Narcolepsy is a lifelong central hypersomnia characterized by excessive daytime sleepiness, cataplex y, sleep paralysis, hypnagogic hallucinations, and disrupted noc- turnal sleep. Behavioral and psychiatric comorbidities are often associated clinical features. It is divided into t wo subt ypes, narcolepsy t ype -1, and narcolepsy t ype -2, depending on the presence of cataplex y and the cerebrospinal fluid hypocretin-1 l evels. An autoimmune process, along with environmental factors, has been hypothesized to cause the disease. Among children and adolescents, incidence in Europe falls bet ween 0.14 and 0.3 in 100,000, with a reported increase in the incidence af ter the 2009 H1N1 pandemic influence and vaccination. Currently, specific pediatric diagnostic criteria and cut-off instrumental values are lacking. Clinical presentation of the disease may differ between children and adults, and misdiagnoses or diagnostic delays are still an issue. The treatment is based on behavioral and pharmacological therapy, but drugs in children a re prescribed off-l abel. Overall, pathogenic mechanism of narcolepsy and data on drug efficac y in chil dren are still li mited: more research is needed to develop new drugs and to reach approval of current treatments in the pediatric population.
Narcolepsy features in young patients / Govi, Annamaria; Antelmi, Elena; Pizza, Fabio; Ingravallo, Francesca; Plazzi, Giuseppe. - In: JOURNAL OF PEDIATRIC BIOCHEMISTRY. - ISSN 1879-5404. - 06:04(2016), pp. 184-190. [10.1055/s-0037-1602258]
Narcolepsy features in young patients
GOVI, ANNAMARIA;PLAZZI, GIUSEPPE
2016
Abstract
Narcolepsy is a lifelong central hypersomnia characterized by excessive daytime sleepiness, cataplex y, sleep paralysis, hypnagogic hallucinations, and disrupted noc- turnal sleep. Behavioral and psychiatric comorbidities are often associated clinical features. It is divided into t wo subt ypes, narcolepsy t ype -1, and narcolepsy t ype -2, depending on the presence of cataplex y and the cerebrospinal fluid hypocretin-1 l evels. An autoimmune process, along with environmental factors, has been hypothesized to cause the disease. Among children and adolescents, incidence in Europe falls bet ween 0.14 and 0.3 in 100,000, with a reported increase in the incidence af ter the 2009 H1N1 pandemic influence and vaccination. Currently, specific pediatric diagnostic criteria and cut-off instrumental values are lacking. Clinical presentation of the disease may differ between children and adults, and misdiagnoses or diagnostic delays are still an issue. The treatment is based on behavioral and pharmacological therapy, but drugs in children a re prescribed off-l abel. Overall, pathogenic mechanism of narcolepsy and data on drug efficac y in chil dren are still li mited: more research is needed to develop new drugs and to reach approval of current treatments in the pediatric population.Pubblicazioni consigliate
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