Narcolepsy-cataplexy

Although difficult to estimate, the prevalence of narcolepsy is between 0.03% and 0.06%.7 Significant differences have been reported for various ethnic groups. Narcolepsy has a higher prevalence in the Japanese and a lower prevalence in the Israeli populations.8,9 Cataplexy is not required for diagnosis however, between 50% and 80% of patients with narcolepsy have accompanying cataplexy.10... 

The onset of narcolepsy-cataplexy is typically in adolescence and not at birth, suggesting that the disease may require... 

Cataplexy A sudden attack of muscle weakness with retention of clear consciousness that follows a strong emotional stimulus (e.g., elation, surprise, or anger) a characteristic symptom of narcolepsy. 

The sleep disorder narcolepsy, which affects around 1 in every 2000 people, is characterized by a tetrad of symptoms excessive daytime sleepiness, cataplexy (loss of muscle tone triggered by emotional arousal), hypnagogic hallucinations,... 

Narcolepsy, a sleep disorder characterized by excessive daytime sleepiness and cataplexy, may be caused by the lack of hypocretin mRNA and peptides in humans (Peyron et al., 2000) or a disruption of the hypocretin receptor 2 or its ligand in dogs and mice (Lin et al., 1999 Chemelli et al., 1999). Hypocretin-containing neurons are located exclusively in the dorsomedial, lateral, and perifornical hypothalamic areas (Peyron et al., 1998). Two hypocretin sequences, Hcrt-1 (orexin-A) and Hcrt-2 (orexin-B), are generated from a single preprohypocretin (De Lecea et al., 1998 Peyron et al, 1998 Sakurai et al, 1998). Axons from these neurons are found in the hypothalamus, locus coeruleus (LC), raphe nuclei, tuberomamillary nucleus, midline thalamus, all levels of spinal cord, sympathetic and parasympathetic centers, and many other brain regions... 

Orexins in sleep and wakefulness rodent models of narcolepsy-cataplexy... 

The cause of the orexin-containing cell loss in typical narcolepsy-cataplexy remains unknown, but the HLA association is intriguing because it suggests that autoimmunity could mediate a central disease process. This is not... 

The phenotypic characterization of genetically modified rodents has advanced the understanding of both the basis of narcolepsy-cataplexy and the functions of the orexin system in the normal animal. Here we review the... 

Severe narcolepsy-cataplexy comparable to the human disorder. 

Milder narcolepsy-cataplexy comparable to canine narcolepsy. OX2R signahng stabilizes wakefulness and also contributes to REM sleep gating Severe narcolepsy-cataplexy... 

Severe narcolepsy-cataplexy indistinguishable from orexin mice... 

Severe narcolepsy-cataplexy comparable to the human disorder. Cataplectic episodes can be separated from wakefulness-REM sleep transitions... 

Beuckmann, C. T., Sinton, C. M., Williams, S. C. et al. (2004). Expression of a poly-glutamine-ataxin-3 transgene in orexin neurons induces narcolepsy-cataplexy in the rat. J. Neurosci 24, 4469-77. 

Broughton, R Dunham, W., Newman, J. et al. (1988). Ambulatory 24 hour sleep-wake monitoring in narcolepsy-cataplexy compared to matched controls. Electroencephalogr. Clin. Neurophysiol. 70, 473-81. 

Broughton, R., Krupa, S., Boucher, B., Rivers, M. 8r Mullington, J. (1998). Impaired circadian waking arousal in narcolepsy-cataplexy. Sleep Res. Online 1, 159-65. 

Dauvilliers, Y., Carlander, B., Rivier, F., Touchon, J. 8r Tafti, M. (2004). Successful management of cataplexy with intravenous immunoglobulins at narcolepsy onset. Ann. Neurol. 56, 905-8. 

Mieda, M. Yanagisawa, M. (2006). Rodent models of narcolepsy-cataplexy. In The Orexin/Hypocretin System Physiology and Pathophysiology, ed. S. Nishino T. Sakurai, Totowa, NJ Humana Press, pp. 255-66. 

Mieda, M., Willie, J. T., Hara, J. et al (2004b). Orexin peptides prevent cataplexy and improve wakefulness in an orexin neuron-ablated model of narcolepsy in mice. Proc. Natl. Acad. Scl USA 101, 4649-54. 

Sturzenegger, C. Bassetti, C. L. (2004). The clinical spectrum of narcolepsy with cataplexy a reappraisal./. Sleep Res. 13, 395-406. 

Willie, J. T. Yanagisawa, M. (2007). Lessons from sleepy mice narcolepsy-cataplexy and the orexin neuropeptide system. In Narcolepsy and Hypersomnia, ed. C. Bassetti, M. Billiard E. Mignot, New York, NY Informa Healthcare, pp. 257-78. 

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