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EnglishNarcolepsy type 1 results from probable autoimmune disruption of hypothalamic hypocretinergic neurons. Secondary narcolepsy can occur as a result of other conditions affecting the central nervous system, including limbic paraneoplastic encephalitis. We report the case of a 19-year-old patient presenting with acute-onset diurnal hypersomnolence, hyperphagia, sexual dysfunction, and psychiatric disturbances. Further investigations revealed a limbic paraneoplastic encephalitis associated with mediastinal thymic seminoma. Tumor removal and immunosuppressive treatment resulted in a partial benefit on psychiatric disturbances but did not improve daytime sleepiness. A comprehensive sleep assessment led to the diagnosis of secondary narcolepsy type 1 with reduced cerebrospinal fluid hypocretin-1 levels and revealed the presence of the HLA DQB1*0602 allele, typically associated with idiopathic narcolepsy, for which we hypothesize a possible immunopathogenic role. Sodium oxybate was successfully administered. Narcolepsy is often overlooked in patients with limbic paraneoplastic encephalitis. A prompt assessment and an adequate symptomatic treatment can improve the disease burden. CITATION: Rossi S, Asioli GM, Rizzo G, et al. Onset of narcolepsy type 1 in a paraneoplastic encephalitis associated with a thymic seminoma. J Clin Sleep Med. 2021;17(12):2557-2560.
| Titolo: | Onset of narcolepsy type 1 in a paraneoplastic encephalitis associated with a thymic seminoma | |
| Autore/i: | Rossi S.; Asioli G. M.; Rizzo G.; Sallemi G.; Moresco M.; Franceschini C.; Pizza F.; Plazzi G. | |
| Autore/i Unibo: | ||
| Anno: | 2021 | |
| Rivista: | ||
| Digital Object Identifier (DOI): | http://dx.doi.org/10.5664/jcsm.9496 | |
| Abstract: | Narcolepsy type 1 results from probable autoimmune disruption of hypothalamic hypocretinergic neurons. Secondary narcolepsy can occur as a result of other conditions affecting the central nervous system, including limbic paraneoplastic encephalitis. We report the case of a 19-year-old patient presenting with acute-onset diurnal hypersomnolence, hyperphagia, sexual dysfunction, and psychiatric disturbances. Further investigations revealed a limbic paraneoplastic encephalitis associated with mediastinal thymic seminoma. Tumor removal and immunosuppressive treatment resulted in a partial benefit on psychiatric disturbances but did not improve daytime sleepiness. A comprehensive sleep assessment led to the diagnosis of secondary narcolepsy type 1 with reduced cerebrospinal fluid hypocretin-1 levels and revealed the presence of the HLA DQB1*0602 allele, typically associated with idiopathic narcolepsy, for which we hypothesize a possible immunopathogenic role. Sodium oxybate was successfully administered. Narcolepsy is often overlooked in patients with limbic paraneoplastic encephalitis. A prompt assessment and an adequate symptomatic treatment can improve the disease burden. CITATION: Rossi S, Asioli GM, Rizzo G, et al. Onset of narcolepsy type 1 in a paraneoplastic encephalitis associated with a thymic seminoma. J Clin Sleep Med. 2021;17(12):2557-2560. | |
| Data stato definitivo: | 2022-02-16T16:35:19Z | |
| Appare nelle tipologie: | 1.01 Articolo in rivista |
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