We examined the nature and extent of neuropsychologic impairment in ch
ildren with sickle cell anemia (SCA) with or without stroke. Twenty-ni
ne children with SCA received cranial magnetic resonance imaging. Stro
kes were classified into three groups: diffuse cortical stroke (n = 11
), anterior stroke (n = 6), or none (n = 12). Children with SCA and 20
age-matched sibling control subjects then received a neurologic exami
nation and a neuropsychologic battery of tests that included motor, ve
rbal, spatial, attentional, and memory measures. Tests of spatial func
tion showed that children with diffuse cortical strokes were impaired,
whereas children with anterior lesions had intrusions of irrelevant m
aterial during list recall. There were no significant differences betw
een children with stroke and sibling control subjects on motor, verbal
, or memory measures. Six children had evidence of stroke on magnetic
resonance imaging without any history of a damaging neurologic event.
These children had impaired neuropsychologic performance relative to t
hat of sibling control subjects in a pattern similar to that of childr
en with overt stroke. Children with SCA without stroke did not differ
from sibling control subjects on any measure. Our results indicate tha
t overt and silent strokes result in lesion-specific neuropsychologic
deficits in children with SCA.