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Quantitative Analysis of Cerebral Cortical Atrophy and Correlation With Clinical Severity in Unilateral Sturge-Weber Syndrome

Departments of Neurology, Johns Hopkins University, Department of Pediatrics, Johns Hopkins University

Laura A. Hatfield, MD

Departments of Neurology, Johns Hopkins University, Department of Pediatrics, Johns Hopkins University

Doris D. M. Lin, MD, PhD

Department of Radiology, Johns Hopkins University, Baltimore, MD

Anne M. Comi, MD

Departments of Neurology, Johns Hopkins University, Department of Pediatrics, Johns Hopkins University, acomi{at}jhmi.edu.

Sturge-Weber syndrome is a neurocutaneous disorder with vascular malformations of the skin, brain, and eye. The objective of this study was to determine whether cortical atrophy in patients with Sturge-Weber syndrome correlates with clinical severity. Eighteen subjects (age 4 months—35 years) with unilateral Sturge-Weber syndrome received a neurologic examination and submitted previous magnetic resonance imaging (MRI) films. A blinded investigator assigned clinical severity scores based on seizures, hemiparesis, visual field cut, and cognitive impairments. Computer-aided analysis of MRIs produced laterality scores for cortical volume asymmetry. A significant relationship existed between clinical severity and laterality scores (Spearman's rho = —0.804; P < .001). Laterality scores also correlated well with hemiparesis subscores and weakly with cognitive impairment subscores (Kendall's tau b; P < .05). Using this simple, computer-aided analysis, cortical volume asymmetry correlated with clinical status. This method offers the advantages of relative simplicity, objectivity, and wide applicability to films from outside institutions, as would be encountered in clinical practice. (J Child Neurol 2005;20:867—870).

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