Cataplexy associated with narcolepsy: epidemiology, pathophysiology and management

Abstract

Although narcolepsy was first described over 100 years ago, most of what is known about the pathological changes in the CNS that are responsible for this unusual disease has been learned during the past few years. It is now known that narcolepsy is caused by the loss of a relatively few neurons that are responsible for producing the neuropeptide hypocretin in the CNS. The onset of narcolepsy typically occurs in early adulthood and may consist of a variety of symptoms; however, cataplexy (an abrupt, bilateral loss of skeletal muscle tone) is most specific to narcolepsy. TCAs were found to be beneficial for the treatment of cataplexy over 40 years ago and, more recently, the SSRIs have been used to treat the condition. The recent availability of sodium oxybate (the first drug to receive regulatory approval for the treatment of cataplexy) represents a significant advance in the treatment of narcolepsy, as it is highly efficacious for the treatment of cataplexy and shows promise for the treatment of excessive sleepiness and for improving sleep quality in patients with narcolepsy.