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A."},{"key":"10.1016\/j.nbd.2020.105063_bb0440","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1136\/jnnp-2018-318619","article-title":"Cortical correlates of behavioural change in amyotrophic lateral sclerosis","volume":"90","author":"Consonni","year":"2019","journal-title":"J. Neurol. Neurosurg. 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A."},{"key":"10.1016\/j.nbd.2020.105063_bb0735","doi-asserted-by":"crossref","first-page":"85","DOI":"10.3109\/21678421.2015.1074707","article-title":"Potential structural and functional biomarkers of upper motor neuron dysfunction in ALS","volume":"17","author":"Grieve","year":"2016","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"key":"10.1016\/j.nbd.2020.105063_bb0740","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1186\/1471-2377-6-17","article-title":"Widespread sensorimotor and frontal cortical atrophy in amyotrophic lateral sclerosis","volume":"6","author":"Grosskreutz","year":"2006","journal-title":"BMC Neurol."},{"key":"10.1016\/j.nbd.2020.105063_bb0745","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1162\/jocn.1996.8.2.135","article-title":"Progressive nonfluent aphasia: language, cognitive, and PET measures contrasted with probable Alzheimer's disease","volume":"8","author":"Grossman","year":"1996","journal-title":"J. Cogn. 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Neurosci."},{"key":"10.1016\/j.nbd.2020.105063_bb0925","doi-asserted-by":"crossref","first-page":"860","DOI":"10.1001\/jamaneurol.2018.0372","article-title":"Selective genetic overlap between amyotrophic lateral sclerosis and diseases of the frontotemporal dementia spectrum","volume":"75","author":"Karch","year":"2018","journal-title":"JAMA Neurol"},{"key":"10.1016\/j.nbd.2020.105063_bb0930","doi-asserted-by":"crossref","first-page":"1399","DOI":"10.1093\/brain\/116.6.1399","article-title":"The relationship between abnormalities of cognitive function and cerebral activation in amyotrophic lateral sclerosis: A neuropsychological and positron emission tomography study","volume":"116","author":"Kew","year":"1993","journal-title":"Brain"},{"key":"10.1016\/j.nbd.2020.105063_bb0935","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1136\/jnnp-2011-301883","article-title":"Atypical, slowly progressive behavioural variant frontotemporal dementia associated with C9orf72 hexanucleotide expansion","volume":"83","author":"Khan","year":"2012","journal-title":"J. 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A."},{"key":"10.1016\/j.nbd.2020.105063_bb1705","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1148\/radiol.2016151846","article-title":"Brain MR imaging in patients with lower motor neuron-predominant disease","volume":"280","author":"Spinelli","year":"2016","journal-title":"Radiology"},{"key":"10.1016\/j.nbd.2020.105063_bb1710","doi-asserted-by":"crossref","first-page":"1668","DOI":"10.1126\/science.1154584","article-title":"TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis","volume":"319","author":"Sreedharan","year":"2008","journal-title":"Science"},{"key":"10.1016\/j.nbd.2020.105063_bb1715","doi-asserted-by":"crossref","first-page":"1260","DOI":"10.1007\/s00415-006-0513-4","article-title":"Altered cortical activation during a motor task in ALS: evidence for involvement of central pathways","volume":"254","author":"Stanton","year":"2007","journal-title":"J. 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Proceedings of the 25th Annual Meeting of ISMRM, Honolulu, USA. Abstract 0028."},{"key":"10.1016\/j.nbd.2020.105063_bb1980","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1001\/archneur.64.3.371","article-title":"Voxel-based morphometry in frontotemporal lobar degeneration with ubiquitin-positive inclusions with and without progranulin mutations","volume":"64","author":"Whitwell","year":"2007","journal-title":"Arch. 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