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We aimed to investigate differences in fractional anisotropy (FA) between primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS) and the relationship between FA and disease progression using tract-based spatial statistics (TBSS).Two scanners at two different sites were used. Differences in FA between ALS patients and controls scanned in London were investigated. From the results of this analysis, brain regions were selected to test for (i) differences in FA between controls, patients with ALS and patients with PLS scanned in Oxford and (ii) the relationship between FA and disease progression rate in the Oxford patient groups.London ALS patients showed a lower FA than controls in several brain regions. Oxford patients with PLS showed a lower FA than ALS patients and than controls in the body of the corpus callosum and in the white matter adjacent to the right primary motor cortex (PMC), while ALS patients showed reduced FA compared with PLS patients in the white matter adjacent to the superior frontal gyrus. Significant correlations were found between disease progression rate and (i) FA in the white matter adjacent to the PMC in PLS, and (ii) FA along the cortico-spinal tract and in the body of the corpus callosum in ALS.We described significant FA changes between PLS and ALS, suggesting that these two presentations of motor neuron disease show different features. The significant correlation between FA and disease progression rate in PLS suggests the tissue damage reflected in FA changes contributes to the disease progression rate.

Original publication

DOI

10.1002/hbm.20527

Type

Journal article

Journal

Human brain mapping

Publication Date

02/2009

Volume

30

Pages

615 - 624

Addresses

Department of Brain Repair and Rehabilitation, Institute of Neurology, University College London, Queen Square, London. o.ciccarelli@ion.ucl.ac.uk

Keywords

Brain, Motor Cortex, Corpus Callosum, Pyramidal Tracts, Nerve Fibers, Myelinated, Humans, Amyotrophic Lateral Sclerosis, Motor Neuron Disease, Disease Progression, Observer Variation, Diffusion Magnetic Resonance Imaging, Brain Mapping, Data Interpretation, Statistical, Cohort Studies, Predictive Value of Tests, Image Processing, Computer-Assisted, Adult, Aged, Middle Aged, Female, Male