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Complex movement disorders at disease onset in childhood narcolepsy with cataplexy.

Plazzi, G; Pizza, F; Palaia, V; Franceschini, C; Poli, F; Moghadam, KK; Cortelli, P; Nobili, L; Bruni, O; Dauvilliers, Y; et al. Plazzi, G; Pizza, F; Palaia, V; Franceschini, C; Poli, F; Moghadam, KK; Cortelli, P; Nobili, L; Bruni, O; Dauvilliers, Y; Lin, L; Edwards, MJ; Mignot, E; Bhatia, KP (2011) Complex movement disorders at disease onset in childhood narcolepsy with cataplexy. Brain, 134 (12). pp. 3477-3489. ISSN 1460-2156 https://doi.org/10.1093/brain/awr244
SGUL Authors: Edwards, Mark John James

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Abstract

Narcolepsy with cataplexy is characterized by daytime sleepiness, cataplexy (sudden loss of bilateral muscle tone triggered by emotions), sleep paralysis, hypnagogic hallucinations and disturbed nocturnal sleep. Narcolepsy with cataplexy is most often associated with human leucocyte antigen-DQB1*0602 and is caused by the loss of hypocretin-producing neurons in the hypothalamus of likely autoimmune aetiology. Noting that children with narcolepsy often display complex abnormal motor behaviours close to disease onset that do not meet the classical definition of cataplexy, we systematically analysed motor features in 39 children with narcolepsy with cataplexy in comparison with 25 age- and sex-matched healthy controls. We found that patients with narcolepsy with cataplexy displayed a complex array of 'negative' (hypotonia) and 'active' (ranging from perioral movements to dyskinetic-dystonic movements or stereotypies) motor disturbances. 'Active' and 'negative' motor scores correlated positively with the presence of hypotonic features at neurological examination and negatively with disease duration, whereas 'negative' motor scores also correlated negatively with age at disease onset. These observations suggest that paediatric narcolepsy with cataplexy often co-occurs with a complex movement disorder at disease onset, a phenomenon that may vanish later in the course of the disease. Further studies are warranted to assess clinical course and whether the associated movement disorder is also caused by hypocretin deficiency or by additional neurochemical abnormalities.

Item Type: Article
Additional Information: © The Author (2011). Published by Oxford University Press on behalf of the Guarantors of Brain. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Keywords: Adolescent, Child, Child, Preschool, Female, Humans, Male, Movement Disorders, Narcolepsy, Prospective Studies, Humans, Movement Disorders, Narcolepsy, Prospective Studies, Adolescent, Child, Child, Preschool, Female, Male, hypotonia, movement disorder, narcolepsy with cataplexy, streptococcal infection, chorea, Neurology & Neurosurgery, 11 Medical And Health Sciences, 17 Psychology And Cognitive Sciences
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) > Neuroscience (INCCNS)
Journal or Publication Title: Brain
ISSN: 1460-2156
Language: eng
Dates:
DateEvent
December 2011Published
19 September 2011Published Online
14 July 2011Accepted
Publisher License: Creative Commons: Attribution-Noncommercial 3.0
Projects:
Project IDFunderFunder ID
2007122nEUroped (PHEA)UNSPECIFIED
PubMed ID: 21930661
Web of Science ID: WOS:000298189800007
Go to PubMed abstract
URI: http://openaccess.sgul.ac.uk/id/eprint/109506
Publisher's version: https://doi.org/10.1093/brain/awr244

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