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Title: Myelin paucity of the superior cerebellar peduncle in individuals with Friedreich ataxia: an MRI magnetization transfer imaging study.
Austin Authors: Corben, Louise A;Kashuk, Saman R;Akhlaghi, Hamed;Jamadar, Sharna;Delatycki, Martin B ;Fielding, Joanne;Johnson, Beth;Georgiou-Karistianis, Nellie;Egan, Gary F
Affiliation: Department of Clinical Genetics, Austin Health, Heidelberg, Victoria 3084, Australia
Monash Biomedical Imaging, Monash University, Clayton, Victoria 3800, Australia
School of Psychological Sciences, Monash University, Clayton, Victoria 3800, Australia
Monash Medical Centre, Monash Health, Clayton, Victoria 3168, Australia
Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Parkville 3052, Victoria, Australia
College of Engineering and Science, Victoria University, Melbourne 8001, Victoria, Australia
Issue Date: 2-Jun-2014
Publication information: Journal of the Neurological Sciences 2014; 343(1-2): 138-43
Abstract: The dentate nucleus (DN) is the major relay station for neural connection between the cerebellum and cerebrum via the thalamus, and is a significant component of the neuropathological profile of Friedreich ataxia (FRDA). We have previously shown that the size of the superior cerebellar peduncle (SCP), which links the DN to cortical and subcortical structures via the thalamus, is significantly reduced in individuals with FRDA compared to control participants. This study used magnetization transfer imaging (MTI) to examine and contrast the integrity of white matter (WM) in the SCP and the corpus callosum (CC) (control region) in ten individuals with FRDA and ten controls. Individuals with FRDA demonstrated a significant reduction in the magnetization transfer ratio (MTR) in the SCP compared to control participants. However, there was no significant difference between groups in MTR in the CC. When comparing regions within groups, there was a significant reduction in MTR in the SCP compared to CC in participants with FRDA only. We suggest that the reduction in MTR in the SCP may be indicative of lack of myelin secondary to axonal loss and oligodendroglial dysfunction in WM tracts in individuals with FRDA.
Gov't Doc #: 24930398
DOI: 10.1016/j.jns.2014.05.057
Journal: Journal of the neurological sciences
Type: Journal Article
Subjects: Axonopathy
Friedreich ataxia
Magnetization transfer ratio
Corpus Callosum.pathology
Diffusion Tensor Imaging
Friedreich Ataxia.pathology
Image Processing, Computer-Assisted
Middle Aged
Myelin Sheath.pathology
Nerve Fibers, Myelinated.pathology
Severity of Illness Index
Statistics as Topic
Young Adult
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