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.
Rossi S., Asioli G.M., Rizzo G., Sallemi G., Moresco M., Franceschini C., et al. (2021). Onset of narcolepsy type 1 in a paraneoplastic encephalitis associated with a thymic seminoma. JOURNAL OF CLINICAL SLEEP MEDICINE, 17(12), 2557-2560 [10.5664/jcsm.9496].
Onset of narcolepsy type 1 in a paraneoplastic encephalitis associated with a thymic seminoma
Rossi S.;Moresco M.;Pizza F.;
2021
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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
