HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Clinical and polysomnographic course of childhood narcolepsy with cataplexy.

Abstract
Our aim was to investigate the natural evolution of cataplexy and polysomnographic features in untreated children with narcolepsy with cataplexy. To this end, clinical, polysomnographic, and cataplexy-video assessments were performed at diagnosis (mean age of 10 ± 3 and disease duration of 1 ± 1 years) and after a median follow-up of 3 years from symptom onset (mean age of 12 ± 4 years) in 21 children with narcolepsy with cataplexy and hypocretin 1 deficiency (tested in 19 subjects). Video assessment was also performed in two control groups matched for age and sex at first evaluation and follow-up and was blindly scored for presence of hypotonic (negative) and active movements. Patients' data at diagnosis and at follow-up were contrasted, compared with controls, and related with age and disease duration. At diagnosis children with narcolepsy with cataplexy showed an increase of sleep time during the 24 h; at follow-up sleep time and nocturnal sleep latency shortened, in the absence of other polysomnographic or clinical (including body mass index) changes. Hypotonic phenomena and selected facial movements decreased over time and, tested against disease duration and age, appeared as age-dependent. At onset, childhood narcolepsy with cataplexy is characterized by an abrupt increase of total sleep over the 24 h, generalized hypotonia and motor overactivity. With time, the picture of cataplexy evolves into classic presentation (i.e., brief muscle weakness episodes triggered by emotions), whereas total sleep time across the 24 h decreases, returning to more age-appropriate levels.
AuthorsFabio Pizza, Christian Franceschini, Hanna Peltola, Stefano Vandi, Elena Finotti, Francesca Ingravallo, Lino Nobili, Oliviero Bruni, Ling Lin, Mark J Edwards, Markku Partinen, Yves Dauvilliers, Emmanuel Mignot, Kailash P Bhatia, Giuseppe Plazzi
JournalBrain : a journal of neurology (Brain) Vol. 136 Issue Pt 12 Pg. 3787-95 (Dec 2013) ISSN: 1460-2156 [Electronic] England
PMID24142146 (Publication Type: Journal Article)
Chemical References
  • HCRT protein, human
  • Intracellular Signaling Peptides and Proteins
  • Neuropeptides
  • Orexins
Topics
  • Adolescent
  • Body Mass Index
  • Child
  • Female
  • Hospitalization
  • Humans
  • Intracellular Signaling Peptides and Proteins (deficiency)
  • Longitudinal Studies
  • Male
  • Narcolepsy (diagnosis, metabolism, physiopathology, therapy)
  • Neuropeptides (deficiency)
  • Orexins
  • Polysomnography
  • Retrospective Studies
  • Streptococcal Infections (complications)
  • Video Recording

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: