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Research Identifies Need for Improved Understanding of Sickle Cell Disease in Adulthood
Date:12/8/2007

tem, including the brain and spinal cord. In children, age is associated with a decline in neuropsychological dysfunction, suggesting adults are at risk for progressive brain injury.

This trial evaluated 138 adults with sickle cell anemia and 37 control subjects with a series of cognitive tests that assessed verbal and nonverbal intelligence, academic achievement, executive functioning, processing speed, attention, and memory. Tests included the Wechsler Adult Intelligence Scale, the Wechsler Memory Scale, the Woodcock-Johnson Tests of Achievement, the Test of Everyday Attention, and others. MRI scans were also evaluated in 82 patients to evaluate brain atrophy and lesions.

The study found that neuropsychological dysfunction and/or undetected brain injury affect the majority of neurologically intact adults with sickle cell anemia, especially in areas of executive functioning, reading, and math fluency. In the study, 32 percent and 39 percent scored below 86 on the Wechsler Adult Intelligence Scale Performance IQ and Processing Speed Index scores, respectively, compared to 15 percent in national norms; 26 percent and 22 percent of patients scored below 86 on the Wechsler Memory Scale Visual Immediate and Immediate Memory scales, respectively, compared to 15 percent in national norms. Controlling for age, gender, and education, patients and controls differed significantly on the Wechsler Adult Intelligence Scale Processing Speed Index score and the Woodcock-Johnson score including reading, math, and following directions. Sub-tests of the Test of Everyday Attention -- in attention and flexibility of thought -- showed significant decrease in performance with age in sickle cell patients, but not in controls.

In 82 patients with MRIs, 63 percent had an abnormal MRI or neuropsychological dysfunction, 38 percent had atrophy and/or ischemic lesions, and 18 percent had ischemic lesions only. Low Performance IQ and Processing Speed Index were signifi
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SOURCE American Society of Hematology
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