PEDIATRICS Vol. 97 No. 6 June 1996, pp. 864-870
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Cognitive Functioning and Brain Magnetic Resonance Imaging in Children With Sickle Cell Disease

F. Daniel Armstrong PhD1, Robert J. Thompson Jr. PhD2, Winfred Wang MD3, Robert Zimmerman MD4, Charles H. Pegelow MD1, Scott Miller MD5, Franklin Moser MD6, Jacqueline Bello MD7, Anita Hurtig PhD8, and Kerstin Vass for the Neuropsychology Committee of the Cooperative Study of Sickle Cell Disease9

1 University of Miami School of Medicine Durham, North Carolina
2 Duke University Medical Center, Durham, North Carolina
3 St Jude Children's Research Hospital, Memphis, Tennessee
4 Children's Hospital of Philadelphia. New York
5 SUNY Health Sciences Center at Brooklyn, Brooklyn, New York
6 Cedar Sinai Hospital, Los Angeles, California
7 Montefiore Medical Center, Bronx, New York
8 University of Illinois, Chicago, Illinois
9 New England Research Institute, Watertown, Massachusetts

Objective. Brain magnetic resonance imaging (MRI) and neuropsychological evaluations were conducted to determine whether neuroradiographic evidence of infarct in children with sickle cell disease between ages 6 and 12 years would result in impairment in cognitive and academic functioning.

Method and Design. Children enrolled in the Cooperative Study of Sickle Cell Disease were evaluated with brain MRI and neuropsychological evaluation. Completed studies were obtained for 194 children, 135 with HbSS. MRIs were categorized according to the presence of T2-weighted, high-intensity images suggestive of infarct and were further categorized on the basis of a clinical history of cerebrovascular accident (CVA). An abnormal MRI but no clinical history of CVA was classified as a silent infarct. Neuropsychological evaluations included assessment of both global intellectual functioning and specific academic and neuropsychological functions.

Results. Central nervous system (CNS) abnormalities were identified on MRI in 17.9% of the children (22.2% of children homozygous for HbS), and a clinical history of CVA (N = 9, 4.6%) was identified in only children with HbSS disease. Subsequent analyses examined only children with HbSS. Children with a history of CVA performed significantly poorer than children with silent infarcts or no MRI abnormality on most neuropsychological evaluation measures. Children with silent infarcts on MRI performed significantly poorer than children with no MRI abnormality on tests of arithmetic, vocabulary, and visual motor speed and coordination.

Conclusions. These results substantiate the importance of careful evaluation, educational planning, and medical intervention for CNS-related complications in children with sickle cell disease.

Submitted on December 22, 1994
Accepted on June 23, 1995




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