Advances in narcolepsy.
Narcolepsy with cataplexy is a rare but life-long and challenging disorder. Current insight into the pathophysiology of this condition seems to be autoimmune-mediated postnatal cell death of hypocretin neurons occurring by organ-specific autoimmune targeting with HLA-T-cell receptor interactions. The hypocretin system seems to have an influence on multiple organ systems beyond its wake-promoting mechanisms. The recent availability of cerebrospinal fluid hypocretin-1 analysis has led to definitive diagnostic criteria for narcolepsy with cataplexy. Pharmacologic first-line treatments for excessive daytime sleepiness and cataplexy is sodium oxybate, with modafinil for daytime sleepiness, in adults and children. Other investigative agents and treatment modalities hold promise in future directions for narcolepsy.
Cao
Michelle
M
Division of Sleep Medicine, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA 94305, USA. mhtcao1@stanford.edu <mhtcao1@stanford.edu>
D000928:Antidepressive Agents / Q000627:therapeutic use; D002385:Cataplexy / Q000188:drug therapy / Q000503:physiopathology; D000697:Central Nervous System Stimulants / Q000627:therapeutic use; D006801:Humans; D018972:Insulin-Like Growth Factor Binding Protein 3 / Q000378:metabolism; D047908:Intracellular Signaling Peptides and Proteins / Q000378:metabolism; D009290:Narcolepsy / Q000188:drug therapy / Q000503:physiopathology; D009479:Neuropeptides / Q000378:metabolism; D000068797:Orexins
D000928:Antidepressive Agents; D000697:Central Nervous System Stimulants; C000596229:HCRT protein, human; D018972:Insulin-Like Growth Factor Binding Protein 3; D047908:Intracellular Signaling Peptides and Proteins; D009479:Neuropeptides; D000068797:Orexins