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EXAMINER executive function battery and neurologic morbidity in pediatric sickle cell disease
Jeffrey Schatz1, Melita Stancil1, Tal Katz1
1Department of Psychology, University of South Carolina, Columbia, South Carolina.
Insights
Sickle cell disease (SCD) impacts brain function. The new EXAMINER Battery effectively detects these neurocognitive effects in youth with SCD, showing promise for assessing disease-related changes.
Area of Science:
- Neuroscience
- Hematology
- Cognitive Psychology
Background:
- Sickle cell disease (SCD) poses significant risks for cerebrovascular complications, affecting neurocognitive functioning.
- Existing cognitive measures may not adequately capture the nuanced neurologic effects of SCD.
- There is a need for sensitive tools to assess neurocognitive deficits in SCD patients.
Purpose of the Study:
- To evaluate the Executive Abilities: Methods and Instruments for Neurobehavioral Evaluation and Research (EXAMINER) Battery's efficacy in detecting neurologic effects in youth with SCD.
- To compare the EXAMINER Battery's performance against established neurocognitive tests.
Main Methods:
- Thirty-two youth with SCD and 60 demographically-matched controls completed the EXAMINER Battery and Woodcock-Johnson Tests of Cognitive Ability (WJ-III).
- Neurologic severity was assessed using clinical history of morbidities and midsagittal corpus callosum (CC) area.
Main Results:
- Cognitive performance declined with increased neurologic morbidity across all tested measures.
- Two EXAMINER Battery factors correlated with CC area, indicating sensitivity to white matter integrity.
- The EXAMINER Battery demonstrated comparable or greater sensitivity to disease effects than WJ-III measures.
Conclusions:
- The EXAMINER Battery is sensitive to neurologic history and white matter changes in SCD.
- This novel tool shows favorable comparison to existing measures for assessing SCD-related neurocognitive effects.
- Further refinement of the EXAMINER Battery is recommended for optimal clinical utility.
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.

