Narcolepsy

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Narcolepsy is caused by deficient hypocretin transmission and is characterised by:

  • Excessive day time sleepiness
  • Abnormal REM sleep

The presence of cataplexy is diagnostic.[1] Sleep paralysis which can occur in the majority of the population at some time or the other and may be disturbing to patients, may need to be distinguished from the rarer but often under diagnosed narcolepsy.

Contents

Aetiology

Primary

  • In man associated with decrease hypocretin-1 in CSF and decreased number hypocretin neurons in brain
  • Most patients have HLA DQ?B1*0602 allele

Secondary

Prevalence

  • Suspected to be underdiagnosed - between 0.025 to 0.075 %
  • More common in Japan

Features

  • Bimodal onset with peaks around 15 years and 35 years
  • Sleepiness
    • Irresistible
    • Short and reoccurring typically at 2 hour intervals with refreshed on recovery
    • Often patients recall dreaming
  • Rapid eye movement manifestations
    • Cataplexy
    • Sleep paralysis
    • Hallucinations on sleep onset (hypnagogic hallucinations)

Differential diagnosis

Treatment

A full recent review[2] commented that scheduled naps can help but only if supplemented by active therapy.

Sleepiness

Cataplexy

See cataplexy for its treatment

Sleep pattern

References

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