This narcolepsy classification has been previously shown to have an interrater reliability of 0.98. 7 These diagnostic criteria require specific electrophysiologic data or physician-witnessed cataplexy with confirmed areflexia. All patients fulfilled the Mayo Narcolepsy Research criteria for definite narcolepsy. The negative and positive predictive value of the abbreviated survey was also measured.įifty-five patients with narcolepsy and cataplexy were selected based on a clear history of cataplexy. ![]() The specific aim of this study was to investigate which emotional experiences triggered cataplexy, observe the manifestations of the episode and determine whether a brief survey that would aid in identifying cataplexy could be developed. The proposed mechanism implicates postsynaptic inhibition of spinal alpha motoneurons by activated cells, including cholinoceptive ones in the midbrain, pontine, and medullary regions. Therefore, cataplexy is believed to represent dissociated REM phenomena intruding into wakefulness after an emotional trigger. The decreased muscle tone observed in cataplexy is similar to the absent electromyographic tone documented during rapid eye movement (REM) sleep. Hypocretin 1 deficiency appears to play a permissive role, allowing certain emotional states to cause rapid shifts in downstream neurotransmitters, which results in cataplexy. 4 The hypocretin neurons project widely throughout the central nervous system, including the spinal cord. Recently, narcolepsy with cataplexy was associated with a deficiency of the neuropeptide hypocretin 1 (also known as orexin A), which is produced by a small number of cell bodies located only in the lateral hypothalamus. Because of this close association, the pathologic features of narcolepsy provide valuable information about the factors conferring vulnerability to cataplexy. 3 When associated with excessive daytime sleepiness, with or without any other narcolepsy symptoms, cataplexy is considered pathognomonic of narcolepsy. Known primarily as a symptom of the sleep disorder narcolepsy, cataplexy, in extremely rare cases, has been associated with other disorders, which include Nieman-Pick disease type C, Norrie’s disease, mid-brain tumors, and familial isolated cataplexy. Few clinical phenomena better illustrate the ability of an emotional experience to cause neurochemical alterations that result in observable behavioral signs. 2 Consciousness and awareness of the environment are preserved throughout the episode. 1 The clinical manifestations are varied, ranging from involuntary eye closure and neck weakness to a subtle buckling of the knees to generalized muscle weakness that causes the patient to collapse. In a state of cataplexy, an intense emotional state triggers objective transient muscle weakness verified by areflexia. All rights reserved.Cataplexy is one of the most intriguing examples of how thought content can alter neurologic functioning. We performed a review of literature (MEDLINE and EMBASE database), without limited date or publication restrictions.Īngelman Syndrome Cataplexy Narcolepsy Niemann-Pick type C Disease Norrie Disease Prader-Willi Syndrome.Ĭopyright © 2019 Elsevier B.V. In this review, we described few conditions of cataplexy not related to narcolepsy. ![]() Therefore, it is essential disconnecting cataplexy from narcolepsy especially in pediatric population and after use of a few medications. Yet, the most prevalent causes of cataplexy without narcolepsy are rare genetic diseases which explains why cataplexy is classically linked to narcolepsy. In addition, cataplexy can be a side effect of several drugs (eg, lamotrigine, clozapine, and gamma-hydroxybutyrate). Other causes of cataplexy include Niemann-Pick type C Disease, Angelman Syndrome, Norrie Disease, Prader-Willi Syndrome. Cataplexy is a transient loss of muscle tone that can be triggered by emotions such as laughter, excitement or fear.
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