Abstract
Sporadic narcolepsy with cataplexy is a disabling disease that is strongly associated with the major histocompatibility class II allele HLA DQB1*0602 and is characterized by profound reduction in the cerebrospinal fluid (CSF) concentration of hypocretin 1 levels. This article provides a comprehensive review of the evidence that neurologic autoimmunity is the pathogenic basis of narcolepsy with cataplexy. Despite this evidence, specific antibody markers for narcolepsy have been elusive. Clinical trials using intravenous immunoglobulin infusions in recent onset narcolepsy with cataplexy have led to improvement in cataplexy in some patients. Future research must focus on elucidation of immune markers and early ameliorative treatments for narcolepsy.
Publication types
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Research Support, N.I.H., Extramural
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Review
MeSH terms
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Alleles
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Autoantigens / immunology
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Autoimmune Diseases of the Nervous System / genetics
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Autoimmune Diseases of the Nervous System / immunology*
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Autoimmune Diseases of the Nervous System / therapy
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Cataplexy / genetics
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Cataplexy / immunology
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Cataplexy / therapy
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HLA-DQ Antigens / genetics
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HLA-DQ beta-Chains
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Humans
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Immunization, Passive
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Intracellular Signaling Peptides and Proteins / cerebrospinal fluid
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Membrane Glycoproteins / genetics
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Narcolepsy / genetics
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Narcolepsy / immunology*
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Narcolepsy / therapy
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Neuropeptides / cerebrospinal fluid
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Orexins
Substances
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Autoantigens
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HCRT protein, human
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HLA-DQ Antigens
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HLA-DQ beta-Chains
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HLA-DQB1 antigen
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Intracellular Signaling Peptides and Proteins
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Membrane Glycoproteins
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Neuropeptides
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Orexins