M M El Mendili1,2, A-M Grapperon3,2,4, R Dintrich3,2,4, J-P Stellmann3,2, J-P Ranjeva3,2, M Guye3,2, A Verschueren3,2, S Attarian4, W Zaaraoui3,2. 1. From the Aix Marseille University (M.M.E.M., A.-M.G., R.D., J.-P.S., J.-P.R., M.G., A.V., W.Z.), Centre national de la recherche scientifique, The Center for Magnetic Resonance in Biology and Medicine, Marseille, France mm.elmendili@univ-amu.fr. 2. APHM, Hopital de la Timone (M.M.E.M., A.-M.G., R.D., J.-P.S., J.-P.R., M.G., A.V., W.Z.), CEMEREM, Marseille, France. 3. From the Aix Marseille University (M.M.E.M., A.-M.G., R.D., J.-P.S., J.-P.R., M.G., A.V., W.Z.), Centre national de la recherche scientifique, The Center for Magnetic Resonance in Biology and Medicine, Marseille, France. 4. APHM, Hôpital de la Timone (A.-M.G., R.D., S.A.), Referral Centre for Neuromuscular Diseases and ALS, Marseille, France.
Abstract
BACKGROUND AND PURPOSE: While conventional MR imaging has limited value in amyotrophic lateral sclerosis, nonconventional MR imaging has shown alterations of microstructure using diffusion MR imaging and recently sodium homeostasis with sodium MR imaging. We aimed to investigate the topography of brain regions showing combined microstructural and sodium homeostasis alterations in amyotrophic lateral sclerosis subgroups according to their disease-progression rates. MATERIALS AND METHODS: Twenty-nine patients with amyotrophic lateral sclerosis and 24 age-matched healthy controls were recruited. Clinical assessments included disease duration and the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale. Patients were clinically differentiated into fast (n = 13) and slow (n = 16) progressors according to the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale progression rate. 3T MR imaging brain protocol included 1H T1-weighted and diffusion sequences and a 23Na density-adapted radial sequence. Quantitative maps of diffusion with fractional anisotropy, mean diffusivity, and total sodium concentration were measured. The topography of diffusion and sodium abnormalities was assessed by voxelwise analyses. RESULTS: Patients with amyotrophic lateral sclerosis showed significantly higher sodium concentrations and lower fractional anisotropy, along with higher sodium concentrations and higher mean diffusivity compared with healthy controls, primarily within the corticospinal tracts, corona radiata, and body and genu of the corpus callosum. Fast progressors showed wider-spread abnormalities mainly in the frontal areas. In slow progressors, only fractional anisotropy measures showed abnormalities compared with healthy controls, localized in focal regions of the corticospinal tracts, the body of corpus callosum, corona radiata, and thalamic radiation. CONCLUSIONS: The present study evidenced widespread combined microstructural and sodium homeostasis brain alterations in fast amyotrophic lateral sclerosis progressors.
BACKGROUND AND PURPOSE: While conventional MR imaging has limited value in amyotrophic lateral sclerosis, nonconventional MR imaging has shown alterations of microstructure using diffusion MR imaging and recently sodium homeostasis with sodium MR imaging. We aimed to investigate the topography of brain regions showing combined microstructural and sodium homeostasis alterations in amyotrophic lateral sclerosis subgroups according to their disease-progression rates. MATERIALS AND METHODS: Twenty-nine patients with amyotrophic lateral sclerosis and 24 age-matched healthy controls were recruited. Clinical assessments included disease duration and the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale. Patients were clinically differentiated into fast (n = 13) and slow (n = 16) progressors according to the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale progression rate. 3T MR imaging brain protocol included 1H T1-weighted and diffusion sequences and a 23Na density-adapted radial sequence. Quantitative maps of diffusion with fractional anisotropy, mean diffusivity, and total sodium concentration were measured. The topography of diffusion and sodium abnormalities was assessed by voxelwise analyses. RESULTS: Patients with amyotrophic lateral sclerosis showed significantly higher sodium concentrations and lower fractional anisotropy, along with higher sodium concentrations and higher mean diffusivity compared with healthy controls, primarily within the corticospinal tracts, corona radiata, and body and genu of the corpus callosum. Fast progressors showed wider-spread abnormalities mainly in the frontal areas. In slow progressors, only fractional anisotropy measures showed abnormalities compared with healthy controls, localized in focal regions of the corticospinal tracts, the body of corpus callosum, corona radiata, and thalamic radiation. CONCLUSIONS: The present study evidenced widespread combined microstructural and sodium homeostasis brain alterations in fast amyotrophic lateral sclerosis progressors.
Authors: Johannes Brettschneider; Kelly Del Tredici; Jon B Toledo; John L Robinson; David J Irwin; Murray Grossman; EunRan Suh; Vivianna M Van Deerlin; Elisabeth M Wood; Young Baek; Linda Kwong; Edward B Lee; Lauren Elman; Leo McCluskey; Lubin Fang; Simone Feldengut; Albert C Ludolph; Virginia M-Y Lee; Heiko Braak; John Q Trojanowski Journal: Ann Neurol Date: 2013-06-19 Impact factor: 10.422
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