Objective: The profile of language impairment in patients with primary progressive aphasia in comparison with the language impairment in patients with Alzheimer's disease and after stroke.
Design: The Western Aphasia Battery and the Mattis Dementia Rating Scale evaluated the language and cognitive impairment. Follow-up studies were done 1 to 5 years after the initial testing in seven of 10 patients with primary progressive aphasia, and a postmortem examination of the brain was done in two patients.
Setting: All 10 patients with primary progressive aphasia were previously healthy, community-dwelling persons when first tested.
Patients and other participants: All patients with primary progressive aphasia reported at least a 2-year history of slowly progressive aphasia without other signs of global dementia. The initial Western Aphasia Battery results of the 10 patients with primary progressive aphasia were compared with those of a sample of 10 patients with probable Alzheimer's disease and with those of a sample of 10 patients with aphasia due to a left hemispheric stroke. Both reference samples were matched for age and sex; the sample with stroke-caused aphasia was additionally matched for the aphasia type.
Results: Expressive language disability with reduced speech fluency and anomia but preserved language comprehension and nonverbal cognition were typical features in early stages of primary progressive aphasia. Spontaneous speech was significantly more impaired in patients with primary progressive aphasia in comparison with those with aphasia after left hemisphere stroke and with language impairment in patients with Alzheimer's disease. Follow-up examinations revealed continuous, often rapid deterioration of language impairment. The neuropathological examination showed Alzheimer's disease in one patient and Pick's disease in the other.
Conclusion: The profile of aphasia suggests that primary progressive aphasia tends to affect anterior parts of the language-dominant cortex first.