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.
    Full-text · Article · Sep 2014 · Sleep Medicine
    • "Narcolepsy, a disease characterized by excessive daytime sleepiness , sleep paralysis, hypnagogic hallucinations, sleep onset rapid eye movement (REM) sleep, and cataplexy, in addition is associated with more specific abnormalities of nocturnal sleep [1]. Sleep latency is typically reduced, sleep architecture is disturbed with increased wake (W) after sleep onset and light sleep (N1), decreased slow-wave sleep (SWS), and frequent sleep stage shifts [2] [3] [4] [5]. Another disturbance of nocturnal sleep found in narcolepsy with cataplexy is the disruption of REM sleep phases [3] [6]. "
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    ABSTRACT: Narcolepsy with cataplexy is a sleep dysregulation disorder with alterations of REM sleep, i.e., sleep onset REM periods and REM sleep instability. Deficient orexin-A (hypocretin-1) signaling is assumed to be a major cause of narcolepsy with cataplexy. In this study we investigated fourteen subjects with narcolepsy with cataplexy in a within-subject, random-order crossover, placebo-controlled design. Patients received double-blinded intranasal orexin-A (435 nmol) or sterile water (placebo) in the morning. Administration was preceded by an adaptation night and followed by a modified maintenance of wakefulness test, attention testing and a second full night of polysomnographic recording. We found comparable sleep behavior during the adaptation nights between both conditions. After orexin-A administration patients had less wake-REM sleep transitions and a decreased REM sleep duration. In the subsequent night, patients showed an increased N2 duration. In the test of divided attention, patients had fewer false reactions after orexin-A administration. Our results support orexin-A to be a REM sleep stabilizing factor and provide functional signs for effects of orexin-A on sleep alterations and attention in narcolepsy with cataplexy.
    No preview · Article · Jan 2014 · Behavioural brain research
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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.
    Full-text · Article · Sep 2011 · Brain
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