Yaqiong Chai1,2,3, Chaoran Ji1,2,4, Julie Coloigner1,5, Soyoung Choi6, Melissa Balderrama7,8, Chau Vu3, Benita Tamrazi2, Thomas Coates7,8, John C Wood5,7, Sharon H O'Neil7,9,10, Natasha Lepore1,2,3,7. 1. CIBORG Laboratory, Department of Radiology, Children's Hospital Los Angeles, Los Angeles, CA, USA. 2. Department of Radiology, Children's Hospital Los Angeles, Los Angeles, CA, USA. 3. Department of Biomedical Engineering, University of Southern California, Los Angeles, CA, USA. 4. Department of Electrical Engineering, University of Southern California, Los Angeles, CA, USA. 5. Division of Cardiology, Children's Hospital Los Angeles, Los Angeles, CA, USA. 6. Neuroscience Graduate Program, University of Southern California, Los Angeles, CA, USA. 7. Department of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA. 8. Division of Hematology, Oncology, and Blood and Marrow Transplantation, Children's Hospital Los Angeles, Los Angeles, CA, USA. 9. Division of Neurology, Children's Hospital Los Angeles, Los Angeles, CA, USA. 10. The Saban Research Institute, Children's Hospital Los Angeles, Los Angeles, CA, USA.
Abstract
INTRODUCTION: Sickle cell disease (SCD) is a hereditary blood disorder in which the oxygen-carrying hemoglobin molecule in red blood cells is abnormal. SCD patients are at increased risks for strokes and neurocognitive deficit, even though neurovascular screening and treatments have lowered the rate of overt strokes. Tract-specific analysis (TSA) is a statistical method to evaluate microstructural WM damage in neurodegenerative disorders, using diffusion tensor imaging (DTI). METHODS: We utilized TSA and compared 11 major brain WM tracts between SCD patients with no history of overt stroke, anemic controls, and healthy controls. We additionally examined the relationship between the most commonly used DTI metric of WM tracts and neurocognitive performance in the SCD patients and healthy controls. RESULTS: Disruption of WM microstructure orientation-dependent metrics for the SCD patients was found in the genu of the corpus callosum (CC), cortico-spinal tract, inferior fronto-occipital fasciculus, right inferior longitudinal fasciculus, superior longitudinal fasciculus, and left uncinate fasciculus. Neurocognitive performance indicated slower processing speed and lower response inhibition skills in SCD patients compared to controls. TSA abnormalities in the CC were significantly associated with measures of processing speed, working memory, and executive functions. CONCLUSION: Decreased DTI-derived metrics were observed on six tracts in chronically anemic patients, regardless of anemia subtype, while two tracks with decreased measures were unique to SCD patients. Patients with WMHs had more significant FA abnormalities. Decreased FA values in the CC significantly correlated with all nine neurocognitive tests, suggesting a critical importance for CC in core neurocognitive processes.
INTRODUCTION:Sickle cell disease (SCD) is a hereditary blood disorder in which the oxygen-carrying hemoglobin molecule in red blood cells is abnormal. SCDpatients are at increased risks for strokes and neurocognitive deficit, even though neurovascular screening and treatments have lowered the rate of overt strokes. Tract-specific analysis (TSA) is a statistical method to evaluate microstructural WM damage in neurodegenerative disorders, using diffusion tensor imaging (DTI). METHODS: We utilized TSA and compared 11 major brain WM tracts between SCDpatients with no history of overt stroke, anemic controls, and healthy controls. We additionally examined the relationship between the most commonly used DTI metric of WM tracts and neurocognitive performance in the SCDpatients and healthy controls. RESULTS: Disruption of WM microstructure orientation-dependent metrics for the SCDpatients was found in the genu of the corpus callosum (CC), cortico-spinal tract, inferior fronto-occipital fasciculus, right inferior longitudinal fasciculus, superior longitudinal fasciculus, and left uncinate fasciculus. Neurocognitive performance indicated slower processing speed and lower response inhibition skills in SCDpatients compared to controls. TSA abnormalities in the CC were significantly associated with measures of processing speed, working memory, and executive functions. CONCLUSION: Decreased DTI-derived metrics were observed on six tracts in chronically anemicpatients, regardless of anemia subtype, while two tracks with decreased measures were unique to SCDpatients. Patients with WMHs had more significant FA abnormalities. Decreased FA values in the CC significantly correlated with all nine neurocognitive tests, suggesting a critical importance for CC in core neurocognitive processes.
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