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Abstract
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.
Glossary
- 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
Footnotes
↵* These authors contributed equally to this work as co–first authors.
↵‡ These authors contributed equally to this work as co–last authors.
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Coinvestigators are listed in appendix 2 at the end of the article.
Editorial, page 469
Class of Evidence: NPub.org/coe
- Received December 9, 2018.
- Accepted in final form May 21, 2019.
- © 2019 American Academy of Neurology
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