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From the Departments of Pediatrics, Division of Pediatric Hematology Oncology (Dr. DeBaun) and Neurology (Dr. Noetzel), and the Mallinckrodt Institute of Radiology (Dr. Siegel), Washington University School of Medicine, St. Louis, MO; the Department of Psychology (Dr. Schatz), Washington University; the Department of Radiology (Dr. Koby), National Institutes of Health, Bethesda, MD; the University of Washington (Dr. Craft), GRECC, Seattle American Lake Veterans Affairs Medical Center, Seattle; the Departments of Pediatrics, Division of Hematology/Oncology (Dr. Resar) and Radiology (Dr. Lee), the Johns Hopkins School of Medicine, Baltimore, MD; the Departments of Pediatrics, Division of Hematology/Oncology (Dr. Chu) and Radiology, Division of Pediatric Radiology (Dr. Launius), St. Louis University; and the Department of Pediatrics (Dadash-Zadeh), Sinai Hospital of Baltimore.
Address correspondence and reprint requests to Dr. Michael R. DeBaun, Department of Pediatrics, Campus Box 8116, Washington University School of Medicine, One Children's Place, St. Louis, MO 63110
Objective In children with sickle cell disease (SCD), silent cerebral infarcts are the most frequent cause of neurologic injury. We determined the sensitivity and specificity of selective neurocognitive measures when separating children with silent cerebral infarcts and SCD from sibling controls. Additionally, we tested the validity of the same cognitive measures to identify patients with overt strokes.
Methods We examined performance on a neuropsycho-logic battery containing measures of attention/executive, spatial, language, memory, and motor functioning for seven children with SCD and silent cerebral infarct, 21 children with SCD and overt stroke, and 17 normal siblings. Diagnosis of cerebral infarct was based on results of MRI.
Results Measures from the attention and executive domains were the most useful for identifying children with silent cerebral infarct. The Test of Variables of Attention was the most robust measure and yielded a sensitivity rate of 86% and a specificity rate of 81%. This measure also showed a sensitivity rate of 95% in identifying overt stroke.
Conclusions Brief cognitive screening measures, if properly constructed, may be an effective means of identifying children with silent cerebral infarct. Future prospective studies should be pursued to assess the utility of cognitive screening for silent cerebral infarcts in SCD.
Received September 24, 1997. Accepted in final form February 13, 1998.
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