<p>Narcolepsy is a&#xa0;sleep–wake disorder with lead symptoms of excessive daytime sleepiness, cataplexy (type&#xa0;1 narcolepsy (NT1)), and others, particularly REM sleep-related disturbances, which significantly impairs daily activities. The symptoms of NT1 are due to an orexin deficiency. This is thought to be caused by an autoimmune process in which the adaptive immune system selectively destroys orexin-producing neurons. The search for autoantibodies and autoreactive T&#xa0;cells has not yet yielded any clear evidence in favor of or against the autoimmune hypothesis. Nonetheless, there is a&#xa0;connection between an H1N1 influenza infection (swine flu) or vaccination with Pandemrix® (swine flu vaccine) and the occurrence of increased cases of NT1 in Scandinavian countries and China, which supports the autoimmune hypothesis. Current therapeutic approaches are purely symptomatic. Rapid diagnosis and appropriate treatment are crucial to prevent impairment of cognitive, emotional, and social development and secondary psychiatric disorders. Initial clinical trials of new orexin receptor agonists have shown very promising results.</p>

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Pathophysiologie der Narkolepsie

  • Ulf Kallweit,
  • Thomas Dittmar

摘要

Narcolepsy is a sleep–wake disorder with lead symptoms of excessive daytime sleepiness, cataplexy (type 1 narcolepsy (NT1)), and others, particularly REM sleep-related disturbances, which significantly impairs daily activities. The symptoms of NT1 are due to an orexin deficiency. This is thought to be caused by an autoimmune process in which the adaptive immune system selectively destroys orexin-producing neurons. The search for autoantibodies and autoreactive T cells has not yet yielded any clear evidence in favor of or against the autoimmune hypothesis. Nonetheless, there is a connection between an H1N1 influenza infection (swine flu) or vaccination with Pandemrix® (swine flu vaccine) and the occurrence of increased cases of NT1 in Scandinavian countries and China, which supports the autoimmune hypothesis. Current therapeutic approaches are purely symptomatic. Rapid diagnosis and appropriate treatment are crucial to prevent impairment of cognitive, emotional, and social development and secondary psychiatric disorders. Initial clinical trials of new orexin receptor agonists have shown very promising results.