Cerebral Small Vessel Disease Clinical, Neuropsychological, and Radiological Phenotypes, Histopathological Correlates, and Described Genotypes: A Review

Table 1

NINDS-AIREN and ADDTC criteria for vascular dementia.

	ADDTC	NINDS-AIREN
Dementia definition	Deterioration from a known level of intellectual function sufficient to interfere with the patients customary affairs of life and which is not isolated to a single category of intellectual performance.	Impairment of memory plus at least two other areas of cognitive domains, which should be severe enough to interfere with activities of daily living and not due to physical effects of stroke alone.
Probable VaD	Requiring all of the following: Dementia.; Evidence of two or more strokes by history, neurological signs, and/or imaging, or a single stroke with a clear temporal relationship to the onset of dementia.; Evidence of at least one infarct outside the cerebellum by CT or T1 weighted MRI.	Requiring all of the following: Dementia.; CVD: focal signs on examination + evidence of relevant CVD by CT/MRI.; A relationship between the above two disorders, manifested by one or more of the following: Dementia onset within 3 months of a stroke.; Abrupt deterioration in cognitive function, or fluctuating stepwise course.
Possible VaD	Dementia and one of the following: History or evidence of a single stroke without a clear temporal relationship to the dementia onset.; Binswanger’s disease that includes all the following: early onset of urinary incontinence or gait disturbance; vascular risk factors; extensive white matter changes on neuroimaging.	May be made in the presence of dementia and focal neurological signs in patients either with no evidence of CVD on neuroimaging, with the absence of a clear temporal relationship between stroke and dementia, or with subtle onset and variable course of cognitive deficit and evidence of CVD.

ADDTC: State of California Alzheimer’s Disease Diagnostic and Treatment Centers; CT: computed tomography; CVD: cerebrovascular disease; MRI: magnetic resonance imaging; NINDS-AIREN: National Institute of Neurological Diseases and Stroke and Association Internationale pour la Recherché et l’Enseignement en Neurosciences; VaD: vascular dementia.