title: The selective anatomical vulnerability of ALS: 'disease-defining' and 'disease-defying' brain regions.
abstract: A large multiparametric MRI study has been undertaken to evaluate anatomical patterns of basal ganglia, white matter and cortical grey matter involvement in ALS. Unaffected brain regions are mapped in patients with significant disability. Multiple white matter diffusivity measures, cortical grey matter density alterations, basal ganglia volumes and subcortical grey matter atrophy patterns are evaluated. Results demonstrated a strikingly selective anatomical vulnerability pattern in ALS that preferentially affects specific grey matter structures, commissural white matter tracts and basal ganglia regions, suggestive of networkwise neurodegeneration in ALS. In conclusion, ALS pathology exhibits predilection for selective and inter-connected anatomical sites that can be comprehensively characterized in vivo by multiparametric neuroimaging. The systematic characterization of unaffected brain regions in ALS has implications for the development of classifier analyses and elucidation of disease biology. The involvement and sparing of contiguous brain regions raises important pathophysiological, phylogenetic and ontogenetic questions regarding ALS pathogenesis and disease spread.
authors.affiliation: a Quantitative Neuroimaging Group, Academic Unit of Neurology , Biomedical Sciences Institute, Trinity College Dublin , Ireland.
authors.forename: Peter
authors.initials: P
authors.lastname: Bede
authors.affiliation: a Quantitative Neuroimaging Group, Academic Unit of Neurology , Biomedical Sciences Institute, Trinity College Dublin , Ireland.
authors.forename: Parameswaran M
authors.initials: PM
authors.lastname: Iyer
authors.affiliation: a Quantitative Neuroimaging Group, Academic Unit of Neurology , Biomedical Sciences Institute, Trinity College Dublin , Ireland.
authors.forename: Christina
authors.initials: C
authors.lastname: Schuster
authors.affiliation: a Quantitative Neuroimaging Group, Academic Unit of Neurology , Biomedical Sciences Institute, Trinity College Dublin , Ireland.
authors.forename: Marwa
authors.initials: M
authors.lastname: Elamin
authors.affiliation: a Quantitative Neuroimaging Group, Academic Unit of Neurology , Biomedical Sciences Institute, Trinity College Dublin , Ireland.
authors.forename: Russell L
authors.initials: RL
authors.lastname: Mclaughlin
authors.affiliation: a Quantitative Neuroimaging Group, Academic Unit of Neurology , Biomedical Sciences Institute, Trinity College Dublin , Ireland.
authors.forename: Kevin
authors.initials: K
authors.lastname: Kenna
authors.affiliation: a Quantitative Neuroimaging Group, Academic Unit of Neurology , Biomedical Sciences Institute, Trinity College Dublin , Ireland.
authors.forename: Orla
authors.initials: O
authors.lastname: Hardiman
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000000981:diagnostic imaging / Q000473:pathology*; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology*; D005260:Female; D066128:Gray Matter / Q000000981:diagnostic imaging; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D066127:White Matter / Q000000981:diagnostic imaging
keywords: Amyotrophic lateral sclerosis; basal ganglia; biomarker; diffusion tensor imaging; magnetic resonance imaging; pathophysiology