作者
Peter Bede, Arun Bokde, Marwa Elamin, Susan Byrne, Russell L McLaughlin, Norah Jordan, Harald Hampel, Laura Gallagher, Catherine Lynch, Andrew J Fagan, Niall Pender, Orla Hardiman
发表日期
2013/7/1
期刊
Journal of Neurology, Neurosurgery & Psychiatry
卷号
84
期号
7
页码范围
766-773
出版商
BMJ Publishing Group Ltd
简介
Background
Body region of onset and functional disability are key components of disease heterogeneity in amyotrophic lateral sclerosis (ALS).
Objectives
To evaluate patterns of grey matter pathology in the motor cortex and correlate focal structural changes with functional disability.
Methods
We conducted a single-centre neuroimaging study of a cohort of 33 cognitively normal patients with amyotrophic lateral sclerosis (ALS) and 44 healthy controls. A voxel-wise generalised linear model was used to investigate the distribution of disease burden within the motor cortex in relation to clinical disability.
Results
Patients with bulbar onset have bilateral focal atrophy in the bulbar segment of the motor homunculus compared with patients with limb onset who have focal cortical changes in the limb segment of their motor strip. Furthermore, the extent to which different body regions are affected in ALS corresponds to the …
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