Abnormalities of the central nervous system in very young children with sickle cell anemia☆,☆☆,★,★★
Section snippets
PATIENTS
Thirty-nine young children with Hemoglobin SS followed up by the Mid-South Sickle Cell Center were studied, including 36 who were neurologically asymptomatic. The protocol was approved by the Institutional Review Board of St. Jude Children's Research Hospital, and informed consent was obtained from a parent or guardian of each patient; the consent included permission for oral sedation. Patient selection was based primarily on proximity of the patient's home to the site of the study and on the
Conventional MRI
All MRI procedures was performed by using a 1.5 Tesla Magnetom SP63 machine (Siemens Medical Systems, Iselin, N.J.) with a standard Siemens head coil. A conventional Tl-weighted MR image set was acquired in the transverse plane with parameters as follows: TR/TE = 266/6 msec; 23 cm field of view; 90° flip angle; 192 × 256 matrix; 19 slices with slice thickness 5 mm; three acquisitions in a total imaging time of 2 minutes 36 seconds. A conventional T2-weighted turbo spin-echo image set was
RESULTS
Overall, 4 (11%) of the 36 asymptomatic patients examined (95% confidence interval 3% to 26%) had abnormal findings on MRI (Table). Patient 1 had small scattered areas of leukomalacia primarily in the right temporo-occipital area (Fig. 1) and had mild to moderate stenosis of the right anterior cerebral artery. Patient 2 had severe stenosis of the supraclinoid portion of the internal
DISCUSSION
We found MRI and MRA abnormalities in a group of children with sickle cell disease and no history of overt CNS events. They had a median age of 18 months and therefore comprised a substantially younger population than any previously described. The prevalence of CNS imaging abnormalities was 11%, although the limited number of patients and the wide confidence interval indicate that this is only a rough estimate of frequency. Four patients had stenotic lesions visible on MRA, including one who
Acknowledgements
We are indebted to Rhonda Dancy, Sylvia Harris, Gloria Brunson, and Sara Day from the MSSCC and to the Diagnostic Imaging and Behavioral Sciences Departments for their contributions to the performance of patient examinations. We thank Patsy Burnside for excellent word processing help and Dr. Xiaoping Xiong for assistance with statistical analysis.
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Cited by (92)
Neurologic complications in children under five years with sickle cell disease
2019, Neuroscience LettersSickle Cell Disease and Stroke
2019, Pediatric NeurologyCitation Excerpt :Although the average age of onset of overt stroke is 7.7 years, SCIs may occur in infants and preschoolers. Even in the absence of history of clinical stroke, very young children have a high rate of infarction and/or stenosis of the major cerebral arteries.13–15 In a study of 36 children younger than 48 months, four (11%) had abnormalities including one with SCI and four with stenotic lesions of the MCA.13
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From the Departments of Hematology/Oncology, Diagnostic Imaging, and Behavioral Sciences, St. Jude Children's Research Hospital, Memphis, Tennessee; the Departments of Pediatrics and Radiology, the University of Tennessee, Memphis; and the Department of Radiology, the Medical College of Georgia, Augusta, Georgia.
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Supported by American-Lebanese-Syrian Associated Charities (ALSAC).
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Reprint requests: Winfred C. Wang, MD, Department of Hematology/Oncology, St. Jude Children's Research Hospital, 332 North Lauderdale, Memphis, TN 38105-2794.
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0022-3476/98/$5.00 + 0 9/21/89716