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 patients with NT1 enrolled at the time of diagnosis underwent clinical evaluation, a semistructured interview (including the Epworth Sleepiness Scale), nocturnal video-polysomnography, and the Multiple Sleep Latency Test. Patients were enrolled in order to establish 5 age-balanced groups (childhood, adolescence, adulthood, middle age, and senior). Results: The Epworth Sleepiness Scale score showed a significant increase with age, while self-reported diurnal total sleep time was lower in older and young adults, with the latter also complaining of automatic behaviors in more than 90% of patients. Children reported the cataplexy attacks to be more frequent (> 1/d in 95% of patients). "Recalling an emotional event,""meeting someone unexpectedly,""stress,"and "anger"were more frequently reported in adult and older adult patients as possible triggers of cataplexy. Neurophysiological data showed a higher number of sleep-onset rapid eye movement periods on the Multiple Sleep Latency Test in adolescent 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 patients with NT1; this variability may contribute to diagnostic delay and misdiagnosis.

Narcolepsy type 1 features across the life span: Age impact on clinical and polysomnographic phenotype / Lividini, A.; Pizza, F.; Filardi, M.; Vandi, S.; Ingravallo, F.; Antelmi, E.; Bruni, O.; Cosentino, F. I. I.; Ferri, R.; Guarnieri, B.; Marelli, S.; Ferini-Strambi, L.; Romigi, A.; Bonanni, E.; Maestri, M.; Terzaghi, M.; Manni, R.; Plazzi, G.. - In: JOURNAL OF CLINICAL SLEEP MEDICINE. - ISSN 1550-9389. - 17:7(2021), pp. 1363-1370. [10.5664/jcsm.9198]

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

Ferini-Strambi L.;
2021-01-01

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 patients with NT1 enrolled at the time of diagnosis underwent clinical evaluation, a semistructured interview (including the Epworth Sleepiness Scale), nocturnal video-polysomnography, and the Multiple Sleep Latency Test. Patients were enrolled in order to establish 5 age-balanced groups (childhood, adolescence, adulthood, middle age, and senior). Results: The Epworth Sleepiness Scale score showed a significant increase with age, while self-reported diurnal total sleep time was lower in older and young adults, with the latter also complaining of automatic behaviors in more than 90% of patients. Children reported the cataplexy attacks to be more frequent (> 1/d in 95% of patients). "Recalling an emotional event,""meeting someone unexpectedly,""stress,"and "anger"were more frequently reported in adult and older adult patients as possible triggers of cataplexy. Neurophysiological data showed a higher number of sleep-onset rapid eye movement periods on the Multiple Sleep Latency Test in adolescent 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 patients with NT1; this variability may contribute to diagnostic delay and misdiagnosis.
2021
Cataplexy
Emotional triggers
Narcolepsy type 1
Nocturnal sleep
Sleepiness
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11768/136536
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