Cataplexy may develop in children affected with Niemann-Pick disease type C. Leading CNS causes include diencephalic and midbrain tumors, MS, strokes, cysts, vascular malformations, encephalitis, cerebral trauma, and paraneoplastic syndrome with anti-Ma2 antibodies and it may present with narcoleptic-like sleep attacks and other manifestations ( Nishino, 2007 Nishino and Mignot, 2011 Clavelou et al., 1995). Depending on the presence of cataplexy/reduced CSF hypocretin levels, narcolepsy type 1 and 2 may be found. “Symptomatic narcolepsy” or “secondary narcolepsy” is the previously used term to describe narcolepsy associated with underlying structural, genetic, inflammatory, or vascular abnormality impacting the hypothalamus and leading to severe CNS hypersomnolence with or without cataplexy or abnormal CSF hypocretin levels. Differential diagnosis of narcolepsy and cataplexy are summarized in Table 101.7 ( Postiglione et al., 2018). The clinical history, pre/post event triggers/confusion, physical examination, and EEG should be helpful in differentiating these conditions. Automatic behavior should be differentiated from the automatisms observed in partial complex seizures and psychogenic fugue. Sleep paralysis, in the context of narcolepsy, should be differentiated from isolated physiological and familial sleep paralysis in which other manifestations of narcolepsy are absent. Atonic seizures are accompanied by transient loss of consciousness and EEG evidence of slow spike-and-wave discharges or multiple spike-and-wave discharges. Absence spells are characterized by staring with vacant expression lasting up to 30 seconds, with an altered state of alertness associated with characteristic 3-Hz spike-and-wave patterns in the EEG. In addition, patients with seizures may have generalized tonic-clonic movements, postictal confusion, and epileptiform discharges on EEG. A partial complex seizure, however, is characterized by an altered state of consciousness, unlike cataplexy. Pseudocataplexy is a functional disorder, often seen in patients with cataplexy, in which there are negative thoughts rather than laughter, the typical precipitant of true cataplexy, and characterized by spells extending over minutes to hours rather than the seconds to minute/s characteristic of bona fide cataplexy ( Plazzi et al., 2010 Shankar et al., 2010). All rights reserved.Joseph Jankovic MD, in Bradley and Daroff's Neurology in Clinical Practice, 2022 Differential Diagnosis of Cataplexy and Other Features of NarcolepsyĬataplectic attacks may be mistaken for partial complex seizures, absence spells, atonic seizures (as well as gelastic-atonic seizures characterized by laughing followed by loss of muscle tone), drop attacks, basilar migraines, vertebrobasilar insufficiency, syncope, and pseudocataplexy ( Box 101.14 ). 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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