The neuropsychological profile of children with Diffuse Intrinsic Pontine Glioma (DIPG) before and after radiation therapy: A prospective longitudinal study

ABSTRACT

Children with Diffuse Intrinsic Pontine Gliomas (DIPG), a malignant brainstem tumor, experience poor prognosis. Because of the disease’s rarity and highly aggressive course, there is a dearth of research on cognitive and psychosocial outcomes in this underserved, vulnerable population. However, evaluating effects of the disease and treatment on the cognitive and daily functioning of these patients is important to better understand their specific needs and improve their quality of life. The current longitudinal study administered prospective neuropsychological assessments to children diagnosed with CNS malignancies, including the largest sample of children with DIPG to date (n = 21, mean age = 7.86 years, range = 3–16) in neurocognitive, behavioral, social-emotional, and adaptive functioning at baseline, two weeks post-radiation, and six months later. The results describe population-based, cross-sectional characteristics and within-patient longitudinal changes. Prior to radiation, children with DIPG exhibited significant weaknesses compared to normative samples in both parent-report and performance-based measures of attention, and tests of processing speed and verbal learning/memory. Younger children demonstrated poorer inhibitory control on performance tests and worse parent-reported behavioral regulation, depression, and social withdrawal compared to older children. Six-months post-radiation, older children exhibited poorer socialization than younger children. Longitudinally, children with DIPG exhibited short-term improvements immediately post-radiation in performance-based attention tests and parent-reported behavior, including attention, hyperactivity, behavioral regulation, and executive function. However, these improvements did not persist and significant decline was documented on tests of attention by six months. Clinical implications for professionals working with children with DIPG and recommendations for cognitive remediation and quality of life interventions are provided.

KEYWORDS:

• DIPG
• pediatric brain tumor
• radiation therapy
• neuropsychological functioning
• quality of life

Disclosure statement

No potential conflict of interest was reported by the author(s).

Additional information

Funding

The authors thank the patients and caregivers in this study. This research was supported by the Intramural Research Program of the National Institutes of Health, National Cancer Institute. This project has been funded in whole or in part with federal funds from the National Cancer Institute, National Institutes of Health, under Contract No. 75N91019D00024. The content of this publication does not necessarily reflect the views or policies of the Department of Health and Human Services, nor does mention of trade names, commercial products, or organizations imply endorsement by the U.S. Government.