Narcolepsy type 1 and
narcolepsy type 2 are central disorders of
hypersomnolence.
Narcolepsy type 1 is characterized by
excessive daytime sleepiness and
cataplexy and is associated with
hypocretin-1 deficiency. On the other hand, in
narcolepsy type 2, cerebrospinal fluid
hypocretin-1 levels are normal and
cataplexy absent. Despite major advances in our understanding of
narcolepsy mechanisms, its current management is only symptomatic. Treatment options may vary from a single drug that targets several symptoms, or multiple medications that each treats a specific symptom. In recent years,
narcolepsy treatment has changed with the widespread use of
modafinil/
armodafinil for
daytime sleepiness,
antidepressants (selective
serotonin and dual
serotonin and noradrenalin reuptake inhibitors) for
cataplexy, and
sodium oxybate for both symptoms. Other psychostimulants can also be used, such as
methylphenidate,
pitolisant and rarely
amphetamines, as third-line
therapy. Importantly, clinically relevant subjective and objective measures of
daytime sleepiness are required to monitor the treatment efficacy and to provide guidance on whether the treatment goals are met. Associated symptoms and comorbid conditions, such as hypnagogic/hypnopompic
hallucinations,
sleep paralysis, disturbed nighttime sleep, unpleasant dreams, REM- and non REM-related
parasomnias, depressive symptoms,
overweight/
obesity, and
obstructive sleep apnea, should also be taken into account and managed, if required. In the near future, the efficacy of new
wake-promoting drugs, anticataplectic agents,
hypocretin replacement
therapy and
immunotherapy at the early stages of the disease should also be evaluated.