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(Stroke. 2008;39:838.)
© 2008 American Heart Association, Inc.

Original Contributions

Diagnostic Criteria of Vascular Dementia in CADASIL

Sarah Benisty, MD; Karen Hernandez, MSc; Anand Viswanathan, MD, PhD; Sonia Reyes, MSc; Annie Kurtz, MSc; Michael O’Sullivan, PhD; Marie-Germaine Bousser, MD; Martin Dichgans, MD, PhD Hugues Chabriat, MD, PhD

From Department of Geriatric Medicine (S.B.) and Department of Neurology (K.H., S.R., A.K., M.-G.B., H.C.), University Paris VII, France; Department of Neurology and Clinical Trials Unit (A.V.), Massachusetts General Hospital and Harvard Medical School, Boston, Mass; Department of Neurology (M.O., M.D.), Klinikum Grosshadern, Ludwig-Maximilians-University, Munich, Germany.

Correspondence to Prof Hugues Chabriat, Department of Neurology, University Paris 7, 2 rue Ambroise Paré, 75010 Paris, France. E-mail hugues.chabriat{at}lrb.aphp.fr

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.

Key Words: CADASIL • diagnostic criteria • leukoariosis • vascular dementia