Abnormalities of the central nervous system in very young children with sickle cell anemia

W. C. Wang, J. W. Langston, R. G. Steen, L. W. Wynn, R. K. Mulbern, J. A. Wilimas, F. M. Kim, Ramon E Figueroa Ortiz

Research output: Contribution to journalArticle

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Abstract

Objective: To determine whether abnormalities of the CNS are present in very young children with sickle cell anemia. Study design: 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.

Original languageEnglish (US)
Pages (from-to)994-998
Number of pages5
JournalJournal of Pediatrics
Volume132
Issue number6
DOIs
StatePublished - Jan 1 1998
Externally publishedYes

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Sickle Cell Anemia
Central Nervous System
Magnetic Resonance Angiography
Seizures
Magnetic Resonance Imaging
Stroke
Brain Infarction
Sickle Hemoglobin
Cerebral Arteries
Psychometrics
Pathologic Constriction
Age Groups
Confidence Intervals
Incidence

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Abnormalities of the central nervous system in very young children with sickle cell anemia. / Wang, W. C.; Langston, J. W.; Steen, R. G.; Wynn, L. W.; Mulbern, R. K.; Wilimas, J. A.; Kim, F. M.; Figueroa Ortiz, Ramon E.

In: Journal of Pediatrics, Vol. 132, No. 6, 01.01.1998, p. 994-998.

Research output: Contribution to journalArticle

Wang, WC, Langston, JW, Steen, RG, Wynn, LW, Mulbern, RK, Wilimas, JA, Kim, FM & Figueroa Ortiz, RE 1998, 'Abnormalities of the central nervous system in very young children with sickle cell anemia', Journal of Pediatrics, vol. 132, no. 6, pp. 994-998. https://doi.org/10.1016/S0022-3476(98)70397-X
Wang, W. C. ; Langston, J. W. ; Steen, R. G. ; Wynn, L. W. ; Mulbern, R. K. ; Wilimas, J. A. ; Kim, F. M. ; Figueroa Ortiz, Ramon E. / Abnormalities of the central nervous system in very young children with sickle cell anemia. In: Journal of Pediatrics. 1998 ; Vol. 132, No. 6. pp. 994-998.
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