Document Detail

Brain injury in children with sickle cell disease: prevalence and etiology.
MedLine Citation:
PMID:  14595645     Owner:  NLM     Status:  MEDLINE    
Our objective was to evaluate the relationship between brain injury by magnetic resonance imaging (MRI) and vasculopathy by magnetic resonance angiography (MRA) in children with hemoglobin SS, the most serious form of sickle cell disease. We reviewed imaging for all 146 SS patients imaged at St. Jude Children's Research Hospital since 1993. Standard MRI criteria were used to identify cystic infarction, leukoencephalopathy, encephalomalacia, or atrophy. Standard MRA criteria were used to identify arterial tortuousity (limited vasculopathy), and stenosis or occlusion (extensive vasculopathy). At an average age of 10 years, the estimated prevalence of infarction, ischemic damage, or atrophy in SS patients was 46%, and of vasculopathy was 64%. Only 28% of patients were normal by both modalities, and patients abnormal by MRA often were abnormal by MRI (p < 0.00001). Patients with cystic infarction had limited vasculopathy, whereas patients with encephalomalacia had stenosis or occlusion (p < 0.0001). Large arteries were affected in 31% of brain injury patients, whereas small arteries are inferred to be abnormal in up to 69% of patients with brain injury. The degree of vasculopathy is closely related to the degree of brain injury, implying that vasculopathy is prodromal to most forms of brain injury in hemoglobin SS.
R Grant Steen; Xiaoping Xiong; James W Langston; Kathleen J Helton
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Annals of neurology     Volume:  54     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  2003 Nov 
Date Detail:
Created Date:  2003-11-03     Completed Date:  2003-12-10     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  564-72     Citation Subset:  IM    
Department of Diagnostic Imaging, St. Jude Children's Research Hospital, Memphis, TN, USA.
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MeSH Terms
Age Factors
Anemia, Sickle Cell / complications*
Arteries / pathology
Brain / blood supply*
Brain Diseases / epidemiology*,  etiology*,  radiography
Cerebrovascular Circulation
Child, Preschool
Magnetic Resonance Angiography
Magnetic Resonance Imaging
Retrospective Studies
Grant Support
Comment In:
Ann Neurol. 2003 Nov;54(5):559-63   [PMID:  14595644 ]

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