Inflammatory Brain diseases (IBrainD) are increasingly recognized causes of devastating neurological deficits in previously healthy children. Although the mortality has dramatically improved, disease and treatment related morbidity and impact on health-related quality of life (HRQoL) remain unclear. Therefore the purpose of this study was to determine the HRQoL in children with IBrainD and identify factors at diagnosis associated poor HRQoL.

Methods:

A multicenter, observational cohort study of children diagnosed with IBrainD at all participating sites of the BrainWorks network was conducted. Children age <18 years at time of diagnosis, who were followed for at least 12 months were included. HRQoL was measured using the Pediatric Quality of Life Inventory Version 4.0 (PedsQL) Generic Core Scales. The total PedsQL score and the physical and psychosocial subdomains were assessed. The relationship between the parent’s perceived HRQoL of the child and the child’s perceived HRQoL were explored. Independent variables evaluated included diagnosis, age at diagnosis, gender, time to diagnosis, presence of clinical symptoms at diagnosis, baseline disease activity as rated on the Physician’s Global Assessment analog scale and neurological functioning at 1 year. The baseline clinical symptoms considered included seizures, hemiparesis and cognitive/behavioural dysfunction. Outcome: Impaired HRQoL as defined by PedsQL. Analyses of trends were performed using regression models adjusted for repeated measures.

Results:

140 patients were included in the study. The average age at diagnosis in this cohort was 9.8 years (Range= 0.4-18.4). Angiography-negative (small vessel) childhood primary angiitis of the CNS was the most common diagnosis. Statistically significant improvements in total PedsQL scores were associated with the absence of seizures (p<0.01) and the absence of cognitive and behavioural dysfunction at baseline (p<0.01). Increases in the physical functioning subdomain score (p<0.01) and the psychosocial subdomain score (p<0.01) were observed if seizures were absent. In the absence of cognitive and behavioural dysfunction, improvement was seen in the psychosocial subdomain score (p<0.01). Gender (p=0.36) or presence of hemiparesis (p=0.45) showed no difference in the total PedsQL score. Identical prognostic factors were found in the parent PedsQl as for the child’s PedsQL scores.

Conclusion:

Children with IBrainD presenting with seizures and cognitive dysfunction at time of diagnosis were at highest risk for poor HRQoL over time. Tight seizure control and early cognitive rehabilitation are mandatory. Additional resources should therefore be considered and allocated to children presenting with these symptoms, including extended rehabilitative services and counselling focused on improving psychosocial HRQoL.