Dysautonomia and functional impairment in rare developmental and epileptic encephalopathies: the other nervous system

• Published in: Developmental medicine and child neurology 2021-12, Vol.63 (12), p.1433-1440
• Main Authors: Berg, Anne T, Coffman, Keith, Gaebler‐Spira, Deborah
• Format: Article
• Language: English
• Subjects: Adolescent; Autonomic Nervous System - physiopathology; Child; Child, Preschool; Cross-Sectional Studies; Epilepsy - physiopathology; Female; Heart Rate - physiology; Humans; Male; Primary Dysautonomias - physiopathology; Young Adult
• ISSN: 0012-1622; 1469-8749
• DOI: 10.1111/dmcn.14990

Aim To determine whether functional impairments and autonomic symptoms are correlated in young people with developmental and epileptic encephalopathies (DEEs). Method Cross‐sectional, online surveys (2018–2020) of parents recruited from family groups obtained information on several aspects of children’s conditions including functional abilities (mobility, hand use, eating, and communication), 18 autonomic symptoms in six groups (cardiac, respiratory, sweating, temperature, gastrointestinal, and other), and parental stress. Bivariate and multivariable logistic regression analyses examined associations of dysautonomias with functional impairment, adjusted for type of DEE and age. Results Of 313 participants with full information on function and dysautonomias, 156 (50%) were females. The median age was 8 years (interquartile range 4–12y); 255 (81%) participants had symptoms in at least one autonomic symptom group; 283 (90%) had impairment in at least one functional domain. The number of functional impairment domains and of autonomic symptom groups varied significantly across DEE groups (both p