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Published on: November 6, 2017
Diagnostic criteria of vascular dementia in CADASIL
Sarah Benisty1, Karen Hernandez, Anand Viswanathan
1Department of Geriatric Medicine, University Paris 7, 2 rue Ambroise Paré, 75010 Paris, France.
Insights
Modified NINDS-AIREN criteria best detect subcortical ischemic vascular dementia (SIVD) in CADASIL patients. Neuroimaging criteria show high sensitivity for SIVD diagnosis in this genetic model.
Area of Science:
- Neurology
- Genetics
- Neuroimaging
Background:
- Subcortical ischemic vascular dementia (SIVD) is a key subtype of vascular dementia (VaD).
- Recent modifications to VaD diagnostic criteria aim to include SIVD.
- CADASIL serves as a genetic model for SIVD, valuable for assessing diagnostic criteria.
Purpose of the Study:
- To compare the effectiveness of various vascular dementia diagnostic criteria in CADASIL patients.
- To evaluate the significance of modified VaD criteria in a specific genetic population.
Main Methods:
- Applied DSM-IV, ICD-10, standard NINDS-AIREN, and modified NINDS-AIREN for SIVD criteria to 115 CADASIL patients.
- Diagnosed dementia and its association with vascular lesions in two steps.
- Analyzed the percentage of patients meeting each criterion and the concordance between them.
Main Results:
- Twenty-nine patients met at least one VaD criterion.
- Sensitivity for VaD diagnosis: DSM-IV (79%), ICD-10 (72%), standard NINDS-AIREN (45%).
- Modified NINDS-AIREN criteria for SIVD showed 90% sensitivity; neuroimaging criteria were met by all dementia patients.
Conclusions:
- Modified NINDS-AIREN criteria demonstrate the highest sensitivity for diagnosing SIVD in CADASIL.
- Neuroimaging criteria are highly sensitive for SIVD but lack specificity.
- Focal neurological signs were inconsistently present in CADASIL patients with dementia.
Background And Purpose:
Subcortical ischemic vascular dementia (SIVD) is a major subtype of vascular dementia (VaD). Recently, the diagnostic criteria of VaD have been modified to encompass this entity. Application of these criteria in CADASIL, a genetic model of SIVD, may help to better assess their significance. The aim of this study was to compare different sets of diagnostic criteria of VaD in a population of CADASIL patients.
Methods:
Different sets of diagnostic criteria of VaD (DSMIV, ICD10, standard NINDS-AIREN, modified NINDS-AIREN for SIVD) were applied to 115 CADASIL patients. Diagnosis of VaD was made through 2 steps: (1) diagnosis of dementia and (2) association of dementia to lesions of vascular origin. The percentage of patients satisfying the different sets and the concordance between these criteria was analyzed.
Results:
At least 1 set of criteria was satisfied for diagnosis in 29 subjects with dementia. In this group of patients, the sensitivity of the DSM IV, ICD 10, and standard NINDS-AIREN criteria for VaD was, respectively, 79%, 72%, and 45%. In contrast, the sensitivity of the NINDS-AIREN criteria for SIVD was 90%. The incomplete sensitivity of these last criteria was related to the absence of focal signs in some patients. The neuroimaging criteria were satisfied in all patients with dementia.
Conclusions:
The modified NINDS-AIREN criteria of SIVD are the most sensitive VaD criteria in CADASIL. Among these criteria, the neuroimaging criteria, although poorly specific to dementia, have a complete sensitivity. In contrast, focal signs were inconstant in CADASIL patients with dementia.
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