Dilative Arteriopathy and Leucencephalopathy as Manifestations of a Neurometabolic Disease



Josef Finsterer *, 1, Adam Bastovansky 2
1 Krankenanstalt Rudolfstiftung
2 Radiological Department, Krankenanstalt Rudolfstiftung, Vienna, Austria


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© Finsterer and Bastovansky; Licensee Bentham Open.

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at the Postfach 20, 1180 Vienna, Austria, Europe; Tel. +43-1-71165-92085; Fax. +43-1-4781711; E-mail: fifigs1@yahoo.de


Abstract

Background:

Dilative arteriopathy plus leucencephalopathy as a manifestation of a mitochondrial disorder (MID) is rare.

Case report:

In a 70yo Caucasian female, height 160cm, weight 62kg, with mild right-sided hemiparesis due to subacute ischemic stroke in the posterior leg of the left internal capsule, a megadolichobasilar artery and marked leucencephalopathy and gliosis of the pons were detected. In addition, microbleeds in a peripheral distribution at the cortical/subcortical border were noted. After the exclusion of various differentials, which could have explained her abnormalities, a MID was suspected.

Conclusions:

Dilative arteriopathy of the intra-cerebral arteries, in association with recurrent stroke and supra- and infratentorial leucencephalopathy, but the absence of neurofibromatosis or increased low-density lipoprotein values, is most likely attributable to a non-syndromic MID.

Keywords: Mitochondrial disorder, genetics, dilative arteriopathy, megadolichobasilar artery, arteriopathy, stroke.