Cognitive functioning and brain magnetic resonance imaging in children with sickle Cell disease. Neuropsychology Committee of the Cooperative Study of Sickle Cell Disease.
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.


METHODS
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.
Armstrong
F D
FD
University of Miami School of Medicine, USA.
Thompson
R J
RJ
Wang
W
W
Zimmerman
R
R
Pegelow
C H
CH
Miller
S
S
Moser
F
F
Bello
J
J
Hurtig
A
A
Vass
K
K
D000755:Anemia, Sickle Cell / Q000150:complications* / Q000235:genetics; D002544:Cerebral Infarction / Q000175:diagnosis / Q000209:etiology*; D002648:Child; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D004521:Educational Measurement; D005260:Female; D005838:Genotype; D006801:Humans; D007361:Intelligence Tests; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D015995:Prevalence