Narcolepsy: immunological aspects.
Narcolepsy with cataplexy is a debilitating sleep disorder with an estimated prevalence of about 0.05%. Narcolepsy is caused by a selective loss of hypocretin (orexin) producing neurons in the perifornical hypothalamus. Based on the very strong association with the HLA subtype DQB1*0602, it is currently hypothesized narcolepsy is caused by an autoimmune-mediated process directed at the hypocretin neurons. So far however, studies focusing on general markers of (auto)immune activation, as well as humoral immunity against the hypocretin system have not yielded consistent results supporting this hypothesis.
Overeem
Sebastiaan
S
Department of Neurology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands. s.overeem@neuro.umcn.nl
Black
John Logan
JL
Lammers
Gert Jan
GJ
D001323:Autoantibodies / Q000276:immunology; D008040:Genetic Linkage; D006683:HLA-DQ Antigens / Q000235:genetics / Q000276:immunology*; D059866:HLA-DQ beta-Chains; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins / Q000235:genetics / Q000378:metabolism; D008562:Membrane Glycoproteins / Q000235:genetics / Q000276:immunology*; D009290:Narcolepsy / Q000235:genetics / Q000276:immunology* / Q000378:metabolism; D009479:Neuropeptides / Q000235:genetics / Q000378:metabolism; D000068797:Orexins
D001323:Autoantibodies; D006683:HLA-DQ Antigens; D059866:HLA-DQ beta-Chains; C069051:HLA-DQB1 antigen; D047908:Intracellular Signaling Peptides and Proteins; D008562:Membrane Glycoproteins; D009479:Neuropeptides; D000068797:Orexins