Increased muscle activity during sleep and more RBD symptoms in H1N1-(Pandemrix)-vaccinated narcolepsy type 1 patients compared with their non-narcoleptic siblings

• Published in: Sleep (New York, N.Y.) Vol. 46; no. 3; p. 1
• Main Authors: Viste, Rannveig, Follin, Louise F, Kornum, Birgitte R, Lie, Benedicte A, Viken, Marte K, Thorsby, Per M, Rootwelt, Terje, Christensen, Julie A E, Knudsen-Heier, Stine
• Format: Journal Article
• Language: English
• Published: US Oxford University Press 09.03.2023
• Subjects: Analysis; Histocompatibility antigens; HLA histocompatibility antigens; Humans; Immunization; Influenza A Virus, H1N1 Subtype; Lamotrigine; Medical research; Medicine, Experimental; Muscle function; Muscle, Skeletal; Narcolepsy; Narcolepsy - diagnosis; Narcolepsy - etiology; Neurological Disorders; Orexins; Risk factors; Siblings; Sleep; Sleep disorders; Vaccination; Norway; dream enactment; post-H1N1 NT1; periodic leg movements; leg movements in sleep; post Pandemrix NT1; disease continuum in first-degree relatives; Narcolepsy type 1; NT1; muscle activity in sleep; CSF hypocretin-1 deficiency; REM sleep behavior disorder
• ISSN: 0161-8105; 1550-9109
• DOI: 10.1093/sleep/zsac316

Abstract Study Objectives Narcolepsy type 1 (NT1) is characterized by unstable sleep-wake and muscle tonus regulation during sleep. We characterized dream enactment and muscle activity during sleep in a cohort of post-H1N1 NT1 patients and their siblings, and analyzed whether clinical phenotypic characteristics and major risk factors are associated with increased muscle activity. Methods RBD symptoms and polysomnography m. tibialis anterior electromyographical signals [long (0.5–15 s); short (0.1–0.49 s)] were compared between 114 post-H1N1 NT1 patients and 89 non-narcoleptic siblings. Association sub-analyses with RBD symptoms, narcoleptic symptoms, CSF hypocretin-1 levels, and major risk factors [H1N1-(Pandemrix)-vaccination, HLA-DQB1*06:02-positivity] were performed. Results RBD symptoms, REM and NREM long muscle activity indices and REM short muscle activity index were significantly higher in NT1 patients than siblings (all p < 0.001). Patients with undetectable CSF hypocretin-1 levels (<40 pg/ml) had significantly more NREM periodic long muscle activity than patients with low but detectable levels (40–150 pg/ml) (p = 0.047). In siblings, REM and NREM sleep muscle activity indices were not associated with RBD symptoms, other narcolepsy symptoms, or HLA-DQB1*06:02-positivity. H1N1-(Pandemrix)-vaccination status did not predict muscle activity indices in patients or siblings. Conclusion Increased REM and NREM muscle activity and more RBD symptoms is characteristic of NT1, and muscle activity severity is predicted by hypocretin deficiency severity but not by H1N1-(Pandemrix)-vaccination status. In the patients’ non-narcoleptic siblings, neither RBD symptoms, core narcoleptic symptoms, nor the major NT1 risk factors is associated with muscle activity during sleep, hence not indicative of a phenotypic continuum. Graphical Abstract Graphical Abstract