Nocturnal aspects of narcolepsy with cataplexy

Department of Neurological Sciences, University of Bologna, Bologna, Italy.
Sleep Medicine Reviews (Impact Factor: 8.51). 05/2008; 12(2):109-28. DOI: 10.1016/j.smrv.2007.08.010
Source: PubMed


Even though the most impressive manifestation of narcolepsy is excessive sleepiness, paradoxically a significant number of patients have trouble sleeping at night. A wide array of alterations can affect the night-time sleep of a narcoleptic patient, and the aim of this review is to increase awareness on this issue, thereby enhancing the care of narcoleptic patients by more specific approaches to their disturbed night sleep. This review covers a broad variety of nocturnal sleep features in narcolepsy. Starting from animal models and the clinical features of patients, the paper then discusses the many comorbid conditions found in narcolepsy at night, the most advanced methods of analysis and the few recent advances in the specific treatment of night sleep in narcoleptic patients.

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    • "Although cataplexy often displays a dramatic aspect and represents the pathognomonic symptom of narcolepsy with cataplexy (NC), the most frequent major complaint reported by patients when they first seek for medical advice, and the one that largely impacts on quality of life [1], is hypersomnolence, namely excessive daytime sleepiness (EDS). Other typical symptoms are sleep paralysis and hypnagogic or hypnopompic hallucinations, but a significant number of NC patients also have trouble sleeping at night [2]. Subjective complaints of poor sleep quality in patients with NC [3] include vivid frightening dreams [4], inability to sleep without awakening, getting up to eat at night [5], early awakenings or unrefreshed feeling upon awakening in the morning [6], sleep paralysis , and hallucinations [7]. "
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    ABSTRACT: Objective To analyze the acute effects of sodium oxybate (SO) on polysomnographic night-time (PSG) and multiple sleep latency test (MSLT) of patients with narcolepsy with cataplexy (NC). Methods Sixteen NC adult patients were recruited, together with 16 normal controls. Two consecutive PSG followed by two MSLT sessions were carried out, before and during the first night of SO assumption, respectively. Results The administration of SO was followed by a significant decrease in number of stage shifts and awakenings, wakefulness after sleep onset, percentage of sleep stage 1. Sleep efficiency and slow wave sleep percentage increased. REM latency decreased significantly from 73 to 12 min. Cyclic alternating pattern (CAP) rate remained unchanged but the percentage of CAP A3 subtypes decreased. The number of CAP A3 subtypes per hour of NREM sleep decreased significantly, whereas that of A1 remained unchanged. The duration of A1 and A3 subtypes was slightly increased. Chin muscle tone was not modified by SO as well as periodic leg movements during sleep, but their periodicity index decreased, becoming similar to that of controls. MSLT sleep latency also significantly improved after SO intake. Conclusions The administration of SO in NC patients is followed by immediate important and complex effects on PSG parameters and MSLT, including an evident (over)increase in slow wave sleep, which does not display a physiological microstructure, a moderate decrease in periodic and isolated LMs, possibly mediated by a disinhibited dopaminergic neuronal activity, and an improvement on daytime mean sleep latency at the MSLT.
    Sleep Medicine 09/2014; 15(9). DOI:10.1016/j.sleep.2014.04.020 · 3.15 Impact Factor
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    • "Cataplexy, a symptom characterized by sudden and brief (5–30 s) losses of muscle tone (partial or total) triggered by emotions such as laughter and surprise, is considered pathognomonic of narcolepsy with cataplexy (American Academy of Sleep Medicine, 2005; Vetrugno et al., 2010). Additionally, nocturnal sleep in narcolepsy with cataplexy is not only fragmented by frequent awakenings, but also disturbed by symptoms reflecting abnormal motor control during sleep, such as periodic and non-periodic limb movements, restless legs syndrome and rapid eye movement (REM) sleep behaviour disorder (Ferri et al., 2006, 2008; Dauvilliers et al., 2007b; Mattarozzi et al., 2008; Knudsen et al., 2010; Plazzi et al., 2008a, 2010). A loss of hypocretin-producing neurons (De Lecea et al., 1998; Sakurai et al., 1998) in the postero-lateral hypothalamus (Peyron et al., 2000; Thannickal et al., 2000) is characteristic of narcolepsy with cataplexy. "
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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.
    Brain 09/2011; 134(Pt 12):3480-92. DOI:10.1093/brain/awr244 · 9.20 Impact Factor
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    • "Nocturnal sleep in narcolepsy with cataplexy (NC) is disturbed by multiple awakenings and stage shifts but also by motor and behavioural events, either during rapid eye movement (REM) or non-REM (NREM) sleep (Dauvilliers et al., 2003; Plazzi et al., 2008). Patients with NC also have a high frequency of leg movements during sleep (Dauvilliers et al., 2007; Ferri et al., 2006b) that are significantly less periodic than those of patients with restless legs syndrome (RLS; Ferri et al., 2006a,b). "
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    ABSTRACT: We compared periodic and non-periodic leg movements during sleep and polysomnography in patients with narcolepsy with cataplexy (NC) with or without restless legs syndrome (RLS) with matched idiopathic RLS (iRLS) and control subjects. We enrolled 100 patients with NC: 17 having RLS were compared with 34 sex- and age-matched patients without RLS and with 17 normal controls and 17 iRLS subjects. Periodic leg movements were highest in iRLS and lowest in controls, with those in NC with RLS very close to iRLS, but higher than those in NC without RLS. The periodicity indexes showed the highest value in iRLS followed by NC with or without RLS and, finally, by controls. The inter-leg movement intervals peaked between 10 and 50 s in NC with RLS and in iRLS, the former did not display the nocturnal gradual decrease of periodic leg movements typical of iRLS. Periodic leg movements during sleep and polysomnography displayed specific features in RLS and NC, respectively, with NC with RLS showing an intermediate pattern. Even if RLS is only detected by targeted interview in NC, its frequency and impact on night-time sleep architecture and continuity suggest that this condition should be routinely searched for in NC.
    Journal of Sleep Research 08/2011; 21(2):155-62. DOI:10.1111/j.1365-2869.2011.00942.x · 3.35 Impact Factor
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