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Paraskevas et al. Neuroimmunol Neuroinflammation 2018;5:49 Neuroimmunology and
DOI: 10.20517/2347-8659.2018.50 Neuroinflammation

Review Open Access

Cerebral autosomal dominant arteriopathy with
subcortical infarcts and leucoencephalopathy vs.
multiple sclerosis. Either one or sometimes both?

George P. Paraskevas, Vasilios C. Constantinides, Elisabeth Kapaki

Cognitive and Movement Disorders Clinic and Neurochemistry Unit, 1st Department of Neurology, National and Kapodistrian University of
Athens, School of Medicine, Eginition Hospital, Athens 11528, Greece.

Correspondence to: George P. Paraskevas, Cognitive and Movement Disorders Clinic and Neurochemistry Unit, 1st Department of
Neurology, National and Kapodistrian University of Athens, School of Medicine, Eginition Hospital, 72 Vas. Sophias Ave, Athens 11528,
Greece. E-mail: geoprskvs44@gmail.com

How to cite this article: Paraskevas GP, Constantinides VC, Kapaki E. Cerebral autosomal dominant arteriopathy with subcortical
infarcts and leucoencephalopathy vs. multiple sclerosis. Either one or sometimes both? Neuroimmunol Neuroinflammation 2018;5:49.
http://dx.doi.org/10.20517/2347-8659.2018.50

Received: 27 Aug 2018 First Decision: 17 Oct 2018 Revised: 5 Nov 2018 Accepted: 14 Nov 2018 Published: 14 Dec 2018

Science Editor: Athanassios P. Kyritsis Copy Editor: Cui Yu Production Editor: Huan-Liang Wu

Abstract
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL), is the
most common cause of inherited cerebral small vessel disease, inherited stroke and inherited vascular dementia.
It is not infrequent for CADASIL to be mistaken and mistreated for multiple sclerosis (MS). A much less frequent
but existing scenario is the co-occurrence of CADASIL and MS (or MS-like inflammatory condition). Such patients
may present with spinal cord lesions, brain or spinal cord enhancing lesions, positive oligoclonal bands and high IgG
index in the cerebrospinal fluid and good response to corticosteroids or immunomodulating treatments. CADASIL
through various mechanisms may trigger or modulate autoimmune reactions, and either be complicated by an
inflammatory component or cause an MS-like disorder.

Keywords: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy, multiple
sclerosis, autoimmunity, NOTCH3

INTRODUCTION
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL),
[1]
caused by mutations in NOTCH3 gene is the most common cause of inherited cerebral small vessel disease,
[2]
inherited stroke and inherited vascular dementia . Patients typically present with various combinations of

© The Author(s) 2018. Open Access This article is licensed under a Creative Commons Attribution 4.0
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