OBJECTIVE: To quantify the impact of inflammatory brain diseases in the pediatric population on health-related quality of life, including the subdomains of physical, emotional, school and social functioning. METHODS: This was a multicenter, observational cohort study of children (< 18 years of age) diagnosed with inflammatory brain disease (IBrainD). Patients were included if they had completed at least one Health Related Quality of Life Questionnaire (HRQoL). HRQoL was measured using the Pediatric Quality of Life Inventory Version 4.0 (PedsQL) Generic Core Scales, which provided a total score out of 100. Analyses of trends were performed using linear regression models adjusted for repeated measures over time. RESULTS: In this study, 145 patients were included of which 80 (55%) were females. Cognitive dysfunction was the most common presenting symptoms (63%), and small vessel childhood primary angiitis of the CNS was the most common diagnosis (33%). The mean child's self-reported PedsQL total score at diagnosis was 68.4, and the mean parent's proxy-reported PedsQL score was 63.4 at diagnosis. Child's self-reported PedsQL scores reflected poor HRQoL in 52.9% of patients at diagnosis. Seizures or cognitive dysfunction at presentation was associated with statistically significant deficits in HRQoL. CONCLUSION: Pediatric IBrainD is associated with significantly diminished health-related quality of life. Future research should elucidate why these deficits occur and interventions should focus on improving HRQoL in the most affected subdomains, in particular for children presenting with seizures and cognitive dysfunction.

Akron Children's Hospital Akron OH USA

Alberta Children's Hospital Research Institute Calgary AB Canada

BC Children's Hospital Vancouver BC Canada

Benioff Children's Hospital University of California San Francisco California USA

Boston's Children Hospital Boston MA USA

Charles University Prague Prague Czech Republic

Children's Hospital at Montefiore Bronx New York USA

Cumming School of Medicine University of Calgary Calgary AB Canada

Department of Medical Imaging and Department of Statistical Sciences University of Toronto Toronto ON Canada

Department of Rheumatology Hospital for Sick Children Toronto ON Canada

Duke Children's Hospital and Health Centre Durham North Carolina USA

Institute of Neuroscience Newcastle University Newcastle upon Tyne UK

IWK Health Centre and Dalhousie University Halifax NS Canada

Joseph M Sanzari Children's Hospital Hackensack NJ USA

Mother and Child Health Care Institute of Serbia Belgrade Serbia

Rheumatology Department of Pediatrics Alberta Children's Hospital 2888 Shaganappi Trail NW Calgary AB T3B 6A8 Canada

School of Medicine Queen's University Kingston ON Canada

Texas Children's Hospital Houston TX USA

See more in PubMed

Twilt M, Benseler SM. Childhood inflammatory brain diseases: pathogenesis, diagnosis and therapy. Rheumatology (Oxford) 2014;53(8):1359–1368. doi: 10.1093/rheumatology/ket398. PubMed DOI

Twilt M, Benseler SM. CNS vasculitis in children. Mult Scler Relat Disord. 2013;2(3):162–171. doi: 10.1016/j.msard.2012.11.002. PubMed DOI

Cellucci T, Benseler SM. Central nervous system vasculitis in children. Curr Opin Rheumatol. 2010;22(5):590–597. doi: 10.1097/BOR.0b013e32833c723d. PubMed DOI

Bigi S, Hladio M, Twilt M, Dalmau J, Benseler SM. The growing spectrum of antibody-associated inflammatory brain diseases in children. Neurol Neuroimmunol Neuroinflamm. 2015;2(92):1–9. PubMed PMC

Twilt M, Benseler SM. The spectrum of CNS vasculitis in children and adults. Nat Rev Rheumatol. 2012;8:97–107. doi: 10.1038/nrrheum.2011.197. PubMed DOI

Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng XY, Lai MZ, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008;7(12):1091–1098. doi: 10.1016/S1474-4422(08)70224-2. PubMed DOI PMC

Cellucci T, Tyrrell PN, Twilt M, Sheikh S, Benseler SM. Distinct phenotype clusters in childhood inflammatory brain diseases: implications for diagnostic evaluation. Arthritis Rheumatol. 2014;66(3):750–756. doi: 10.1002/art.38274. PubMed DOI

de Wit M, Hajos T. Health-related quality of life. In: Gellman MD, Turner JR, editors. Encyclopedia of behavioral medicine. New York, NY: Springer New York; 2013. pp. 929–931.

Varni JW, Burwinkle TM, Seid M, Skarr D. The PedsQL 4.0 as a pediatric population health measure: feasibility, reliability, and validity. Ambul Pediatr. 2003;3(6):329–341. doi: 10.1367/1539-4409(2003)003<0329:TPAAPP>2.0.CO;2. PubMed DOI

Eiser C. Morse R. a review of measures of quality of life for children with chronic illness. Arch Dis Child. 2001;84:205–211. doi: 10.1136/adc.84.3.205. PubMed DOI PMC

Kitchen L, Westmacott R, Friefeld S, MacGregor D, Curtis R, Allen A, et al. The pediatric stroke outcome measure: a validation and reliability study. Stroke. 2012;43(6):1602–1608. doi: 10.1161/STROKEAHA.111.639583. PubMed DOI

Varni JW, Seid M, Smith Knight T, Burwinkle T, Brown J, Szer IS. The PedsQL in pediatric rheumatology: reliability, validity, and responsiveness of the pediatric quality of life inventory generic Core scales and rheumatology module. Arthritis Rheum. 2002;46(3):714–725. doi: 10.1002/art.10095. PubMed DOI

Huang IC, Thompson LA, Chi YY, Knapp CA, Revicki DA, Seid M, et al. The linkage between pediatric quality of life and health conditions: establishing clinically meaningful cutoff scores for the PedsQL. Value Health. 2009;12(5):773–781. doi: 10.1111/j.1524-4733.2008.00487.x. PubMed DOI PMC

Kim J, Chung H, Amtmann D, Salem R, Park R, Askew RL. Symptoms and quality of life indicators among children with chronic medical conditions. Disabil Health J. 2014;7(1):96–104. doi: 10.1016/j.dhjo.2013.08.007. PubMed DOI PMC

Lundberg V, Lindh V, Eriksson C, Peterson S, Eurenius E. Health-related quality of life in girls and boys with juvenile idiopathic arthritis: self- and parental reports in a cross-sectional study. Pediatr Rheumatol. 2012;10(33):1–8. PubMed PMC

MacAllister WS, Christodoulou C, Troxell R, Milazzo M, Block P, Preston TE, et al. Fatigue and quality of life in pediatric multiple sclerosis. Mult Scler. 2009;15(12):1502–1508. doi: 10.1177/1352458509345902. PubMed DOI

Russell K, Selci E, Chu S, Fineblit S, Ritchie L, Ellis M. Longitudinal assessment of health-related quality of life following adolescent sports-related concussion. J Neurotrauma. 2017;34(13):2147–2153. doi: 10.1089/neu.2016.4704. PubMed DOI

Ilmer EC, Lambregts SA, Berger MA, de Kloet AJ, Hilberink SR, Roebroeck ME. Health-related quality of life in children and youth with acquired brain injury: two years after injury. Eur J Paediatr Neurol. 2016;20(1):131–139. doi: 10.1016/j.ejpn.2015.09.003. PubMed DOI

Benseler SM. Central nervous system Vasculitis in children. Curr Rheumatol Rep. 2006;8:442–449. doi: 10.1007/s11926-006-0040-4. PubMed DOI

Sprangers MAG, Scwartz CE. Integrating response shift into health-related quality of life research: a theoretical model. Soc Sci Med. 1999;48:1507–1515. doi: 10.1016/S0277-9536(99)00045-3. PubMed DOI