• Natasha A Choudhury, Michael R DeBaun, Mark Rodeghier, Allison A King, John J Strouse, and Robert C McKinstry.
• From the School of Medicine (N.A.C.), Meharry Medical College; Department of Pediatrics (N.A.C., M.R.D.), Vanderbilt-Meharry Center of Excellence in Sickle Cell Disease, Vanderbilt University Medical Center, Nashville, TN; Rodeghier Consultants (M.R.), Chicago, IL; Program in Occupational Therapy and Department of Pediatrics Hematology/Oncology (A.A.K.) and Pediatric Radiology and Neuroradiology Sections (R.C.M.), Washington University School of Medicine, St. Louis, MO; and Department of Pediatrics and Medicine (J.J.S.), Division of Hematology, Johns Hopkins University School of Medicine, Baltimore, MD.
• Neurology. 2018 Jan 16; 90 (3): e239-e246.

ObjectiveTo evaluate whether application of the adult definition of silent cerebral infarct (SCI) (T2-weighted hyperintensity ≥5 mm with corresponding T1-weighted hypointensity on MRI) is associated with full-scale IQ (FSIQ) loss in children with sickle cell anemia (SCA), and if so, whether this loss is greater than that of the reference pediatric definition of SCI (T2-weighted hyperintensity ≥3 mm in children on MRI; change in FSIQ -5.2 points; p = 0.017; 95% confidence interval [CI] -9.48 to -0.93).MethodsAmong children with SCA screened for SCI in the Silent Cerebral Infarct Transfusion trial, ages 5-14 years, a total of 150 participants (107 with SCIs and 43 without SCIs) were administered the Wechsler Abbreviated Scale of Intelligence. A multivariable linear regression was used to model FSIQ in this population, with varying definitions of SCI independently substituted for the SCI covariate.ResultsThe adult definition of SCI applied to 27% of the pediatric participants with SCIs and was not associated with a statistically significant change in FSIQ (unstandardized coefficient -3.9 points; p = 0.114; 95% CI -8.75 to 0.95), with predicted mean FSIQ of 92.1 and 96.0, respectively, for those with and without the adult definition of SCI.ConclusionsThe adult definition of SCI may be too restrictive and was not associated with significant FSIQ decline in children with SCA. Based on these findings, we find no utility in applying the adult definition of SCI to children with SCA and recommend maintaining the current pediatric definition of SCI in this population.Copyright © 2017 American Academy of Neurology.

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