Nature and Science of Sleep (May 2021)
Reviewing the Clinical Implications of Treating Narcolepsy as an Autoimmune Disorder
Abstract
Maria Pia Giannoccaro,1,2 Rocco Liguori,1,2 Giuseppe Plazzi,1,3 Fabio Pizza1,2 1IRCCS Istituto delle Scienze Neurologiche di Bologna, Ospedale Bellaria, Bologna, Italy; 2Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy; 3Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, Modena, ItalyCorrespondence: Giuseppe PlazziIRCCS Istituto delle Scienze Neurologiche di Bologna, Ospedale Bellaria, via Altura 3, Bologna, 40139, ItalyTel +39 051 4966926Fax +39 051 4966176Email [email protected]: 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.Keywords: narcolepsy type 1, immunotherapy, immunomodulation, intravenous immunoglobulin, steroid, monoclonal antibodies
