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The CADA-MRIT: An MRI Inventory Tool for Evaluating Cerebral Lesions in CADASIL Across Cohorts
Ruiting Zhang1, Chih-Hao Chen1, Sophie Tezenas Du Montcel1
1From the Paris-Cité University (R.Z., J.L., H.C.), Inserm U1141 NeuroDiderot, France; Department of Radiology (R.Z.), the Second Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou, China; Department of Neurology (C.-H.C., Y.-W.C., S.-C.T.), National Taiwan University Hospital, Taipei; Department of Clinical Neurosciences (C.-H.C.), University of Calgary, Alberta, Canada; Sorbonne Université (S.T.D.M.), Paris Brain Institute, INSERM, INRIA, CNRS, APHP; Lariboisière University Hospital (J.L., H.C.), APHP, Translational Neurovascular Centre and Department of Neurology, Reference Center for Rare Vascular Diseases of the Central Nervous System and the Retina (CERVCO), FHU NeuroVasc, Paris, France; Department of Neurology (Y.-W.C.), National Taiwan University Hospital Hsinchu Branch; Institute for Stroke and Dementia Research (M.D.), University Hospital, Ludwig Maximilian University, Munich; German Center for Neurodegenerative Diseases (DZNE) (M.D.), Munich; and Munich Cluster for Systems Neurology (SyNergy) (M.D.), Germany.
Insights
A new tool, CADA-MRIT, simplifies MRI analysis for Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL). It reliably assesses key lesions, enabling cross-population comparisons of this genetic cerebrovascular disease.
Area of Science:
- Neurology
- Radiology
- Genetics
Background:
- Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common inherited cerebrovascular disorder.
- Significant variability exists in clinical presentation and MRI findings across different CADASIL patient cohorts.
- Limitations in standardizing MRI acquisition hinder comparisons between studies and populations.
Purpose of the Study:
- To develop and validate a user-friendly tool for rapid inventory of key MRI features in CADASIL.
- To enable standardized comparison of imaging data across diverse patient populations and international cohorts.
- To improve the consistency and comparability of MRI assessments in CADASIL research.
Main Methods:
- The CADA-MRIT tool was developed through expert consensus, comprising 11 imaging items.
- The tool assesses white matter hyperintensity (WMH), lacunes, cerebral microbleeds (CMB), dilated perivascular spaces (dPVS), atrophy, infarcts, and macrobleeds.
- Reliability, clinical relevance, and time-effectiveness were evaluated using data from 671 patients across three independent cohorts (France, Germany, Taiwan).
Main Results:
- CADA-MRIT scores for WMH, CMB, and dPVS demonstrated high reliability (weighted κ > 0.60) across different scanners and sequences.
- Intrarater and interrater agreement for the tool were consistently good to very good (weighted κ > 0.60).
- Global WMH and atrophy scores correlated strongly with volumetric measures, and imaging scores associated with clinical manifestations; assessment time was 2-3 minutes per patient.
Conclusions:
- CADA-MRIT is a reliable, easy-to-use instrument for analyzing and comparing common MRI lesions in CADASIL.
- The tool facilitates consistent imaging data comparison across different populations, scanners, and sequences.
- CADA-MRIT provides clinically relevant scores efficiently, aiding research and clinical practice in CADASIL.
Background And Objectives:
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 MRI vary widely not only within families but also between different cohorts reported worldwide. Many limitations prevent comparison of imaging data obtained with different scanners and sequences in different patient cohorts. We aimed to develop and validate a simple tool to inventory quickly the key MRI features in CADASIL to compare imaging data across different populations.
Methods:
The Inventory Tool (CADA-MRIT) was designed by consensus after repeated expert meetings. It consists of 11 imaging items to assess periventricular, deep, and superficial white matter hyperintensity (WMH), lacunes, cerebral microbleeds (CMB), centrum semiovale and basal ganglia dilated perivascular spaces (dPVS), superficial and deep atrophy, large infarcts, and macrobleeds. The reliability, clinical relevance, and time-effectiveness of CADA-MRIT were assessed using data from 3 independent patient cohorts.
Results:
Imaging data from 671 patients with CADASIL (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, whereas CMB occurred in 83% of patients from the Asian cohort and in only 35% of European patients. CADA-MRIT scores obtained for WMH, CMB, and dPVS were comparable regardless of the scanner or sequence used (weighted κ > 0.60). Intrarater and interrater agreements were from good to very good (weighted κ > 0.60). Global WMH and atrophy scores correlated strongly with accurate 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 1 patient was approximately 2-3 minutes.
Discussion:
The CADA-MRIT is an easy-to-use tool for analyzing and comparing the most frequent MRI lesions of CADASIL across different populations. This instrument is reliable. It can be used with different imaging sequences or scanners. It also provides clinically relevant scores in a very short time for completion.
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