Progression of alpha-synuclein pathology in multiple system atrophy of the cerebellar type.

ABSTRACT:

Aims

The aim of this study was to identify early foci of α-synuclein (α-syn pathology) accumulation, subsequent progression and neurodegeneration in multiple system atrophy of the cerebellar type (MSA-C).

Methods

We analysed 70-μm-thick sections of 10 cases with MSA-C and 24 normal controls.

Results

MSA-C cases with the lowest burden of pathology showed α-syn glial cytoplasmic inclusions (GCIs) in the cerebellum as well as in medullary and pontine cerebellar projections. Cerebellar pathology was highly selective and severely involved subcortical white matter, whereas deep white matter and granular layer were only mildly affected and the molecular layer was spared. Loss of Purkinje cells increased with disease duration and was associated with neuronal and axonal abnorm

SUBMITTER: Brettschneider J 

PROVIDER: S-EPMC5362365 | biostudies-literature | 2017 Jun

REPOSITORIES: biostudies-literature