Vanishing White Matter Hyperintensities in CADASIL: A Case Report with Insight into Disease Mechanisms

Article type: Short Communication

Authors: Jouvent, Eric^{a; b; c; d; *} | Alili, Nassira^{b; c} | Hervé, Dominique^{b; c} | Chabriat, Hugues^{a; b; c; d}

Affiliations: [a] Université de Paris, Paris, France | [b] APHP, Lariboisière Hospital, Department of Neurology and Referral Center for rare vascular diseases of the brain and retina (CERVCO), Paris, France | [c] FHU NeuroVasc, Université de Paris, Paris, France | [d] U1141 INSERM, Paris, France

Correspondence: [*] Correspondence to: Eric Jouvent, MD, PhD, Hôpital Lariboisière, 2 rue Ambroise Paré, 75010 Paris, France. Tel.: +1 331 49 95 65 29; E-mail: eric.jouvent@aphp.fr.

Abstract: In a woman with Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) followed for 15 years, we observed magnetic resonance imaging white matter hyperintensities that vanished in the anterior temporal poles while the brain volume decreased unexpectedly. These imaging changes were transient and detected when the patient was being treated by valproic acid for stabilizing mood disturbances. This intriguing case supports that mechanisms underlying white matter hyperintensities can vary from one brain area to another and that important modifications of water influx into the brain tissue might be involved in some imaging features of CADASIL.

Keywords: Brain volume, CADASIL, valpromide, white matter hyperintensities

DOI: 10.3233/JAD-201086

Journal: Journal of Alzheimer's Disease, vol. 78, no. 3, pp. 907-910, 2020