• Ignacio Illán-Gala, Victor Montal, Jordi Pegueroles, Eduard Vilaplana, Daniel Alcolea, Oriol Dols-Icardo, Noemi de Luna, 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, and Ricard Rojas-García.
• From the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain. iillan@santpau.cat jfortea@santpau.cat.
• Neurology. 2020 Nov 3; 95 (18): e2565-e2576.

ObjectiveTo characterize the cortical macrostructure and microstructure of behavioral and cognitive changes along the amyotrophic lateral sclerosis (ALS)-frontotemporal dementia (FTD) continuum.MethodsWe 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.ResultsThe 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.ConclusionsCortical mean diffusivity might be a useful biomarker for the study of extramotor cortical neurodegeneration in the ALS-FTD clinical spectrum.Classification Of EvidenceThis study provides Class III evidence that the cortical microstructure correlates with cognitive impairment in the ALS-FTD continuum.© 2020 American Academy of Neurology.

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