{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T08:38:29Z","timestamp":1790671109484,"version":"4.1.0"},"reference-count":29,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2013,9,19]],"date-time":"2013-09-19T00:00:00Z","timestamp":1379548800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2014,3]]},"abstract":"<jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>To describe speech, neurological and imaging characteristics of a series of patients presenting with progressive spastic dysarthria as the first and predominant sign of a presumed neurodegenerative disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Participants were 25 patients with spastic dysarthria as the only or predominant speech disorder. Clinical features, pattern of <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> volume loss on voxel\u2010based morphometry and pattern of hypometabolism on F18\u2010fluorodeoxyglucose positron emission tomography (<jats:styled-content style=\"fixed-case\">FDG<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">PET<\/jats:styled-content>) scan are described.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>All patients demonstrated speech characteristics consistent with spastic dysarthria, including strained voice quality, slow speaking rate, monopitch and monoloudness, and slow and regular speech alternating motion rates. Eight patients did not have additional neurological findings on examination. Pseudobulbar affect, upper motor neuron pattern limb weakness, spasticity, Hoffman sign and positive Babinski reflexes were noted in some of the remaining patients. Twenty\u2010three patients had electromyographic assessment and none had diffuse motor neuron disease or met El Escorial criteria for amyotrophic lateral sclerosis. Voxel\u2010based morphometry revealed striking bilateral white matter volume loss affecting the motor cortex (<jats:styled-content style=\"fixed-case\">BA<\/jats:styled-content> 4), including the frontoparietal operculum (<jats:styled-content style=\"fixed-case\">BA<\/jats:styled-content> 43) with extension into the middle cerebral peduncle. <jats:styled-content style=\"fixed-case\">FDG<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">PET<\/jats:styled-content> showed subtle hypometabolism affecting the premotor and motor cortices in some patients, particularly in those who had a disease duration longer than 2\u00a0years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>A neurodegenerative disorder that begins focally with spastic dysarthria due to involvement of the motor and premotor cortex and descending corticospinal and corticobulbar pathways is characterized. The descriptive label \u2018progressive spastic dysarthria\u2019 to best capture the dominant presenting feature of the syndrome is proposed.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.12271","type":"journal-article","created":{"date-parts":[[2013,9,21]],"date-time":"2013-09-21T08:53:56Z","timestamp":1379753636000},"page":"368-376","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["Clinical and imaging characterization of progressive spastic dysarthria"],"prefix":"10.1111","volume":"21","author":[{"given":"H. M.","family":"Clark","sequence":"first","affiliation":[{"name":"Division of Speech Pathology Department of Neurology Mayo Clinic Rochester MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. 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