RT Journal Article SR Electronic T1 Validation of Multiple Sleep Latency Test for the diagnosis of pediatric narcolepsy type 1 JF Neurology JO Neurology FD Lippincott Williams & Wilkins SP e1034 OP e1044 DO 10.1212/WNL.0000000000008094 VO 93 IS 11 A1 Pizza, Fabio A1 Barateau, Lucie A1 Jaussent, Isabelle A1 Vandi, Stefano A1 Antelmi, Elena A1 Mignot, Emmanuel A1 Dauvilliers, Yves A1 Plazzi, Giuseppe A1 for the MonBo Study Group YR 2019 UL http://n.neurology.org/content/93/11/e1034.abstract AB Objective To validate polysomnographic markers (sleep latency and sleep-onset REM periods [SOREMPs] at the Multiple Sleep Latency Test [MSLT] and nocturnal polysomnography [PSG]) for pediatric narcolepsy type 1 (NT1) against CSF hypocretin-1 (hcrt-1) deficiency and presence of cataplexy, as no criteria are currently validated in children.Methods Clinical, neurophysiologic, and, when available, biological data (HLA-DQB1*06:02 positivity, CSF hcrt-1 levels) of 357 consecutive children below 18 years of age evaluated for suspected narcolepsy were collected. Best MSLT cutoffs were obtained by receiver operating characteristic (ROC) curve analysis by contrasting among patients with available CSF hcrt-1 assay (n = 228) with vs without CSF hcrt-1 deficiency, and further validated in patients without available CSF hcrt-1 against cataplexy (n = 129).Results Patients with CSF hcrt-1 deficiency were best recognized using a mean MSLT sleep latency ≤8.2 minutes (area under the ROC curve of 0.985), or by at least 2 SOREMPs at the MSLT (area under the ROC curve of 0.975), or the combined PSG + MSLT (area under the ROC curve of 0.977). Although specificity and sensitivity of reference MSLT sleep latency ≤8 minutes and ≥2 SOREMPs (nocturnal SOREMP included) was 100% and 94.87%, the combination of MSLT sleep latency and SOREMP counts did not improve diagnostic accuracy. Age or sex also did not significantly influence these results in our pediatric population.Conclusions At least 2 SOREMPs or a mean sleep latency ≤8.2 minutes at the MSLT are valid and reliable markers for pediatric NT1 diagnosis, a result contrasting with adult NT1 criteria.Classification of evidence This study provides Class III evidence that for children with suspected narcolepsy, polysomnographic and MSLT markers accurately identify those with narcolepsy type 1.ALL-SOREMPs=the total number of sleep-onset REM periods when merging nocturnal polysomnography sleep-onset REM periods to Multiple Sleep Latency Test naps opportunities; AUC=area under the curve; BMI=body mass index; EDS=excessive daytime sleepiness; ESS=Epworth Sleepiness Scale; hcrt-1=hypocretin-1; MSLT=Multiple Sleep Latency Test; MSLT-SL=Multiple Sleep Latency Test mean sleep latency to the first epoch of sleep; MSLT-SOREMPs=number of Multiple Sleep Latency Test sleep-onset REM periods across 5 naps; NT1=type 1 narcolepsy; NT2=type 2 narcolepsy (narcolepsy without cataplexy); PSG=polysomnography; ROC=receiver operating characteristic; SL=sleep latency; SOREMP=sleep-onset REM period