Cortical microstructure in the amyotrophic lateral sclerosis-frontotemporal dementia continuum

Ignacio Illán-Gala*, Victor Montal, Jordi Pegueroles, Eduard Vilaplana, Daniel Alcolea, Oriol Dols-Icardo, Janina Turón-Sans, Elena Cortés-Vicente, Luis Martinez-Roman, Maria Belén Sánchez-Saudinós, Andrea Subirana, Laura Videla, Isabel Sala, Isabel Barroeta, Sílvia Valldeneu, Rafael Blesa, Jordi Clarimón, Alberto Lleó, Juan Fortea, Ricard Rojas-García

*Corresponding author for this work

Research output: Contribution to journalArticleResearchpeer-review

12 Citations (Scopus)


Objective To characterize the cortical macrostructure and microstructure of behavioral and cognitive changes along the amyotrophic lateral sclerosis (ALS)-frontotemporal dementia (FTD) continuum. Methods We prospectively recruited 88 participants with a 3T MRI structural and diffusion-weighted imaging sequences: 31 with ALS, 20 with the behavioral variant of FTD (bvFTD), and 37 cognitively normal controls. Participants with ALS underwent a comprehensive cognitive and behavioral assessment and were dichotomized into 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 ALS Screen. Results The 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 restricted mainly to the dorsal motor cortex. In contrast, in the ALScbi group, cortical thinning was observed primarily on frontoinsular and temporal regions bilaterally. There were progressive cortical mean diffusivity changes along the ALSno-cbi, ALScbi, and bvFTD clinical continuum. Participants with ALS 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 extramotor cortical neurodegeneration in the ALS-FTD clinical spectrum.

Original languageEnglish
Pages (from-to)E2565-E2576
Issue number18
Publication statusPublished - 3 Nov 2020


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