Narcolepsy type 1 features across the life span: age impact on clinical and polysomnographic phenotype

Althea Lividini, Fabio Pizza, Marco Filardi, Stefano Vandi, Francesca Ingravallo, Elena Antelmi, Oliviero Bruni, Filomena Irene Ilaria Cosentino, Raffaele Ferri, Biancamaria Guarnieri, Sara Marelli, Luigi Ferini-Strambi, Andrea Romigi, Enrica Bonanni, Michelangelo Maestri, Michele Terzaghi, Raffaele Manni, Giuseppe Plazzi

Research output: Contribution to journalArticlepeer-review

Abstract

STUDY OBJECTIVES: Narcolepsy type 1 (NT1) is a chronic neurological disorder typically arising during adolescence and young adulthood. Recent studies demonstrated that NT1 presents with age-specific features, especially in children. With this study we aimed to describe and to compare the clinical pictures of NT1 in different age groups.

METHODS: In this cross-sectional, multicenter study, 106 untreated NT1 patients, enrolled at the time of diagnosis, underwent clinical evaluation, a semi-structured interview (including the Epworth Sleepiness Scale - ESS), nocturnal video-polysomnography and the Multiple Sleep Latency Test (MSLT). Patients were enrolled in order to establish five age-balanced groups (childhood, adolescence, adulthood, middle-aged and seniors).

RESULTS: The ESS score showed a significant increase with age, while self-reported diurnal total sleep time was lower in elderly and young adults, with the latter also complaining of automatic behaviors in more than 90% of cases. Children reported the cataplexy attacks to be more frequent (>1/day in 95% of cases). "Recalling an emotional event", "meeting someone unexpectedly", "stress" and "anger" were more frequently reported in adult and elderly patients as possible triggers of cataplexy. Neurophysiological data showed a higher number of SOREMPs on the MSLT in adolescents compared to senior patients and an age-progressive decline in sleep efficiency.

CONCLUSIONS: Daytime sleepiness, cataplexy features and triggers, and nocturnal sleep structure showed age-related difference in NT1 patients; this variability may contribute to diagnostic delay and misdiagnosis.

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