Frontal and Cerebellar Atrophy Supports FTSD-ALS Clinical Continuum.

Autor: Pizzarotti B; Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.; Department of Clinical Neuroscience, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland., Palesi F; Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.; Brain Connectivity Center, IRCCS Mondino Foundation, Pavia, Italy., Vitali P; Radiology Unit, IRCCS Mondino Foundation, Pavia, Italy.; Department of Radiology, IRCCS Policlinico San Donato, Milan, Italy., Castellazzi G; NMR Research Unit, Department of Neuroinflammation, Queen Square MS Centre, UCL Queen Square Institute of Neurology, Faculty of Brain Sciences, University College London, London, United Kingdom.; Department of Electrical, Computer and Biomedical Engineering, University of Pavia, Pavia, Italy.; IRCCS Mondino Foundation, Pavia, Italy., Anzalone N; Neuroradiology Unit, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy., Alvisi E; Department of Neurology and Laboratory Neuroscience, IRCCS Italian Auxological Institute, Milan, Italy., Martinelli D; Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.; Headache Science and Neurorehabilitation, IRCCS Mondino Foundation, Pavia, Italy., Bernini S; Laboratory of Neuropsychology, IRCCS Mondino Foundation, Pavia, Italy., Cotta Ramusino M; Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.; Unit of Behavioral Neurology, IRCCS Mondino Foundation, Pavia, Italy., Ceroni M; Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.; Department of Neurology, IRCCS Mondino Foundation, Pavia, Italy., Micieli G; Department of Emergency Neurology, IRCCS Mondino Foundation, Pavia, Italy., Sinforiani E; Laboratory of Neuropsychology, IRCCS Mondino Foundation, Pavia, Italy., D'Angelo E; Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.; Brain Connectivity Center, IRCCS Mondino Foundation, Pavia, Italy., Costa A; Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.; Unit of Behavioral Neurology, IRCCS Mondino Foundation, Pavia, Italy., Gandini Wheeler-Kingshott CAM; Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.; Brain Connectivity Center, IRCCS Mondino Foundation, Pavia, Italy.; NMR Research Unit, Department of Neuroinflammation, Queen Square MS Centre, UCL Queen Square Institute of Neurology, Faculty of Brain Sciences, University College London, London, United Kingdom.
Jazyk: angličtina
Zdroj: Frontiers in aging neuroscience [Front Aging Neurosci] 2020 Nov 26; Vol. 12, pp. 593526. Date of Electronic Publication: 2020 Nov 26 (Print Publication: 2020).
DOI: 10.3389/fnagi.2020.593526
Abstrakt: Background: Frontotemporal Spectrum Disorder (FTSD) and Amyotrophic Lateral Sclerosis (ALS) are neurodegenerative diseases often considered as a continuum from clinical, epidemiologic, and genetic perspectives. We used localized brain volume alterations to evaluate common and specific features of FTSD, FTSD-ALS, and ALS patients to further understand this clinical continuum.
Methods: We used voxel-based morphometry on structural magnetic resonance images to localize volume alterations in group comparisons: patients (20 FTSD, seven FTSD-ALS, and 18 ALS) versus healthy controls (39 CTR), and patient groups between themselves. We used mean whole-brain cortical thickness ( C T ¯ ) to assess whether its correlations with local brain volume could propose mechanistic explanations of the heterogeneous clinical presentations. We also assessed whether volume reduction can explain cognitive impairment, measured with frontal assessment battery, verbal fluency, and semantic fluency.
Results: Common (mainly frontal) and specific areas with reduced volume were detected between FTSD, FTSD-ALS, and ALS patients, confirming suggestions of a clinical continuum, while at the same time defining morphological specificities for each clinical group (e.g., a difference of cerebral and cerebellar involvement between FTSD and ALS). C T ¯ values suggested extensive network disruption in the pathological process, with indications of a correlation between cerebral and cerebellar volumes and C T ¯ in ALS. The analysis of the neuropsychological scores indeed pointed toward an important role for the cerebellum, along with fronto-temporal areas, in explaining impairment of executive, and linguistic functions.
Conclusion: We identified common elements that explain the FTSD-ALS clinical continuum, while also identifying specificities of each group, partially explained by different cerebral and cerebellar involvement.
Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
(Copyright © 2020 Pizzarotti, Palesi, Vitali, Castellazzi, Anzalone, Alvisi, Martinelli, Bernini, Cotta Ramusino, Ceroni, Micieli, Sinforiani, D’Angelo, Costa and Gandini Wheeler-Kingshott.)
Databáze: MEDLINE