Open AccessReviewing the Clinical Implications of Treating Narcolepsy as an Autoimmune Disorder
Author(s) -
Maria Pia Giannoccaro,
Rocco Liguori,
Giuseppe Plazzi,
Fabio Pizza
Publication year - 2021
Publication title -
nature and science of sleep
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.715
H-Index - 34
ISSN - 1179-1608
DOI - 10.2147/nss.s275931
Subject(s) - narcolepsy , medicine , etiology , cataplexy , autoimmunity , immunotherapy , excessive daytime sleepiness , disease , sleep disorder , psychiatry , intensive care medicine , pediatrics , immunology , modafinil , immune system , insomnia
Narcolepsy type 1 (NT1) is a lifelong sleep disorder, primarily characterized clinically by excessive daytime sleepiness and cataplexy and pathologically by the loss of hypocretinergic neurons in the lateral hypothalamus. Despite being a rare disorder, the NT1-related burden for patients and society is relevant due to the early onset and chronic nature of this condition. Although the etiology of narcolepsy is still unknown, mounting evidence supports a central role of autoimmunity. To date, no cure is available for this disorder and current treatment is symptomatic. Based on the hypothesis of the autoimmune etiology of this disease, immunotherapy could possibly represent a valid therapeutic option. However, contrasting and limited results have been provided so far. This review discusses the evidence supporting the use of immunotherapy in narcolepsy, the outcomes obtained so far, current issues and future directions.