Althea Lividini1, Fabio Pizza2,3, Marco Filardi2, Stefano Vandi2,3, Francesca Ingravallo1, Elena Antelmi4, Oliviero Bruni5, Filomena Irene Ilaria Cosentino6, Raffaele Ferri6, Biancamaria Guarnieri7, Sara Marelli8, Luigi Ferini-Strambi8, Andrea Romigi9, Enrica Bonanni10, Michelangelo Maestri10, Michele Terzaghi11,12, Raffaele Manni11, Giuseppe Plazzi3,13. 1. Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy. 2. Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy. 3. IRCCS, Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy. 4. Neurology Unit, Movement Disorders Division, Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona, Italy. 5. Department of Developmental and Social Psychology, Sapienza University of Rome, Italy. 6. Sleep Research Center, Department of Neurology IC, Oasi Research Institute-IRCCS, Troina, Italy. 7. Center of Sleep Medicine, Department of Neurology, Villa Serena Hospital and Villa Serena Foundation for Research, Città S. Angelo, Pescara, Italy. 8. Department of Clinical Neurosciences, IRCCS San Raffaele Scientific Institute, Neurology-Sleep Disorders Centre, Milan, Italy. 9. IRCCS Neuromed Istituto Neurologico Mediterraneo Sleep Medicine Centre, Pozzilli, Italy. 10. Department of Clinical and Experimental Medicine, Neurology Unit, University of Pisa, Italy. 11. Unit of Sleep Medicine and Epilepsy, IRCCS Mondino Foundation, Pavia, Italy. 12. Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy. 13. Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, Modena, Italy.
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.
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.
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