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

Creator(s)

Anne T Berg
Keith A. Coffman, Children's Mercy HospitalFollow
Deborah Gaebler-Spira

Document Type

Article

Publication Date

12-2021

Identifier

DOI: 10.1111/dmcn.14990

Abstract

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 < 0.001). The number of functional impairment domains and of autonomic symptom groups were correlated (Spearman's r=0.35, p < 0.001) on bivariate and multivariable analysis adjusted for DEE group and age. Parental stress was also independently correlated with dysautonomias (p < 0.001).

Interpretation: Parent-reported dysautonomias are common in children with DEEs. They correlate with extent of functional impairment and may contribute to caregiver stress. What this paper adds Dysautonomic symptoms are common in young people with developmental and epileptic encephalopathies (DEEs). Burden of dysautonomias is strongly correlated with burden of functional impairments. Aspects of dysautonomic function may provide biomarkers of DEE disease severity.

Journal Title

Developmental medicine and child neurology

Volume

63

Issue

12

First Page

1433

Last Page

1440

Comments

Grant support

• Stanley Manne Children's Research Institute and Ann & Robert H Lurie Children's Hospital of Chicago under the Precision Medicine Strategic Research Initiative
• Pediatric Epilepsy Research Foundation

Recommended Citation

Berg AT, Coffman K, Gaebler-Spira D. Dysautonomia and functional impairment in rare developmental and epileptic encephalopathies: the other nervous system. Dev Med Child Neurol. 2021;63(12):1433-1440. doi:10.1111/dmcn.14990