The posterior fossa syndrome questionnaire: using science to inform practice.

INTRODUCTION: Up to 34% of patients with medulloblastoma develop posterior fossa syndrome (PFS) following brain tumor resection and have increased risk of long-term neurocognitive impairments. Lack of agreement in conceptualization and diagnosis of PFS calls for improvements in diagnostic methods. The current study aimed to describe psychometric properties of a new posterior fossa syndrome questionnaire (PFSQ). METHODS: The PFSQ was informed by prior research and developed by a multidisciplinary team with subject matter expertise. Participants (N = 164; 63.4% Male; 78.7% White; Mage at diagnosis = 10.38 years, SD = 5.09, range 3-31 years) included patients with newly diagnosed medulloblastoma enrolled in the SJMB12 clinical trial. Forty-four patients (26.8%) were classified as having PFS based on attending physician's post-surgical yes/no report. A PFSQ was completed by a neurologist within 2 weeks of coming to St. Jude Children's Research Hospital for adjuvant treatment, irrespective of suspicion for PFS. RESULTS: PFSQ items ataxia (100.00%), dysmetria (95.45%), and speech/language changes (79.55%) were most sensitive. However, ataxia (26.50%) and dysmetria (46.61%) demonstrated low specificity. Speech/language changes (81.36%), mutism (95.76%), orofacial apraxia (98.29%) and irritability (96.61%) had high specificity. A principal component analysis found four components: (1) speech/language changes, (2) apraxias (including mutism), (3) motor/oromotor, and (4) emotional lability. CONCLUSIONS: The PFSQ is a dimensional diagnostic approach that can be used to improve diagnostic consistency across clinical and research groups to help accelerate understanding of PFS etiology, identify surgical correlates of risk, predict long-term impairments, and develop targeted interventions. Additional measure validation, including correlation with symptom resolution, is required.

Adaptive style and differences in parent and child report of health-related quality of life in children with cancer.

The objective of this study was to examine the self-reported health-related quality of life (HRQL) of children with cancer, and the consistency between child and parent reports of child HRQL, as a function of the child's adaptive style. Participants included 199 children with cancer, 108 healthy children, and their parents. Children completed self-report measures of HRQL and adaptive style. Measures of adaptive style were used to categorize children as high anxious, low anxious, defensive high anxious or repressor. Parents completed measures reporting their children's HRQL. Adaptive style was a significant predictor of child-reported HRQL, particularly on the psychosocial scales, with children identified as repressors reporting the best HRQL. Adaptive style was also predictive of discrepancies between parent and child report of child HRQL. Repressor and low anxious children reported better HRQL than did their parents, while high anxious children reported poorer HRQL, regardless of health status. Adaptive style is a significant determinant of self-reported HRQL in children, particularly in psychosocial domains, while health status (i.e. cancer patient vs healthy control) is predictive only of physical health domains. Researchers and clinicians should be aware of the impact of child adaptive style when assessing HRQL outcomes using self- or parent report.

Assessment of health-related quality of life in children with cancer: consistency and agreement between parent and child reports.

BACKGROUND: In pediatric settings, measures of health-related quality of life (HRQL) are often obtained by proxy, especially from parents, but the correlation between parental report and patient report has not been clearly established. In the current study, the authors examined the agreement between child and parent reports of HRQL in children with cancer (both those receiving treatment and those off treatment) and healthy controls. METHODS: Two groups of children with cancer who differed with regard to treatment status (n = 199) and a group of healthy control children (n = 108) were assessed using a standardized measure of HRQL. Both children and parents reported on the child's HRQL using parallel forms of the Children's Health Questionnaire. RESULTS: Significant parent-child correlations were found for all 10 HRQL scales, and these correlations were higher in the cancer groups compared with controls. Parents in the cancer groups tended to underestimate the HRQL of their children compared with the child report, although there were no significant parent-child mean differences in the group of patients who were off treatment and only 2 significant differences in the group of children receiving treatment. In contrast, parents in the control group tended to overestimate the HRQL of their children, and the parent-child differences were found to be larger, achieving statistical significance on 8 of 10 scales. CONCLUSIONS: Parents and children tend to report comparable child HRQL outcomes, and this is particularly true in oncology populations. In cases in which the child is either too young or too ill to provide a self-report, parent-reported HRQL can be viewed as a reliable substitute.