Normalization of hypocretin-1 in narcolepsy after intravenous immunoglobulin treatment

Abstract

In May 2006, a 28-year-old woman abruptly experienced excessive daytime sleepiness (EDS) and 2 to 3 short, refreshing but irresistible naps per day. She also reported increased nighttime sleep duration of more than 3 hours. Her Epworth Sleepiness Scale score was 22, indicating severe sleepiness. She also developed clear-cut generalized and partial cataplexy provoked by laughter but also by stress with a frequency of more than 1 attack per day. A rapid but large weight gain was noted up to 6 kg within 2 weeks, without a clear change in appetite. She never experienced sleep paralysis, hypnagogic hallucinations, or mood disorders. She had no personal or familial history of a particular disease, and no treatment. She was seen by us 15 days after the first cataplexy episode to confirm the diagnosis of narcolepsy with cataplexy.1 Nighttime polysomnography results were unremarkable; a multiple sleep latency test (PSG-MSLT) indicated a mean sleep latency of 5 minutes and 2 sleep-onset REM periods. She was positive for HLA DRB1*1501 and DQB1*0602. Brain …