Utility of modified Rankin Scale for brain vascular malformations in hereditary hemorrhagic telangiectasia.
K P ThompsonJ NelsonH KimS M WeinsheimerD A MarchukM T LawtonT KringsMarie E Faughnannull nullPublished in: Orphanet journal of rare diseases (2021)
Most HHT-brain VM patients had good functional capacity (mRS scores 0-2) at baseline that did not change significantly over 3.4 mean years of follow-up, suggesting that mRS may not be useful for predicting or measuring outcomes in these patients. However, HHT patients with GI bleeding, anemia history or liver VMs had worse mRS scores, suggesting significant impact of these manifestations on functional capacity. Our study demonstrates the insensitivity of the mRS as an outcomes measure in HHT brain VM patients and reinforces the continued need to develop outcomes measures, and their predictors, in this group.