Abstract
A 69-year-old right-handed man with a seven-year history of slowly progressive non-fluent aphasia characterized by marked anarthria and mild spelling dysgraphia is described. His naming ability has remained at an exceptionally high level and there has been no impairment of auditory comprehension or reading.
Non-verbal skills and memory function have also been largely preserved. MR images showed focal atrophy of the left inferior frontal gyrus and the pre-and postcentral gyri, mild dilatation of the left parietal sulci, and sparing of the temporal lobe. SPECT scans demonstrated hypoperfusion in the pars opercularis frontalis. We argue that the selective language deficits in this patient and protracted course are characteristic of aphemia and are considered typical of primary progressive aphasia (PPA) as described by Mesulam. The importance of clinical distinction between PPA and other cases of progressive aphasia preceding global dementia was emphasized.
