Sleep polygraphic study of children and adolescents with narcolepsy/cataplexy. 2009

Raffaele Ferri, and Christian Franceschini, and Marco Zucconi, and Valeria Drago, and Mauro Manconi, and Stefano Vandi, and Francesca Poli, and Oliviero Bruni, and Giuseppe Plazzi
Sleep Research Centre, Department of Neurology I.C., Oasi Institute (IRCCS), Troina, Italy.

The alterations of the Cyclic Alternating Pattern (CAP) recently found in narcoleptic adult patients suggest the presence of an impaired modulation of the fluctuations of the arousal level during their non-rapid eye movement (NREM) sleep, possibly because of the persistence of neurophysiological mechanisms typical of rapid eye movement (REM) sleep. The same mechanism might play a role in the occurrence of leg movement (LM) activity during sleep characterized by low levels of periodicity. The aim of this study was to evaluate CAP and sleep LM activity in a group of children and adolescents with narcolepsy, to interpret the results under a developmental point of view and integrate this new information with data already available for adults. Thirteen young patients with narcolepsy/cataplexy were consecutively recruited for this study, together with 13 age- and sex-matched normal controls. Nocturnal polysomnography was carried out after a night of adaptation in a sleep laboratory room; sleep stages, CAP, and LMs were scored and evaluated following standard criteria. Narcoleptic patients showed shorter sleep onset and REM sleep latency, higher number of stage shifts and awakenings per hour of sleep, and higher percentage of wakefulness after sleep onset; CAP rate was found to be decreased in all NREM sleep stages (in particular CAP A1 subtypes) in narcoleptic patients who also showed significant higher values of all types of LMs (periodic or isolated), during both REM and NREM sleep; however, the most evident differences were found during REM sleep. The results of this study confirm that the sleep microstructure and LM activity changes observed in adulthood are already present and detectable in childhood and might have a role in the already known impaired prefrontal functioning of these subjects. The well-established orexin deficiency might be the unifying factor playing a major role in the modulation of CAP and LMs during sleep in children and adolescents with narcolepsy/cataplexy.

UI MeSH Term Description Entries
D007866 Leg The inferior part of the lower extremity between the KNEE and the ANKLE. Legs
D008297 Male Males
D009068 Movement The act, process, or result of passing from one place or position to another. It differs from LOCOMOTION in that locomotion is restricted to the passing of the whole body from one place to another, while movement encompasses both locomotion but also a change of the position of the whole body or any of its parts. Movement may be used with reference to humans, vertebrate and invertebrate animals, and microorganisms. Differentiate also from MOTOR ACTIVITY, movement associated with behavior. Movements
D009290 Narcolepsy A condition characterized by recurrent episodes of daytime somnolence and lapses in consciousness (microsomnias) that may be associated with automatic behaviors and AMNESIA. CATAPLEXY; SLEEP PARALYSIS, and hypnagogic HALLUCINATIONS frequently accompany narcolepsy. The pathophysiology of this disorder includes sleep-onset rapid eye movement (REM) sleep, which normally follows stage III or IV sleep. (From Neurology 1998 Feb;50(2 Suppl 1):S2-S7) Gelineau Syndrome,Narcolepsy-Cataplexy Syndrome,Paroxysmal Sleep,Gelineau's Syndrome,Narcoleptic Syndrome,Gelineau's Syndromes,Gelineaus Syndrome,Narcolepsy Cataplexy Syndrome,Narcolepsy-Cataplexy Syndromes,Narcoleptic Syndromes,Sleep, Paroxysmal,Syndrome, Gelineau,Syndrome, Gelineau's,Syndrome, Narcolepsy-Cataplexy,Syndrome, Narcoleptic,Syndromes, Gelineau's,Syndromes, Narcolepsy-Cataplexy,Syndromes, Narcoleptic
D002385 Cataplexy A condition characterized by transient weakness or paralysis of somatic musculature triggered by an emotional stimulus or physical exertion. Cataplexy is frequently associated with NARCOLEPSY. During a cataplectic attack, there is a marked reduction in muscle tone similar to the normal physiologic hypotonia that accompanies rapid eye movement sleep (SLEEP, REM). (From Adams et al., Principles of Neurology, 6th ed, p396) Henneberg Syndrome,Status Cataplexicus,Tonelessness Syndrome,Cataleptic Attacks,Attack, Cataleptic,Attacks, Cataleptic,Cataleptic Attack,Syndrome, Henneberg,Syndrome, Tonelessness,Syndromes, Tonelessness,Tonelessness Syndromes
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D004569 Electroencephalography Recording of electric currents developed in the brain by means of electrodes applied to the scalp, to the surface of the brain, or placed within the substance of the brain. EEG,Electroencephalogram,Electroencephalograms
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths

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