TYPE 1 NARCOLEPSY WITH CATAPLEXY HOW TO

However, more recently, patients with narcolepsy have tested negative for TRIB2 antibodies suggesting that something unique occurred in those first cases, for example the occurrence of a specific NT1 triggering infectious agent in mid-2000s that has since disappeared. This finding was replicated in two independent studies in other patient populations (American and Japanese). In 2010, a European study reported autoantibodies against tribbles homolog 2 (TRIB2) in 14% of patients versus 5% of controls. Older studies typically searched for serum or cerebrospinal fluid autoantibodies directed against prepro-hypocretin, brain extracts, or hypocretin neurons but with no striking findings. Researchers in the field have for many years been searching for evidence of autoantibodies or T-cell reactivity directed toward hypocretin neurons. Yes: A Specific Adaptive Immune Response Is Directed to Hypocretin/Orexin Neurons in NT1

TYPE 1 NARCOLEPSY WITH CATAPLEXY HOW TO

The focus of the review is on the criteria for autoimmunity and how to classify NT1 in light of recent findings. This review is inspired by the many interesting discussions at the Symposium. Refs 4вЂ“6)) increasing our understanding of the immunological, genetic, and epidemiological aspects of the disease. In September 2018, at the 7th International Symposium on Narcolepsy in Boston, several new breakthroughs in the field were presented (and since published, e.g. Two or more of these criteria must be fulfilled for a disease to be considered autoimmune.

For classification of autoimmune diseases, the following criteria are often used: (1) a specific adaptive immune response is directed to the affected organ or tissue, (2) autoreactive T cells and/or autoantibodies are present in the affected organ or tissue, (3) autoreactive T cells and/or autoantibodies can transfer the disease to healthy individuals or animals, (4) immunization with the autoantigen induces the disease in animal models, and (5) elimination or suppression of the autoimmune response prevents disease progression or even ameliorates the clinical manifestation. Nevertheless, it remains a matter of debate on how to prove that a given disease is indeed an autoimmune disease.ДЄ broadly accepted definition of autoimmunity states that an autoimmune disease is caused by self-directed inflammation, where aberrant adaptive immune system responses lead to breaking of tolerance and consequently the development of immune reactivity toward one or more self-antigens. To date, about 60вЂ“80 diseases (not including NT1) have a proven or strongly suspected autoimmune etiology. Even though this points in the direction of pathogenesis involving the immune system, it is not proof of autoimmunity.

From epidemiological studies, there is substantial evidence that upper airway infections, most likely influenza A and ОІ-hemolytic streptococcal infections, can precipitate the onset of narcolepsy. Ample genetic and epidemiological evidence points in this direction, but this evidence is mainly circumstantial. IntroductionДЇor many years, researchers have hypothesized that the loss of hypocretin/orexin signaling in Narcolepsy Type 1 (NT1) is caused by an autoimmune process. The review summarizes the latest findings on autoimmunity in narcolepsy type 1 and discusses what remaining puzzles need to be solved before narcolepsy type 1 can be classified as a genuine autoimmune disease. In conclusion, narcolepsy type 1 does still not fully meet the criteria for being classified as a genuine autoimmune disease, but more and more results are pointing in that direction. Most importantly, it is still controversial whether suppression of the autoimmune response can prevent disease progression. It is also unclear if the autoreactive T cells and/or autoantibodies can transfer the disease to healthy individuals or animals or if immunization with the proposed autoantigens can induce the disease in animal models. Autoreactive T cells and autoantibodies have been detected in blood samples from patients, but it remains to be seen if these T cells or antibodies are in fact present in the hypothalamus.

In favor of the autoimmune hypothesis are data showing that in narcolepsy type 1 a specific adaptive immune response is directed to hypocretin/orexin neurons. In this review, a set of commonly used criteria for autoimmunity is described and applied to narcolepsy type 1. In fact, it remains a matter of debate how to prove that a given disease is indeed an autoimmune disease. Ample genetic and epidemiological evidence points in the direction of a pathogenesis involving the immune system, but this is not considered proof of autoimmunity. Narcolepsy type 1 is hypothesized to be an autoimmune disease targeting the hypocretin/orexin neurons in the hypothalamus.

Type 1 narcolepsy with cataplexy