W. Wang et al., Neuropsychologic performance in school-aged children with sickle cell disease: A report from the Cooperative Study of Sickle Cell Disease, J PEDIAT, 139(3), 2001, pp. 391-397
Objectives: To compare the results of serial neuropsychologic testing in ch
ildren with sickle cell disease with the results of serial magnetic resonan
ce imaging (MRI) examinations, particularly to evaluate neuropsychologic fu
nction in die absence of overt stroke.
Study design: In the Cooperative Study of Sickle Cell Disease, serial neuro
psychologic and MRI tests were performed in 373 patients (255 with hemoglob
in SS and 118 with hemoglobin SC), 6 to 18 years of age. MRI of the brain a
nd a neuropsychologic battery that included the Wechsler Intelligence Scale
for Children (WISC-R or WISC-III) and the Woodcock-Johnson Math and Readin
g Achievement Tests were performed concurrently and repeated every 2 to 3 y
ears. A silent infarct was defined as an MRI finding of increased signal In
tensity on T-2 imaging in a patient without a history of stroke.
Results: Twenty-seven patients, all with hemoglobin SS, had overt strokes a
nd 62 had silent infarcts (52 with hemoglobin SS). Patients with hemoglobin
SS and silent infarcts had significantly lower scores for math and reading
achievement, Full-Scale IQ Verbal IQ and Performance IQ when compared with
those with normal MRI findings. In children with hemoglobin SS and normal
MRI findings, the scores for Verbal IQ math achievement, and coding (a subs
cale of Performance IQ) declined with increasing age.
Conclusions: School-aged children with sickle cell disease had compromised
neuropsychologic function in the presence of silent infarcts. In addition,
they had declines in performance in certain areas of function over time. Th
erapeutic interventions that prevent or lessen cognitive impairment are nee
ded before school entry for children with sickle cell disease.