Autosomal dominant progressive syndrome of motor-speech loss without dementia

Sandra Bond Chapman, Roger N. Rosenberg, Myron F. Weiner, Angela Shobe

Research output: Contribution to journalArticle

31 Scopus citations

Abstract

This patient report describes a 68-year-old man with progressive dissolution in motor-speech without concomitant language or cognitive decline, with presumed autosomal dominant inheritance. Motor-speech impairments included marked difficulty in articulating words and in coordinating articulation, phonation, and respiration. Brain imaging results revealed severe focal atrophy of the posterior frontal region extending to the anterior parietal and superior temporal regions bilaterally on structural (MRI) and functional (single photon emission computed tomography) brain imaging studies. The involved neural substrate represented the primary motor cortex, premotor cortex (supplementary motor area), and the postcentral gyrus. Familial history included similar difficulties in his mother, her sister, and his own sister. The isolated involvement of the motor-speech processes alone indicated that this syndrome was distinguishable from progressive aphasia associated with prominent loss of language and from Alzheimer's disease.

Original languageEnglish (US)
Pages (from-to)1298-1306
Number of pages9
JournalNeurology
Volume49
Issue number5
DOIs
StatePublished - Nov 1997

ASJC Scopus subject areas

  • Clinical Neurology

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