Neuroscore
JHU Ref #: C17050
Technology Description: Researchers at Johns Hopkins University have developed a product that provides a uniform clinical score for Sturge-Weber Syndrome by determining whether cortical atrophy correlates to clinical severity. The clinical severity was evaluated using computer aided analysis of MRIs to produce laterality scores for cortical volume asymmetry. It was found that this clinical scale had a significant correlation with clinical severity scores assigned by independent investigators who utilized symptomatic seizure, hemiparesis, visual field cut and cognitive impairments.
Unmet Need
· Sturge-Weber Syndrome is a neurocutaneous disorder that causes vascular malformations of skin, brain, and eye. These can lead to seizures, a facial port-wine stain, and glaucoma (Kelley et al.).
· 1 in 20,000 – 50,000 newborns have this condition. The first indication is an incidence of seizure (Singh et al.).
· Because treatment for the disease is largely only based on symptomatic effects like seizures, there is a need for quantitative measures of biological markers to evaluate treatment interventions.
Value Proposition
· Multiple biomedical companies and academic centers can use this product to compare clinical trial outcomes for Sturge-Weber Syndrome with historical control data.
· This score offers a simple, objective and wide application for outside institution.
· There is no other clinical score or scale for this condition.
Stage of Development
· NIH funded project with human studies
· Technology can be implemented at any time
Data Availability
· Published clinical data available in the publication below.
Publication
Quantitative Analysis of Cerebral Cortical Atrophy and Correlation With Clinical Severity in Unilateral Sturge‐Weber Syndrome Thomas M. Kelley, BS; Laura A. Hatfield, BS; Doris D. M. Lin, MD, PhD; Anne M. Comi, MD J Child Neurology Volume 20, Number 11, November 2005
