The CADA-MRIT: an MRI Inventory Tool for evaluating cerebral lesions in CADASIL across cohorts

Abstract

Introduction

Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and leukoencephalopathy (CADASIL) is the most frequent genetic cerebrovascular disease. The clinical aspects of the disease in relation to the various types of lesions on Magnetic Resonance Imaging (MRI) vary widely within families but also between multiple cohorts reported worldwide. Many limitations prevent the comparison of imaging data obtained using different scanners and sequences, in different patients cohorts. We aimed to develop and validate a practical tool to inventory quickly the most important MRI features in CADASIL for comparing imaging data across different populations.

Methods

The Inventory Tool (CADA-MRIT) was designed by consensus after repeated expert meetings. It consisted of eleven imaging items for evaluating periventricular, deep, and superficial white matter hyperintensity (WMH), lacunes, cerebral microbleeds (CMB), centrum semiovale and basal ganglia dilated perivascular spaces (PVS), superficial and deep atrophy, large infarcts, and macrobleeds. The reliability, clinical relevance and time-effectiveness of the CADA-MRIT were assessed using data from three independent cohorts of patients.

Results

Imaging data from 671 CADASIL patients (440 from France, 119 from Germany and 112 from Taiwan) were analyzed. Their mean age was 53.4 ± 12.2 years, 54.5% were women, 56.2% had stroke, and 31.1% had migraine with aura. Any lacune was present in at least 70% of individuals, while CMB were present in 83% of patients from the Asian cohort and in only 35% European patients. The CADA-MRIT scores obtained for WMH, CMB and PVS were comparable regardless of the scanner or sequence used (weighted κ > 0.60). Intrarater and interrater agreements were good to very good (weighted κ > 0.60). Global WMH and atrophy scores strongly correlated with the exact volumetric quantification of WMH or brain parenchymal fraction (Pearson r > 0.60). Different imaging scores were significantly associated with the main clinical manifestations of the disease. The time for evaluating one patient was around 2-3 minutes.

Discussion

The CADA-MRIT is an easy-to-use instrument for analyzing and comparing the most frequent MRI lesions of CADASIL across different populations. This tool is reliable. It can be used with different imaging sequences or scanners.