Slowly progressive aphasia: a four-year follow-up study.

This paper reports the long-term follow-up of GC, a patient with primary progressive aphasia of the fluent type. GC presented at onset with an anomia characterized by sparing of first letter knowledge, that applied mainly to proper names and living categories. No semantic deficits were observed in the first stage of the disease, and MRI showed a left temporal lobe atrophy with a gradient from the pole to the posterior regions, the latter being less involved. We now report the clinical evolution of GC from the 2nd to the 4th year of disease. As the disease progressed, the anomia became more severe and the phenomenon of first letter sparing was no longer detectable. Also semantic knowledge was gradually affected and, eventually, was dramatically lost. However, no other cognitive deficits were seen at the last examination. By that time, the temporal atrophy shown by MRI was bilateral, although still more evident on the left side.