A case series of cerebrovascular abnormalities in Townes sickle cell mice visualized with magnetic resonance imaging and angiography.

Abstract

Arterial complications in sickle cell disease (SCD), including stenoses and occlusions, are critical contributors to stroke. Townes SCD mice exhibit neurocognitive deficits and micro-vasculopathy, however stenoses and occlusions that could be causal to ischemic strokes have not yet been confirmed, which has led to challenges whether murine pathology reflects human pathology for strokes due to SCD. In our longitudinal study using label-free magnetic resonance angiography (MRA) to image carotid arteries in Townes SCD mice as they aged from 1 to 7 months, we identified multiple stenoses and occlusions consistent with abnormalities seen in individuals with SCD and stroke complications. We report three cases showing abnormalities: Case 1, middle cerebral artery occlusion associated with seizures and a T1-weighted hyperintense lesion in the brain; Case 2, persistent internal carotid artery occlusion and side-specific artery size differences; and Case 3, persistent stenosis in the common carotid artery with aging. This study highlights the utility of MRA in tracking arterial changes in SCD mice where assessing symptomatic damage due to stroke is challenging. This study provides evidence of mouse-to-mouse variability in initiation and persistence of stenoses and occlusions resembling the variability of SCD complications in humans. HEADLINE: MRA identifies arterial lesions in mice with SCD.