Risk For Neuropsychological Deficits Research Articles

B - 101 Associations between Oxygen Saturation and Neurocognitive Performance in Sickle Cell Disease

Abstract Objective Patients with sickle cell disease (SCD) are at risk for neuropsychological deficits. Studies have documented that peripheral oxygen saturation is associated with neurocognitive performance, but the results are inconsistent. Our objective was to examine the associations between oxygen saturation and neurocognitive performance in SCD using several different measurement techniques. Methods A lifetime cohort study of 413 patients ranging from 8–25 years of age (Mean = 14.18, Standard Deviation = 4.81) diagnosed with SCD (60% HbSS) received a neuropsychological evaluation including measures of intelligence, processing speed, and working memory. Analyses were conducted separately based on SCD genotype (HbSS/Sβ0 vs. HbSC/SB+). Oxygen saturation values were examined as predictors of neurocognitive performance based on 1) mean lifetime value, 2) lifetime standard deviation, 3) most recent value (within 3 months from neuropsychological assessment), and 4) change over time. Measurement types were entered into separate multivariable models covarying for age, sex, socioeconomic status and hydroxyurea usage. Results Among patients diagnosed with HbSS/Sβ0genotypes, the lifetime standard deviation in oxygen saturation was associated with working memory (pFDR = 0.004) and the change over time in oxygen saturation was associated with processing speed (pFDR <0.001). There were no associations between oxygen saturation and neurocognitive performance among patients with the SC/SB+ genotypes. Conclusions Associations between oxygen saturation and neurocognitive performance in SCD differ based on when the values are collected and how they are analyzed. Our analyses suggest that the change over time and variability in oxygen saturation is most predictive of neurocognitive performance.

Predictors of Cognitive Performance Among Infants Treated for Brain Tumors: Findings From a Multisite, Prospective, Longitudinal Trial.

Infants treated for CNS malignancies experience a significantly poorer response to treatment and are particularly at risk for neuropsychological deficits. The literature is limited and inconsistent regarding cognitive outcomes among this group. We investigated predictors of cognitive outcomes in children treated for brain tumors during infancy as part of a large, prospective, multisite, longitudinal trial. One hundred thirty-nine infants with a newly diagnosed CNS tumor were treated with chemotherapy, with or without focal proton or photon radiation therapy (RT). Cognitive assessments were conducted at baseline, 6 months, 1 year, and then annually for 5 years. The median length of follow-up was 816 days (26.8 months). Neurocognitive testing included assessment of intellectual functioning (intellectual quotient [IQ]), parent ratings of executive functioning and emotional and behavioral functioning, and socioeconomic status. At baseline, IQ, parent-reported working memory, and parent-reported adaptive functioning were worse than normative expectations. Baseline cognitive difficulties were associated with younger age at diagnosis and lower socioeconomic status. Linear mixed models did not demonstrate a decline in IQ over time. There were increased parent-reported attention and executive problems over time. Increased concerns were related to supratentorial tumor location and CSF diversion. There were no differences in cognitive outcomes based on treatment exposure (chemotherapy-only v chemotherapy with RT and proton v photon focal RT). Even before adjuvant therapy, young children with brain tumors experience cognitive difficulties that can affect quality of life. Changes in cognitive functioning over time were dependent on tumor location and surgical factors rather than adjuvant therapy. These findings may serve to guide treatment planning and indicate targets for cognitive monitoring and intervention.

Neurocognitive functioning in symptomatic adults with sickle cell disease: A description and comparison with unaffected siblings

ABSTRACT Children and adults with sickle cell disease (SCD) are at risk for neuropsychological deficits; however, the neurocognitive functioning of adults with SCD and related comorbidities has not been widely reported in the literature. We examined specific cognitive domains in symptomatic adults with SCD and compared them with their unaffected siblings. We also examined relationships between cognitive scores, patient-reported outcomes (PROs), and medical/laboratory values. Thirty patient-sibling pairs (M patient age = 32.5 years, M sibling age = 32.1 years) completed evaluations as part of a medical clinical trial (NCT00061568). All patient and sibling neurocognitive test scores were within normal limits. Patients scored significantly lower (M = 91.0 ± 11.3) than their siblings (M = 100.6 ± 12.3; t = −3.5, p < .01) on the Wechsler Processing Speed Index. They also indicated more problems than siblings on an executive functioning questionnaire, although these differences were nonsignificant after accounting for depressive symptoms. Higher fetal hemoglobin and lower creatinine correlated with better scores on particular cognitive and PRO measures. In summary, our sample of adults with symptomatic SCD demonstrated worse processing speed and experience more executive challenges than their siblings, despite treatment with hydroxyurea. These relative weakness likely relate to disease processes but the specific physiological mechanism is unclear.