence (CDH). In this review, we summarize the available therapeutic options for narcolepsy, including the pharmacological, behavioral, and psychosocial interventions. Moreover, overall narcolepsy symptoms management can also benefit from non-pharmacological approaches such as cognitive behavioral therapies (CBTs) and psychosocial interventions to improve the patients’ quality of life in both adult and pediatric-affected individuals as well as the well-being of their families. A definitive cure for narcolepsy is not available to date, and although the research in the field is highly promising, up to now, current treatments have aimed to reduce the symptoms by means of different pharmacological approaches. Thus, the most reliable pathogenic hypothesis is an autoimmune process destroying hypothalamic hypocretin-producing cells. Its etiology is still largely unknown, but studies have reported a strong association between NT1 and HLA, as well as a pathogenic association with the deficiency of cerebrospinal hypocretin-1. Narcolepsy is a rare, chronic, and disabling central nervous system hypersomnia two forms can be recognized: narcolepsy type 1 (NT1) and narcolepsy type 2 (NT2).

Narcolepsy with cataplexy cbt