Narcolepsy with cataplexy

• Published in: The Lancet (British edition) Vol. 369; no. 9560; pp. 499 - 511
• Main Authors: Dauvilliers, Yves, Dr, Arnulf, Isabelle, MD, Mignot, Emmanuel, Prof
• Format: Journal Article
• Language: English
• Published: London Elsevier Ltd 10-02-2007; Lancet; Elsevier Limited
• Subjects: Adolescent; Adult; Animals; Antidepressants; Apoptosis; Attention deficit hyperactivity disorder; Biological and medical sciences; Disease; Disease Models, Animal; Disorders of higher nervous function. Focal brain diseases. Central vestibular syndrome and deafness. Brain stem syndromes; Dogs; Environment; Female; General aspects; Genetic Predisposition to Disease; HLA Antigens - genetics; Humans; Internal Medicine; Intracellular Signaling Peptides and Proteins - cerebrospinal fluid; Intracellular Signaling Peptides and Proteins - physiology; Male; Medical sciences; Mice; Narcolepsy - diagnosis; Narcolepsy - drug therapy; Narcolepsy - genetics; Narcolepsy - physiopathology; Nervous system (semeiology, syndromes); Neurology; Neuropeptides - cerebrospinal fluid; Neuropeptides - physiology; Orexins; Patients; Sleep disorders; Studies; Substance abuse treatment; Human; Medicine; Nervous system diseases; Sleep disorder; Narcolepsy; Neurological disorder; Cataplexy
• ISSN: 0140-6736; 1474-547X
• DOI: 10.1016/S0140-6736(07)60237-2

Summary Narcolepsy with cataplexy is a disabling sleep disorder affecting 0·02% of adults worldwide. It is characterised by severe, irresistible daytime sleepiness and sudden loss of muscle tone (cataplexy), and can be associated with sleep-onset or sleep-offset paralysis and hallucinations, frequent movement and awakening during sleep, and weight gain. Sleep monitoring during night and day shows rapid sleep onset and abnormal, shortened rapid-eye-movement sleep latencies. The onset of narcolepsy with cataplexy is usually during teenage and young adulthood and persists throughout the lifetime. Pathophysiological studies have shown that the disease is caused by the early loss of neurons in the hypothalamus that produce hypocretin, a wakefulness-associated neurotransmitter present in cerebrospinal fluid. The cause of neural loss could be autoimmune since most patients have the HLA DQB1*0602 allele that predisposes individuals to the disorder. Treatment is with stimulant drugs to suppress daytime sleepiness, antidepressants for cataplexy, and γ hydroxybutyrate for both symptoms. Because narcolepsy is an under-recognised disease, it is important that general practitioners and other primary health-care workers identify abnormal daytime sleepiness early.