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Journal of Pediatric Biochemistry 2016; 06(04): 184-190
DOI: 10.1055/s-0037-1602258
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Narcolepsy Features in Young Patients

Annamaria Govi
1   Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy
,
Elena Antelmi
2   Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy
3   IRCCS, Institute of Neurological Sciences, AUSL Bologna, Italy
,
Fabio Pizza
2   Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy
3   IRCCS, Institute of Neurological Sciences, AUSL Bologna, Italy
,
Francesca Ingravallo
1   Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy
,
Giuseppe Plazzi
2   Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy
3   IRCCS, Institute of Neurological Sciences, AUSL Bologna, Italy
› Author Affiliations
Further Information

Publication History

02 March 2017

03 March 2017

Publication Date:
18 April 2017 (online)

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Abstract

Narcolepsy is a lifelong central hypersomnia characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, hypnagogic hallucinations, and disrupted nocturnal sleep. Behavioral and psychiatric comorbidities are often associated clinical features. It is divided into two subtypes, narcolepsy type-1, and narcolepsy type-2, depending on the presence of cataplexy and the cerebrospinal fluid hypocretin-1 levels. An autoimmune process, along with environmental factors, has been hypothesized to cause the disease. Among children and adolescents, incidence in Europe falls between 0.14 and 0.3 in 100,000, with a reported increase in the incidence after 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 are prescribed off-label. Overall, pathogenic mechanism of narcolepsy and data on drug efficacy in children are still limited: more research is needed to develop new drugs and to reach approval of current treatments in the pediatric population.

Keywords

children - sleep - sleepiness - cataplexy - cataplectic facies
 

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