Introduction: Pre-/post-contrast MRI obtained under anesthesia is used to evaluate and follow brain abnormalities in children with Sturge-Weber syndrome (SWS). However, anesthesia and contrast administration carry risks. We explored the feasibility and diagnostic utility of a safe non-contrast, non-sedate MRI acquisition with susceptibility-weighted imaging [SWI] in SWS children and their siblings with a wide range of cognitive and behavioral functioning.

Methods: Thirty children (15 with SWS, 15 siblings; mean age: 11.8 and 11.1 years, respectively) underwent a non-sedate 15-minute native brain MRI (3T) acquisition (SWI, T1, T2, fluid-attenuated inversion recovery) and formal neurocognitive and behavioral assessment, prospectively. Children were given extra time in the scanner, and young children were scanned in natural sleep. MRI abnormalities in the SWS group were compared to those identified on previous clinical pre-/post-contrast MRI obtained with anesthesia.

Results: Twenty-nine children (96%) completed the MRI with good (n=28) or adequate quality, including all SWS children, and all seven children <8 years of age. The SWS group had lower cognitive functions than the siblings (full-scale IQ: 85±14 vs. 108±14, p<0.001) and 11/30 children (37%; 5 SWS, 6 siblings) had clinically significant behavioral issues without an effect on the MRI success rate and quality. SWI detected both normal veins and SWS vascular abnormalities and calcifications (Figure) and, together with the other sequences, detected vascular and parenchymal abnormalities similar to or better than contrast-enhanced MRI.

Conclusion: Non-sedate, non-contrast MRI acquisition with SWI can provide high diagnostic yield in children with SWS, including those with cognitive impairment and/or behavioral concerns.

Csaba Juhasz

Professor

Pediatrics and Neurology

Wayne State University

Detroit, United States

Michael Behen

Assistant Professor

Pediatrics and Neurology

Wayne State University

Detroit, United States

Nore Gjolaj

Research ssistant

Pediatrics and Neurology

Wayne State University

Detroit, United States

Aimee Luat

Associate Professor

Pediatrics

Central Michigan University

Mt. Pleasant, United States

Yang Xuan

Research Assistant

MR Research Facility

Wayne State University

Detroit, United States

Otto Muzik

Professor

Pediatrics and Neurology

Wayne State University

Detroit, United States

Jeong-Won Jeong

Professor

Pediatrics and Neurology

Wayne State University

Detroit, United States