Lucie Barateau1,2,3, Michel Lecendreux4,5, Sofiene Chenini6,2, Anna Laura Rassu6,2, Régis Lopez6,2,3, Carole Pesenti2, Isabelle Jaussent3, Séverine Béziat3, Yves Dauvilliers1,2,3. 1. Sleep-Wake Disorders Unit, Department of Neurology, Gui-de-Chauliac Hospital, CHU Montpellier, France ydauvilliers@yahoo.fr lucie.barateau@gmail.com. 2. National Reference Centre for Orphan Diseases, Narcolepsy, Idiopathic Hypersomnia, and Kleine-Levin Syndrome, Montpellier, France. 3. INM, Univ Montpellier, INSERM, Montpellier, France. 4. Pediatric Sleep Centre, Hospital Robert-Debré, Paris, France. 5. National Reference Centre for Orphan Diseases, Narcolepsy, Idiopathic Hypersomnia, and Kleine-Levin Syndrome, Paris, France. 6. Sleep-Wake Disorders Unit, Department of Neurology, Gui-de-Chauliac Hospital, CHU Montpellier, France.
Abstract
BACKGROUND AND OBJECTIVE: We validated the Narcolepsy Severity Scale (NSS) in adults with NT1 to quantify the severity, frequency and consequences of the five key narcolepsy symptoms over the last month. We now developed the pediatric Narcolepsy Severity Scale (NSS-P). The aims of this study were: (1) to assess NSS-P psychometric properties, validity, and reliability, and (2) to evaluate its responsiveness to treatment in a well-characterized sample of children and adolescents with NT1. METHODS: The NSS was reformulated for children and the item about driving removed. The total score of the 14-item NSS-P ranges from 0 to 54, and higher scores reflect more severe disease. Children and adolescents (n=209, 6-17 years of age) with NT1 diagnosed in two Reference Centers for Narcolepsy in France were consecutively asked to fill in the NSS-P. The scale was fully and correctly completed by 160 (10-18 years of age, 68 untreated). Moreover, 65 participants completed it twice (33 before/during treatment, and 32 under the same treatment). The NSS-P psychometric properties, score changes before/during treatment, and convergent validity with other clinical parameters were assessed. RESULTS: The NSS-P showed adequate psychometric properties with significant item-total score correlations. Factor analysis indicated a four-factor solution with good reliability. The NSS-P score was lower in treated than untreated patients with a mean difference of 3.71±1.45, with a minimum clinically important difference between untreated and treated patients in the longitudinal sample estimated at 4 points. Four severity levels were defined (mild, moderate, severe, very severe) with between-group differences related to treatment. The NSS-P total score was associated with self-reported sleepiness, insomnia, and depressive symptoms. Its temporal stability was satisfactory. DISCUSSION: We validated a brief instrument to assess NT1 symptom frequency, severity and consequences in ≥10-year-old children and adolescents, with four clinically relevant severity score ranges. This scale constitutes a relevant tool to improve and provide guidance for NT1 management in pediatric populations. The ease of administration, its good psychometric properties, and its sensitivity to detect symptom changes after treatment ensure NSS-P future use in clinical and research settings.
BACKGROUND AND OBJECTIVE: We validated the Narcolepsy Severity Scale (NSS) in adults with NT1 to quantify the severity, frequency and consequences of the five key narcolepsy symptoms over the last month. We now developed the pediatric Narcolepsy Severity Scale (NSS-P). The aims of this study were: (1) to assess NSS-P psychometric properties, validity, and reliability, and (2) to evaluate its responsiveness to treatment in a well-characterized sample of children and adolescents with NT1. METHODS: The NSS was reformulated for children and the item about driving removed. The total score of the 14-item NSS-P ranges from 0 to 54, and higher scores reflect more severe disease. Children and adolescents (n=209, 6-17 years of age) with NT1 diagnosed in two Reference Centers for Narcolepsy in France were consecutively asked to fill in the NSS-P. The scale was fully and correctly completed by 160 (10-18 years of age, 68 untreated). Moreover, 65 participants completed it twice (33 before/during treatment, and 32 under the same treatment). The NSS-P psychometric properties, score changes before/during treatment, and convergent validity with other clinical parameters were assessed. RESULTS: The NSS-P showed adequate psychometric properties with significant item-total score correlations. Factor analysis indicated a four-factor solution with good reliability. The NSS-P score was lower in treated than untreated patients with a mean difference of 3.71±1.45, with a minimum clinically important difference between untreated and treated patients in the longitudinal sample estimated at 4 points. Four severity levels were defined (mild, moderate, severe, very severe) with between-group differences related to treatment. The NSS-P total score was associated with self-reported sleepiness, insomnia, and depressive symptoms. Its temporal stability was satisfactory. DISCUSSION: We validated a brief instrument to assess NT1 symptom frequency, severity and consequences in ≥10-year-old children and adolescents, with four clinically relevant severity score ranges. This scale constitutes a relevant tool to improve and provide guidance for NT1 management in pediatric populations. The ease of administration, its good psychometric properties, and its sensitivity to detect symptom changes after treatment ensure NSS-P future use in clinical and research settings.
Authors: Michel Lecendreux; Giuseppe Plazzi; Yves Dauvilliers; Carol L Rosen; Chad Ruoff; Jed Black; Rupa Parvataneni; Diane Guinta; Y Grace Wang; Emmanuel Mignot Journal: J Clin Sleep Med Date: 2022-09-01 Impact factor: 4.324
Authors: Zhongxing Zhang; Yves Dauvilliers; Giuseppe Plazzi; Geert Mayer; Gert Jan Lammers; Joan Santamaria; Markku Partinen; Sebastiaan Overeem; Rafael Del Rio Villegas; Karel Sonka; Rosa Peraita-Adrados; Raphaël Heinzer; Aleksandra Wierzbicka; Birgit Högl; Mauro Manconi; Eva Feketeova; Antonio Martins da Silva; Jitka Bušková; Claudio L A Bassetti; Lucie Barateau; Fabio Pizza; Elena Antelmi; Jari K Gool; Rolf Fronczek; Carles Gaig; Ramin Khatami Journal: Nat Sci Sleep Date: 2022-05-31