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Brief Screening of Vascular Cognitive Impairment in Patients With Cerebral Autosomal-Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy Without Dementia.

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Brookes, Rebecca L 
Hollocks, Matthew J 
Tan, Rhea YY 
Morris, Robin G 
Markus, Hugh S 


BACKGROUND AND PURPOSE: Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a monogenic form of cerebral small vessel disease leading to early-onset stroke and dementia, with younger patients frequently showing subclinical deficits in cognition. At present, there are no targeted cognitive screening measures for this population. However, the Brief Memory and Executive Test (BMET) and the Montreal Cognitive Assessment (MoCA) have shown utility in detecting cognitive impairment in sporadic small vessel disease. This study assesses the BMET and the MoCA as clinical tools for detecting mild cognitive deficits in CADASIL. METHODS: Sixty-six prospectively recruited patients with CADASIL, and 66 matched controls completed the BMET, with a subset of these also completing the MoCA. Receiver operating characteristic curves were calculated to examine the sensitivity and specificity of clinical cutoffs for the detection of vascular cognitive impairment and reduced activities of daily living. RESULTS: Patients with CADASIL showed more cognitive impairment overall and were poorer on both executive/processing and memory indices of the BMET relative to controls. The BMET showed good accuracy in predicting vascular cognitive impairment (85% sensitivity and 84% specificity) and impaired instrumental activities of daily living (92% sensitivity and 77% specificity). The MoCA also showed good predictive validity for vascular cognitive impairment (80% sensitivity and 78% specificity) and instrumental activities of daily living (75% sensitivity and 76% specificity). The most important background predictor of vascular cognitive impairment was a history of stroke. CONCLUSIONS: The results indicate that the BMET and the MoCA are clinically useful and sensitive screening measures for early cognitive impairment in patients with CADASIL.



CADASIL, activities of daily living, cerebral small vessel disease, cognition, stroke, Adult, Aged, Alopecia, Cerebral Infarction, Cognition, Cognitive Dysfunction, Executive Function, Female, Humans, Leukoencephalopathies, Male, Memory, Middle Aged, Neuropsychological Tests, Prospective Studies, Sensitivity and Specificity, Spinal Diseases

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Ovid Technologies (Wolters Kluwer Health)
British Heart Foundation (None)
Stroke Association (Grant ID: TSA2008/10), British Heart Foundation (Grant ID: PG/13/30/30005), Stroke Association/British Heart Foundation (Grant ID: TSA BHF 2010/01), Agency for Science, Technology and Research, Singapore, National Institute for Health Research (Senior Investigator award), Cambridge University Hospital Comprehensive National Institute for Health Research Biomedical Research Unit