![]() However, individual differences in sleep requirements make it particularly difficult to assess excessive sleepiness in very young children. The first sign of sleepiness may consist of no more than wanting to sleep longer overnight. The adult picture of sleep attacks against a background of general sleepiness (caused by disturbed overnight sleep) is seen in only the minority of prepubertal patients. ![]() This is the predominant complaint in most cases but it can take various forms. The exact proportion of non-classic presentations is not clear, but a number of ambiguous presentations have been described, which have contributed to diagnostic confusion and delays (sometimes for years), with repeated referrals to different clinical services. However, this clear cut, classic picture seems to be unusual in the early stages of the disorder. 3Ī confident diagnosis of childhood narcolepsy is possible if excessive daytime sleepiness (including sleep attacks) develops suddenly, combined with cataplexy, sleep paralysis, and hypnagogic hallucinations, together with the abnormalities of sleep physiology characteristically seen in adults-rapid onset of sleep and the early appearance of REM sleep. Basic neurochemical mechanisms are not yet well defined. However, the physiopathogenesis of narcolepsy seems to be more complicated than this, with evidence of non-REM (NREM) sleep and possibly circadian sleep wake rhythm abnormalities. In narcolepsy, these aspects become dissociated and intrude into wakefulness. Each of the classic tetrad of symptoms represents a main feature of this form of sleep: sleep attacks (sleep), cataplexy and sleep paralysis (atonia of the skeletal musculature), hypnagogic and hypnopompic hallucinations (dreaming). Narcolepsy has been viewed as primarily a disorder of rapid eye movement (REM) sleep mechanisms. Additional symptoms can include automatic behaviour (complicated behaviour in a sleepy state with impairment of consciousness), poor memory and concentration, and visual disturbances such as blurred vision and diplopia. ![]() In narcolepsy, overnight sleep is generally disrupted, causing some degree of persistent tiredness. These symptoms occur in various combinations and less than half of those with narcolepsy develop all four of them. Estimates for the occurrence of the non-sleepiness (“ancillary”) components of the syndrome are: cataplexy (all cases where cataplexy is required for the diagnosis of narcolepsy however, others accept that in about 20% of cases cataplexy is not present), hypnagogic or hypnopompic hallucinations (50–60%), and sleep paralysis (40%). In its fully developed form the “narcolepsy syndrome” also includes sudden loss of muscle tone in response to strong emotion (cataplexy), vivid dream-like experiences before falling asleep (hypnagogic hallucinations) or on waking (hypnopompic hallucinations), and episodes of inability to move after waking in the morning (sleep paralysis). Narcolepsy is a neurological disorder, the main classic feature of which is excessive sleepiness during the day, with recurrent episodes of irresistible sleep (sleep attacks).
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