Early white matter changes in CADASIL: evidence of segmental intramyelinic oedema in a pre-clinical mouse model.

ABSTRACT:

Introduction

Small vessel disease (SVD) of the brain is a leading cause of age- and hypertension-related cognitive decline and disability. Cerebral white matter changes are a consistent manifestation of SVD on neuroimaging, progressing silently for many years before becoming clinically evident. The pathogenesis of these changes remains poorly understood, despite their importance. In particular, their pathological correlate at early stages remains largely undefined. Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL), caused by dominant mutations of the NOTCH3 receptor, is regarded as a paradigm for the most common form of sporadic SVD. In this study, we used immunohistochemistry, confocal microscopy and electron microscopy, together

SUBMITTER: Cognat E 

PROVIDER: S-EPMC4035092 | biostudies-literature | 2014 Apr

REPOSITORIES: biostudies-literature