Cognition, mood and behavior in CADASIL

Cerebral Circulation - Cognition and Behavior, vol. 3, pp. 100043

Abstract

CADASIL is the most common familial cerebral small vessel disease (cSVD). Stereotyped mutations of the NOTCH3 gene are responsible for this archetypal ischemic cSVD that can lead, at the very end stage, to severe dementia. Variable cognitive alterations, mood, or behavior disturbances are frequently observed during the course of the disease. In this review, these clinical manifestations, their occurrence, severity and duration are analyzed in relation to the disease progression. Also, the potential relationships with cerebral lesions and treatment options are discussed.

Authors 2

1. Hugues Chabriat corresponding

   Inserm · Assistance Publique – Hôpitaux de Paris · Université Paris Cité · Hôpital Lariboisière · NeuroDiderot

   Affiliation as printed

   Referral Center for Rare Neurovascular Disorder, Translational Neurovascular Centre and CERVCO, Hopital Lariboisiere, APHP, 2 Rue A Paré, Paris 7010, France; University of Paris and INSERM U1141

   Referral Center for Rare Neurovascular Disorder, Translational Neurovascular Centre and CERVCO, Hopital Lariboisiere, APHP, 2 Rue A Paré, Paris 7010, France

   University of Paris and INSERM U1141,

2. Saskia A.J. Lesnik Oberstein Aachen Department of Clinical Genetics

   Leiden University Medical Center

   Affiliation as printed

   Department of Clinical Genetics, Leiden University Medical Center, The Netherland