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

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...

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Bibliographic Details
Published in:Stroke (1970) 2016-10, Vol.47 (10), p.2482-2487
Main Authors: Brookes, Rebecca L, Hollocks, Matthew J, Tan, Rhea Y.Y, Morris, Robin G, Markus, Hugh S
Format: Article
Language:English
Subjects:
Adult
Aged
Alopecia - complications
Alopecia - psychology
Cerebral Infarction - complications
Cerebral Infarction - psychology
Cognition - physiology
Cognitive Dysfunction - diagnosis
Cognitive Dysfunction - etiology
Executive Function - physiology
Female
Humans
Leukoencephalopathies - complications
Leukoencephalopathies - psychology
Male
Memory - physiology
Middle Aged
Neuropsychological Tests
Original Contributions
Prospective Studies
Sensitivity and Specificity
Spinal Diseases - complications
Spinal Diseases - psychology
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Summary: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.
ISSN:0039-2499
1524-4628
DOI:10.1161/STROKEAHA.116.013761