ABNORMALITIES OF THE CENTRAL-NERVOUS-SYSTEM IN VERY YOUNG-CHILDREN WITH SICKLE-CELL-ANEMIA

Objective: To determine whether abnormalities of the CNS are present i n very young children with sickle cell anemia. Study design: Thirty-ni ne children with hemoglobin SS between the ages of 7 and 48 months wer e examined with magnetic resonance imaging (MRI) and magnetic resonanc e angiography (MRA). No child had a history of clinical stroke, althou gh 3 had a history of seizures (2 neonatal). Twenty-one patients under went 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 sile nt infarct observed on MRI and a stenotic lesion on MRA; 3 other patie nts had stenotic lesions on MRA. The 3 patients who had a history of s eizures all had lesions consistent with infarcts on MRI. Of the asympt omatic patients who had psychometric testing, 1 of 18 was developmenta lly delayed. One of 3 with a history of seizures had mild developmenta l delay. Conclusions: Very young children with sickle cell anemia (and no history of clinical stroke) have infarction in the brain and/or st enosis 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 ne ed for early therapeutic intervention to prevent CNS sequelae of sickl e cell disease.