EXAMINER Executive Function Battery and Neurologic Morbidity in Pediatric Sickle Cell Disease

Abstract

Sickle cell disease (SCD) is blood disorder with a high risk for cerebral vascular morbidities that impact neurocognitive functioning. Specific cognitive abilities are known to be more sensitive to neurologic effects of SCD than IQ scores, yet there is little consensus about which measures to use to assess neurocognitive functioning. We evaluated the ability of the Executive Abilities: Methods and Instruments for Neurobehavioral Evaluation and Research (EXAMINER) Battery to detect neurologic effects in SCD. Thirty-two youth with SCD and sixty demographically-matched comparison youth completed the EXAMINER Battery and selected tests from the Woodcock-Johnson Tests of Cognitive Ability, 3rd edition (WJ-III). Neurologic severity was examined via clinical history for morbidities and midsagittal corpus callosum (CC) area. Results indicated cognitive performance decreased with increasing neurologic morbidity across all cognitive measures; two of four EXAMINER factors were related to CC area. The association with clinical history and midsagittal CC area appeared at least as large for the Examiner Battery scores as for the WJ-III measures. The Examiner Battery showed sensitivity to neurologic history and white matter effects in SCD; this new measure compares favorably to established measures of disease-related neurocognitive effects, but would benefit from further development.

Working Memory in Children With Neurocognitive Effects From Sickle Cell Disease: Contributions of the Central Executive and Processing Speed

“…Test–retest reliability for the battery was evaluated in 122 normal adult controls at an average interval of 25 days, which is the source of reliability data presented below (Kramer et al, 2014). Validity data for the association of factor scores with degree of neurocognitive risk in SCD was published previously with the present sample (Schatz Stancil, Katz, & Sanchez, 2014). We used the Working Memory factor and the component tests to evaluate working memory and the Cognitive Control factor to represent central executive functions.…”

“…Exclusion criteria consisted of diagnosis of a co-morbid major medical, psychiatric, or developmental condition (e.g., autism, bipolar mood disorder, cancer, intellectual disability) or cognitive/motor limitations that would prohibit the child from participating in the cognitive testing. A detailed description of the neurologic history of the SCD group was reported previously (Schatz et al, 2014). Among the 32 children with SCD, 20 children had a history of sleep apnea, 12 children had a history of abnormal cerebral blood flow on transcranial Doppler ultrasound, 16 children had cerebral vessel narrowing on magnetic resonance angiography, 15 children had cerebral vascular lesions evident on structural MRI, and 7 children had a history of overt stroke.…”

Higher executive abilities following a blood transfusion in children and young adults with sickle cell disease

“…Using tests from the NIH Toolbox, we focused on executive abilities, a cognitive domain in which individuals with SCD experience particular difficulty . Nonexecutive abilities were also evaluated to determine whether change was specific to executive abilities or more general.…”

Comparing the Executive Function Ability of Autistic and Non-autistic Adolescents with a Manualised Battery of Neuropsychological Tasks

“…There have been two studies, to our knowledge, using the NIH-EXAMINER battery with neurodevelopmental populations. In one study, with a sample of young people with sickle cell disease, performance on all individual measures within the NIH-EXAMINER battery decreased with increasing neurologic morbidity and two factor scores were related to reduced white matter area (Schatz, Stancil, Katz, & Sanchez, 2014). In a second study, children and adolescents with Attention Deficit/Hyperactivity Disorder (ADHD) have been shown to score lower on the working memory but not on the fluency or cognitive control factor scores than those without ADHD (Schreiber, Possin, Girard, & Rey-Casserly, 2014).…”