Quantitative susceptibility mapping depicts severe myelin deficit and iron deposition in a transgenic model of multiple system atrophy.

Quantitative susceptibility mapping depicts severe myelin deficit and iron deposition in a transgenic model of multiple system atrophy.
Exp Neurol. 2020 Apr 14;:113314
Authors: Lambrecht V, Hanspach J, Hoffmann A, Seyler L, Mennecke A, Straub S, Marxreiter F, Bäuerle T, Laun FB, Winkler J
Abstract
Despite internationally established diagnostic criteria, multiple system atrophy (MSA) is frequently misdiagnosed, particularly at disease onset. While neuropathological changes such as demyelination and iron deposition are typically detected in MSA, these structural hallmarks were so far only demonstrated post-mortem. Here, we examine whether myelin deficit observed in a transgenic murine model of MSA can be visualized and quantified in vivo using specific magnetic resonance imaging (MRI) approaches. Reduced myelin content was measured histologically in prototypical white matter as well as mixed grey-white matter regions i.e. corpus callosum, anterior commissure, and striatum of transgenic mice overexpressing human α-synuclein under the control of the myelin basic protein promotor (MBP29-hα-syn mice). Correspondingly, in vivo quantitative susceptibility mapping (QSM) showed a strongly reduced susceptibility contrast in white matter regions. Furthermore, T2-weighted MR imaging revealed a significantly reduced grey-white matter contrast in MBP29-hα-syn mice. In addition, morphological analysis suggested a pronounced, white matter-specifi...

Source: Experimental Neurology - April 13, 2020 Category: Neurology Authors: Lambrecht V, Hanspach J, Hoffmann A, Seyler L, Mennecke A, Straub S, Marxreiter F, Bäuerle T, Laun FB, Winkler J Tags: Exp Neurol Source Type: research