Though little is known about the underlying neuropathology, narcolepsy without cataplexy may simply be caused by less severe injury to the orexin neurons (Thannickal et al., 2009), resulting in mainly sleepiness and a small reduction in CSF orexin level (Mignot et al., 2002; Andlauer et al., 2012). The orexin neurons provide direct, excitatory inputs to the cortex, thalamus, and spinal cord. Naar navigatie springen Naar zoeken springen. Quelles sont ses causes ? CATAPLEXY, a symptom of narcolepsy, is an illuminating example of paralysis of voluntary movement induced by emotion. The goals of this review are to describe briefly the symptoms, etiology, and management of narcolepsy, and then review the underlying neurobiology and important directions for future research. Clearly, much more needs to be done to determine whether an autoimmune process kills the orexin neurons, and if so, to discover how that process can be altered. DOI: https://doi.org/10.1523/JNEUROSCI.2630-12.2012. Les personnes qui ont de telles attaques se produire, sont dans un état de veille et de comprendre ce qui se passe. La catalepsie pourrait être … Copyright © 2020 by the Society for Neuroscience.JNeurosci   Print ISSN: 0270-6474   Online ISSN: 1529-2401. Confinement : les annulations chez les médecins en hausse de 30%. Les manifestations de la catalepsie sont ainsi : Face à ses signes, à une époque où la médecine ne disposait pas d'outils diagnostics perfectionnés, des patients en état de catalepsie ont pu être considérés à tort comme décédés. Prolonged periods of wakefulness increase homeostatic sleep drive, and it is possible that people with narcolepsy have higher sleep drive than normal. No eLetters have been published for this article. Monoamine neurotransmitters, especially dopamine, promote arousal, while some such as norepinephrine and serotonin suppress cataplexy. This would be a great accomplishment as narcolepsy is remarkably simple when compared with other neurological disorders: sleepiness, cataplexy, and the other symptoms are likely all due to a loss of orexin signaling. Sodium oxybate, the sodium salt gamma hydroxybutyrate, is also quite effective for treating sleepiness and cataplexy (Boscolo-Berto et al., 2011).Sodium oxybate is given at bedtime and promotes deep non-REM sleep, probably through activation of GABA B receptors (Vienne et al., 2010).After several weeks, sleepiness and cataplexy often improve, but the mechanisms underlying this slow … La paralysie du sommeil peut être intimidant, mais il est dangereux. Masashi Yanagisawa's group produced an orexin ligand knock-out mouse with sleepiness and cataplexy strikingly similar to human narcolepsy (Chemelli et al., 1999). They are presented in the hope of helping researchers identify clinical implications of their research. Enter multiple addresses on separate lines or separate them with commas. Researchers have now studied several engineered and naturally occurring animal models of narcolepsy (Chen et al., 2009; Scammell et al., 2009). These studies focused on patients that have narcolepsy with cataplexy, yet much less is understood about the neuropathology of narcolepsy without cataplexy. He went on to speculate that the sleepiness of narcolepsy might be caused by injury to this region, but for decades this hypothesis could not be tested as so little was understood about the cells and functions of the hypothalamus. La maladie a un impact significatif sur la vie humaine et même le rend invalide. L'un des 2.000 personnes souffre de narcolepsie. This review will focus on the primary symptoms of narcolepsy, sleepiness and cataplexy, though numerous reviews discuss other roles for the orexin system (Aston-Jones et al., 2008; Mieda and Sakurai, 2009; Sakurai and Mieda, 2011; Sinton, 2011; Nixon et al., 2012). The orexin neurons are active during wake and they help maintain normal muscle tone by exciting neurons in the vlPAG/LPT, monoamine neurons, and motor neurons. Positive emotions may activate neurons in the amygdala that excite the SLD and inhibit the vlPAG/LPT. Beth Israel Deaconess Medical Center, Center for Life Science, Room 705, 330 Brookline Avenue, Boston, MA 02215. Dans d'autres cas, la rigidité du corps est telle qu'aucune mobilisation n'est possible. Ces derniers sont souvent causés par des émotions intenses telles que la colère ou la surprise ou par le rire. Confinement : les médecins ne ferment pas les portes de leur cabinet ! Cela allait de 14 hospitalisations pour 1 patient, 9 pour 1 autre, 6 pour 2 autres et 1 à 3 pour les 7 autres. doi:10.1001/archneurpsyc.1953.02320260082006. The most striking occurrence is on laughter. The endogenous opiate dynorphin and NARP (a protein involved in glutamate signaling) are also produced by the orexin neurons, and both of these markers are absent in the lateral hypothalamus of patients with narcolepsy (Blouin et al., 2005; Crocker et al., 2005). A, Several pathways suppress atonia during normal wakefulness. Cataplexie peut être légère, manifestant sensation momentanée de faiblesse dans les genoux, ou peut provoquer un effondrement complet de la physique, conduisant à une chute. The definitive link between narcolepsy and orexin followed soon after when researchers demonstrated a lack of orexin peptides in the hypothalami and CSF of narcolepsy patients (Peyron et al., 2000; Thannickal et al., 2000; Mignot et al., 2002). La cataplexie est généralement déclenchée par une fortedes émotions telles que le rire, la colère ou la surprise. It includes a feeling of sleepiness waxing and waning throughout the day and episodes of irresistible sleep recurring daily or … After several weeks, sleepiness and cataplexy often improve, but the mechanisms underlying this slow improvement are unknown. Just like in people with narcolepsy, emotional stimuli seem to trigger cataplexy in animal models: narcoleptic dogs have marked increases in cataplexy when playing or presented with highly palatable food (Baker et al., 1982; Siegel et al., 1989) and narcoleptic mice have increased cataplexy with social interaction, palatable food, or running wheels (España et al., 2007; Clark et al., 2009; Scammell et al., 2009). La catalepsie peut être d'ordre psychiatrique notamment dans le cadre de maladies comme la schizophrénie ou l'hystérie. sign up for alerts, and more, to access your subscriptions, sign up for alerts, and more, to download free article PDFs, sign up for alerts, customize your interests, and more, to make a comment, download free article PDFs, sign up for alerts and more, Archives of Neurology & Psychiatry (1919-1959), Subscribe to the Archives of Neurology & Psychiatry journal, FDA Approval and Regulation of Pharmaceuticals, 1983-2018, Global Burden of Skin Diseases, 1990-2017, Health Care Spending in the US and Other High-Income Countries, Life Expectancy and Mortality Rates in the United States, 1959-2017, Medical Marketing in the United States, 1997-2016, Practices to Foster Physician Presence and Connection With Patients in the Clinical Encounter, US Burden of Cardiovascular Disease, 1990-2016, Waste in the US Health Care System: Estimated Costs and Potential for Savings. The SLD then excites neurons in the medial medulla and spinal cord that strongly hyperpolarize motor neurons, resulting in cataplexy. Narcolepsy is linked to polymorphisms in the T-cell receptor α gene that may alter immune responses to some antigens (Hallmayer et al., 2009). Comportement automatique peut être un symptôme de la narcolepsie et constitue une menace si une personne effectue des actions potentiellement dangereuses, telles que la conduite ou la cuisson. Toute reproduction ou représentation totale ou partielle de ce site par quelque procédé que ce soit, sans autorisation expresse, est interdite. Les symptômes de la narcolepsie peuvent apparaître tout à la fois ou de développer progressivement sur plusieurs années. – …Et puis ça fait toujours bien de connaître de jolis mots savants ! In addition, antibodies against tribbles homolog 2, a protein found in many cell types including the orexin neurons, are sometimes elevated in narcolepsy (Cvetkovic-Lopes et al., 2010; Kawashima et al., 2010; Toyoda et al., 2010). Coronavirus : les courbes sont elles vraiment en baisse ? Cataplexie La cataplexie est un symptôme de la narcolepsie qui consiste en de brefs et soudains épisodes de perte du tonus musculaire. to download free article PDFs, CATAPLEXY, a symptom of narcolepsy, is an illuminating example of paralysis of voluntary movement induced by emotion. Thus, H3 inverse-agonists increase activity in monoamine neurons and reduce sleepiness in people, dogs, and mice with narcolepsy (Guo et al., 2009; Inocente et al., 2012). Stimulants (e.g., dextroamphetamine, methylphenidate) improve sleepiness by enhancing release and decreasing reuptake of dopamine and other monoamine neurotransmitters (Kuczenski and Segal, 1997; Nishino et al., 1998; Kanbayashi et al., 2000; Wisor et al., 2001; Leonard et al., 2004). 1953;69(2):224–235. Atonia pathways triggering cataplexy. The clinical features of cataplexy: a questionnaire study in narcolepsy patients with and without hypocretin-1 deficiency, The brain H3-receptor as a novel therapeutic target for vigilance and sleep–wake disorders, Increased incidence and clinical picture of childhood narcolepsy following the 2009 H1N1 pandemic vaccination campaign in Finland, Excitatory effects of hypocretin-1 (orexin-A) in the trigeminal motor nucleus are reversed by NMDA antagonism, Neurons containing hypocretin (orexin) project to multiple neuronal systems, A mutation in a case of early onset narcolepsy and a generalized absence of hypocretin peptides in human narcoleptic brains, Narcolepsy-cataplexy associated with precocious puberty, Connectomics of orexin-producing neurons: interface of systems of emotion, energy homeostasis and arousal, Orexins and orexin receptors: a family of hypothalamic neuropeptides and G protein-coupled receptors that regulate feeding behavior, Comparison of clinical characteristics among narcolepsy with and without cataplexy and idiopathic hypersomnia without long sleep time, focusing on HLA-DRB1(*)1501/DQB1(*)0602 finding, Pharmacogenetic modulation of orexin neurons alters sleep/wakefulness states in mice, Modafinil: a novel stimulant for the treatment of narcolepsy, Hypothalamic arousal regions are activated during modafinil-induced wakefulness, A consensus definition of cataplexy in mouse models of narcolepsy, Fluvoxamine and clomipramine in the treatment of cataplexy, English translations of the first clinical reports on narcolepsy and cataplexy by Westphal and Gelineau in the late 19th century, with commentary, Comparative effects of modafinil and amphetamine on daytime sleepiness and cataplexy of narcoleptic dogs, Heart rate and blood pressure changes during sleep-waking cycles and cataplexy in narcoleptic dogs, Orexin/hypocretin plays a role in the response to physiological disequilibrium, Orexin/hypocretin 1 receptor antagonist reduces heroin self-administration and cue-induced heroin seeking, Effect of inhibitory amino acid antagonists on IPSPs induced in lumbar motoneurons upon stimulation of the nucleus reticularis gigantocellularis during active sleep, Obesity accompanies narcolepsy with cataplexy but not narcolepsy without cataplexy, The clinical spectrum of narcolepsy with cataplexy: a reappraisal, Sleep deprivation in narcoleptic subjects: effect on sleep stages and EEG power density, Reduced number of hypocretin neurons in human narcolepsy, Hypocretin (orexin) cell loss in Parkinson's disease, Localized loss of hypocretin (orexin) cells in narcolepsy without cataplexy, Anti-Tribbles homolog 2 autoantibodies in Japanese patients with narcolepsy, Acute optogenetic silencing of orexin/hypocretin neurons induces slow-wave sleep in mice, Differential effects of GABAB receptor subtypes, {gamma}-hydroxybutyric acid, and baclofen on EEG activity and sleep regulation, Eigentümliche mit Einschlafen verbundene Anfälle, Distinct narcolepsy syndromes in orexin receptor-2 and orexin null mice: molecular genetic dissection of non-REM and REM sleep regulatory processes, Modafinil more effectively induces wakefulness in orexin-null mice than in wild-type littermates, Dopaminergic-adrenergic interactions in the wake promoting mechanism of modafinil, Dopaminergic role in stimulant-induced wakefulness, Locus coeruleus neurons: cessation of activity during cataplexy, Activity of dorsal raphe cells across the sleep-waking cycle and during cataplexy in narcoleptic dogs, Excitatory projections from the amygdala to neurons in the nucleus pontis oralis in the rat: an intracellular study, Hypocretinergic control of spinal cord motoneurons, Disease Mechanisms and Therapeutic Approaches in Spinal Muscular Atrophy, Migraine: Multiple Processes, Complex Pathophysiology, Advances in the Diagnosis and Treatment of Vestibular Disorders: Psychophysics and Prosthetics, Visit Society for Neuroscience on Facebook, Follow Society for Neuroscience on Twitter, Follow Society for Neuroscience on LinkedIn, Visit Society for Neuroscience on Youtube, http://www.jneurosci.org/misc/ifa_minireviews.dtl. In 1998, two labs independently discovered a pair of hypothalamic neuropeptides termed orexin-A and -B (or hypocretin 1 and 2) and their receptors (OX1 and OX2) (de Lecea et al., 1998; Sakurai et al., 1998). Can we better understand the mechanisms of narcolepsy and the drugs used in its treatment to develop better and safer therapies? Bien que la cause exacte est inconnue, la narcolepsie,apparemment est une maladie du cerveau qui contrôle le processus de sommeil et l'éveil. Background. Since narcolepsy results from selective loss of the orexin neurons, restoration of orexin signaling should be a highly effective and targeted treatment. In addition, the orexin peptides may prevent atonia by directly exciting neurons at multiple levels of this system, including those in the vlPAG/LPT, monoaminergic regions, and motor neurons (Horvath et al., 1999; Peever et al., 2003; Yamuy et al., 2004; Lu et al., 2006). Orexin neurons project throughout the brain to promote and maintain wakefulness. Hospitalisations en psychiatrie. En savoir plus sur les causes et les manifestations de la narcolepsie lire l'article. However, although sodium oxybate immediately improves sleep, the improvements in daytime alertness may not become apparent until weeks later (Boscolo-Berto et al., 2011). In fully developed narcolepsy there are sleep attacks and paralytic attacks. Modafinil has some similarities with traditional stimulants and may be a more selective dopamine reuptake blocker (Scammell et al., 2000; Wisor and Eriksson, 2005; Golicki et al., 2010). La cataplexie est un symptôme de la narcolepsie qui consiste en de brefs et soudains épisodes de perte du tonus musculaire. However, this seems unlikely as the total amounts of sleep in people and in mice with narcolepsy are essentially normal, as are their responses to sleep deprivation (Tafti et al., 1992; Besset et al., 1994; Mochizuki et al., 2004). Medications that reduce sleepiness and cataplexy in narcolepsy. Pour quelles raisons survient-il et comment y remédier ? Pathologies psychiatriques, comme la schizophrénie ou l'hystérie. In all the domain of psychosomatic medicine cataplexy is almost without a peer as a compelling illustration of how emotion can give rise, immediately and dramatically, to a physical symptom. La cataplexie est un des symptômes majeurs de la narcolepsie : c'est une perte brusque du tonus musculaire sans altération de la conscience et survenant à un moment quelconque de la journée. Clinical trials are now underway to establish the efficacy and safety of these drugs in treating sleepiness and cataplexy. Les personnes atteintes de narcolepsie rapportent souventles symptômes suivants: vision double, incapacité à se concentrer, la perte et des maux de tête mémoire. Injection of orexin-A into the lateral ventricles of narcoleptic mice improves wakefulness and reduces cataplexy (Mieda et al., 2004), and both intravenous and nasal delivery of orexin-A to nonhuman primates alleviate performance deficits after sleep deprivation (Deadwyler et al., 2007). Des études récentes chez des patients atteints de narcolepsie a été trouvé pour réduire le niveau d'un ingrédient chimique du cerveau - hypocrétine. Normally, the many effects of the orexin system and a continued monoaminergic drive to the pons and directly to motor neurons would counter this triggering of atonia, but in the absence of orexins, these excitatory drives are lost and cataplexy occurs. Narcolepsie - troubles du sommeil, qui se manifestesomnolence constante et une tendance à dormir à des moments inappropriés. Cystiphane, la gamme force et vitalité des cheveux qui va au cœur de la fibre. Ce document intitulé « Catalepsie - Définition » issu de Journal des Femmes (sante-medecine.journaldesfemmes.fr) est soumis au droit d'auteur. Atonia is driven by neurons in the SLD that activate neurons in the spinal cord and medial medulla (MM) that inhibit motor neurons using GABA and glycine. As in this young woman, all individuals with narcolepsy experience persistent daytime sleepiness. Ce symptôme peut être observé dans différents contextes, associés ou non à une maladie. For over 100 years, clinicians have recognized narcolepsy (Westphal, 1877; Gelineau, 1880; Schenck et al., 2007), but only in the last decade have neuroscientists been able to shed light on its true cause and underlying neurobiology. Mild to moderate loss of the orexin neurons has also been demonstrated in Parkinson's disease (Fronczek et al., 2007, 2009; Thannickal et al., 2007) and traumatic brain injury (Baumann et al., 2009), disorders that often produce sleepiness but no cataplexy. The loss of muscle tone can be partial, affecting just the face and neck, or complete, resulting in full postural collapse. SfN does not assume any responsibility for any injury and/or damage to persons or property arising from or related to any use of any material contained in JNeurosci. On laughing heartily, the patient will collapse in a heap, conscious but unable to move or speak, and will lie helpless for some, LEVIN M. AGGRESSION, GUILT AND CATAPLEXY. Cependant, chez les patients souffrant de narcolepsie, ces phénomènes (perte de tonus musculaire et un sentiment de rêves) se produisent au mauvais moment. Dépression souriante : quand le mal-être est masqué, Cancer : un mois de retard de traitement suffit à augmenter le risque de mortalité, Bouillottes : certaines peuvent être “dangereuses”, Coronavirus : le discret succès de la Finlande face au Covid-19, Covid-19 : il serait possible de guérir rapidement tout en étant immunisé, Confinement : le télétravail aggrave les addictions des Français. During REM sleep, motor neurons are strongly inhibited by GABAergic and glycinergic neurons in the spinal cord and medial medulla (Soja et al., 1987; Kodama et al., 2003; Brooks and Peever, 2008). Mice lacking orexins have a decreased tendency for addiction (DiLeone et al., 2003; Smith and Aston-Jones, 2012), but whether this occurs in people with narcolepsy is not yet clear (Dimitrova et al., 2011). No matter how much she slept at night, she struggled to stay awake while studying and her grades began to slip. Thank you for sharing this Journal of Neuroscience article. HLA DQB1*0602 is found in ∼90% of patients with narcolepsy, and simply carrying this gene increases the risk of narcolepsy ∼200-fold (Mignot et al., 1993, 1994). Thus, a man may be stricken with paralysis at the moment he raises his hand in anger against his child. Les adultes ne peuvent pas être en mesure d'effectuer des tâches quotidiennes au travail. The ideas and opinions expressed in JNeurosci do not necessarily reflect those of SfN or the JNeurosci Editorial Board. As an alternative, orexin gene therapy has been used to induce expression of orexin peptides in a variety of neurons in or near the hypothalamus in mice and has resulted in reductions in both sleepiness and cataplexy (Liu et al., 2008, 2011). An episode of cataplexy usually lasts from a few seconds up to 1 or 2 min, and during this time consciousness is fully preserved. Elle peut également être neurologique due entre autres à une tumeur cérébrale ou à un syndrome parkinsonien. The orexins have since been demonstrated to play essential roles in maintaining wakefulness and regulating transitions between sleep and wake (Chemelli et al., 1999; Mochizuki et al., 2004; Adamantidis et al., 2007; Diniz Behn et al., 2010; Sasaki et al., 2011). narcolepsie Unrecognized et incontrôlée peut affecter très négativement la qualité de vie du patient. Cataplexie et la paralysie du sommeil sont semblables à la perte de tonus musculaire qui accompagne un rêve normal. Psychiatrie et psychopathologie Ce symptôme psychiatrique se retrouve dans des maladies telle la schizophrénie, l'hystérie, ou dans certains états hypnotiques. Dogs with autosomal recessive narcolepsy have a mutation in the gene coding for the orexin OX2 receptor, resulting in sleepiness and severe cataplexy elicited by social interaction and palatable food (Mitler et al., 1974, 1976; Mitler, 1975; Lin et al., 1999).

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