TY - JOUR
T1 - Autosomal dominant progressive syndrome of motor-speech loss without dementia
AU - Chapman, Sandra Bond
AU - Rosenberg, Roger N.
AU - Weiner, Myron F.
AU - Shobe, Angela
PY - 1997/11
Y1 - 1997/11
N2 - 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.
AB - 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.
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U2 - 10.1212/WNL.49.5.1298
DO - 10.1212/WNL.49.5.1298
M3 - Article
C2 - 9371912
AN - SCOPUS:0030712286
SN - 0028-3878
VL - 49
SP - 1298
EP - 1306
JO - Neurology
JF - Neurology
IS - 5
ER -