Abstract
Narcolepsy is now recognized as a distinctive disorder with specific pathophysiology and neurochemical abnormalities. Findings on the role of the neuropeptide hypocretin are opening new avenues of research and new strategies for therapy. Recently, neuropsychological and electrophysiological studies have provided evidence for reduced memory performance on standard memory tests in addition to subjective complaints of forgetfulness which may be related to changes in attentional processing. Further studies are, however, necessary to clarify the neuropsychological profile in narcolepsy. This review focuses on the recent advances in understanding narcolepsy.
Highlights
Narcolepsy is recognized as a distinctive disorder with specific pathophysiology and neurochemical abnormalities
This finding led to the suggestion that cataplexy, hypnagogic hallucinations as well as sleep paralysis might be due to changes in REM sleep mechanisms [107,126]
Narcolepsy can be clearly distinguished from disorders that are characterized by excessive sleepiness and nocturnal sleep disturbances, such as sleep apnoea or restless leg syndrome by criteria set by the International Classification for Sleep Disorders [62]
Summary
Descriptions of unusual excessive sleepiness appeared as early as 1829 and 1836 in publications by Schindler and Bright [35,72]. A few years later in 1880, Gelineau [39] introduced the term narcolepsy (Grecian αpkη: “convulsion” and λεπσıσ: “receive”) to define pathological conditions characterized by unexpected short sleep episodes as well as spontaneous muscle tone loss which occurred in conjunction with emotional events. Later Hennenberg [51] introduced the term cataplexy for the description of sudden loss of muscle tone, and Redlich [108] used the term “affektiver Tonusverlust” (affective loss of tone) to describe the same symptom. Of special importance was the discovery of sleep onset REM Periods (SOREM) in narcoleptic patients in 1960 [133]. This finding led to the suggestion that cataplexy, hypnagogic hallucinations as well as sleep paralysis might be due to changes in REM sleep mechanisms [107,126]. The discovery of narcolepsy in dogs and its genetic basis in the 1970s [33,67] and the observation of the role of the hypocretin system [75,94] are regarded as the beginning of a new era of narcolepsy research
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