AUTOSOMAL-DOMINANT PROGRESSIVE SYNDROME OF MOTOR-SPEECH LOSS WITHOUT DEMENTIA

This patient report describes a 68-year-old man with progressive disso lution in motor-speech without concomitant language or cognitive decli ne, with presumed autosomal dominant inheritance. Motor-speech impairm ents included marked difficulty in articulating words and in coordinat ing articulation, phonation, and respiration. Brain imaging results re vealed 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 tomog raphy) brain imaging studies. The involved neural substrate represente d the primary motor cortex, premotor cortex (supplementary motor area) , and the postcentral gyrus. Familial history included similar difficu lties in his mother, her sister, and his own sister. Tile isolated inv olvement of the motor-speech processes alone indicated that this syndr ome was distinguishable from progressive aphasia associated with promi nent loss of language and from Alzheimer's disease.