Data from: Cortical microstructure in the amyotrophic lateral sclerosis-frontotemporal dementia continuum



Objective: We aimed to characterize cortical macro- and micro-structure of behavioral and cognitive changes along the amyotrophic lateral sclerosis (ALS) – frontotemporal dementia (FTD) continuum. Methods: We prospectively recruited 88 participants with a three-Tesla MRI structural and diffusion-weighted imaging sequences: 31 ALS, 20 bvFTD, and 37 cognitively normal controls. ALS participants underwent a comprehensive cognitive and behavioral assessment and were dichotomized in ALS without cognitive or behavioral impairment (ALSno-cbi, n=12) and ALS with cognitive or behavioral impairment (ALScbi, n=19). We computed cortical thickness and cortical mean diffusivity using a surface-based approach and explored the cortical correlates of cognitive impairment with the Edinburgh Cognitive and Behavioral Amyotrophic lateral sclerosis Screen (ECAS). Results: ALSno-cbi and ALScbi groups showed different patterns of reduced cortical thickness and increased cortical mean diffusivity. In the ALSno-cbi group, cortical thinning was mainly restricted to the dorsal motor cortex. In contrast, in the ALScbi group, cortical thinning was observed primarily on fronto-insular and temporal regions bilaterally. There were progressive cortical mean diffusivity changes along the ALSno-cbi, ALScbi, and bvFTD clinical continuum. Importantly, ALS participants with either cognitive or behavioral impairment showed increased cortical mean diffusivity in the prefrontal cortex in the absence of cortical thickness. Conclusions: Cortical mean diffusivity might be a useful biomarker for the study of extra motor cortical neurodegeneration in the ALS-FTD clinical spectrum.
Date made available13 Jul 2021
  • Cortical microstructure in the amyotrophic lateral sclerosis-frontotemporal dementia continuum

    Illán-Gala, I., Montal, V., Pegueroles, J., Vilaplana, E., Alcolea, D., Dols-Icardo, O., Turón-Sans, J., Cortés-Vicente, E., Martinez-Roman, L., Sánchez-Saudinós, M. B., Subirana, A., Videla, L., Sala, I., Barroeta, I., Valldeneu, S., Blesa, R., Clarimón, J., Lleó, A., Fortea, J. & Rojas-García, R., 3 Nov 2020, In: Neurology. 95, 18, p. E2565-E2576

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