Abnormalities of the central nervous system in very young children with sickle cell anemia.
OBJECTIVE
To determine whether abnormalities of the CNS are present in very young children with sickle cell anemia.


METHODS
Thirty-nine children with hemoglobin SS between the ages of 7 and 48 months were examined with magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). No child had a history of clinical stroke, although 3 had a history of seizures (2 neonatal). Twenty-one patients underwent developmental testing with the Bayley or McCarthy Scales.


RESULTS
The overall prevalence of CNS abnormalities in asymptomatic children was 4 of 36 (11%, confidence interval 3, 26%). One patient had a silent infarct observed on MRI and a stenotic lesion on MRA; 3 other patients had stenotic lesions on MRA. The 3 patients who had a history of seizures all had lesions consistent with infarcts on MRI. Of the asymptomatic patients who had psychometric testing, 1 of 18 was developmentally delayed. One of 3 with a history of seizures had mild developmental delay.


CONCLUSIONS
Very young children with sickle cell anemia (and no history of clinical stroke) have infarction in the brain and/or stenosis of major cerebral arteries, similar to those reported in older children. These findings indicate a need for larger studies to define the incidence of CNS lesions in this age group and to determine the need for early therapeutic intervention to prevent CNS sequelae of sickle cell disease.
Wang
W C
WC
Department of Hematology/Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105-2794, USA.
Langston
J W
JW
Steen
R G
RG
Wynn
L W
LW
Mulhern
R K
RK
Wilimas
J A
JA
Kim
F M
FM
Figueroa
R E
RE
D000755:Anemia, Sickle Cell / Q000150:complications*; D001921:Brain / Q000473:pathology*; D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000209:etiology*; D002539:Cerebral Arterial Diseases / Q000175:diagnosis / Q000209:etiology; D002544:Cerebral Infarction / Q000175:diagnosis / Q000209:etiology; D002652:Child Behavior; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D018730:Infant Behavior; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D011581:Psychological Tests