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Abstract Details

MRI-Based Quantitative Assessment of Brain Atrophy in Sturge-Weber Syndrome
General Neurology
P3 - Poster Session 3 (11:45 AM-12:45 PM)
2-010
To evaluate the correlation between quantitative brain atrophy measurements derived from imaging data and clinical subjective atrophy scores by physicians in patients with Sturge-Weber Syndrome (SWS). By achieving this goal, we may improve the diagnosis and treatment of SWS by providing a more objective and quantitative assessment of brain atrophy.
Sturge-Weber Syndrome (SWS) is a rare genetic condition characterized by a facial port-wine stain and can lead to significant neurological complications, including seizures, cognitive impairment, and structural brain abnormalities. Despite its impact, there remains a lack of robust quantitative measures to accurately evaluate the extent of structural brain abnormalities in SWS patients.

We conducted cortical surface extraction on high-resolution MRI brain scans from 5 SWS patients at multiple timepoints. This allowed us to quantify gray and white matter volume changes in each hemisphere and brain region. These volumetric measurements were then compared with previously assigned qualitative atrophy scores (0-2) to assess their correlation with clinical evaluations.

We analyzed data from 5 SWS patients at 2 different time points. We observed that the difference in percentage of left vs right hemisphere total volume is highly correlated (r = 0.97) with qualitative atrophy scores. When a similar evaluation was done on gray matter and white matter individually, the correlation was lower (0.9), suggesting that total brain volume is comparatively more accurate in estimating brain atrophy.
This study demonstrates the feasibility of using quantitative brain atrophy measurements derived from MRI scans in Sturge-Weber Syndrome. By correlating these measurements with previous qualitative scores, we have established a promising approach for objectively quantifying brain atrophy in SWS patients and attaining improved understanding of disease progression and clinical outcomes.
Authors/Disclosures
Jenny Y. Wang
PRESENTER 		Ms. Wang has nothing to disclose.
Biswajit Maharathi, PhD Dr. Maharathi has nothing to disclose.
Csaba Juhasz, MD (University Health Center) The institution of Dr. Juhasz has received research support from NIH.
Jeffrey A. Loeb, MD, PhD (University of Illinois At Chicago) Dr. Loeb has received personal compensation in the range of $0-$499 for serving as an officer or member of the Board of Directors for Sturge Weber Foundation. Dr. Loeb has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for various Law firms. The institution of Dr. Loeb has received research support from Patrick Grange Memorial Foundation. Dr. Loeb has received intellectual property interests from a discovery or technology relating to health care.