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Narcolepsy, a syndrome of unknown origin is characterised by excessive daytime sleepiness and often disturbed nocturnal sleep and pathological manifestations of REM sleep. From the Greek ναρκωστς meaning to benumb or stupefy, narcolepsy is often called Gélineau’s syndrome, after the French physician Jean Baptiste Edouard Gélineau, who in 1880 gave the first comprehensive description.1 Westphal described it but more notable among early recognisable clinical portrayals is that of Thomas Willis.2 Willis did not use the word narcolepsy that was first coined by Gélineau as:
“a rare neurosis … characterised by an urgent necessity to sleep, sudden and of short duration which recurred at intervals more or less long.”1
Willis noted that it was not just an embarrassing somnolence but a humoral disease. He thought that the body was able to produce its own narcotic substances.
Adie and later Daniels recognised an association between daytime sleepiness,5 cataplexy, sleep paralysis, and hypnagogic hallucination—associations not known to Willis or Gélineau.
“utter incapacity for motion or speech, difficult respirations, and extreme dread.”
More recently Mignot and others have shown that neurons that secrete hypocretins (primarily in the hypothalamus) are depleted or damaged in narcolepsy.8 And, they are absent or diminished in CSF of most narcoleptics studied. Although dogs have a mutation of the gene encoding hypocretins (orexins), so far consistent genetic anomalies have been rare in man.
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