A referred cohort of 67 clinically defined PPA patients were
compared to 99 AD patients with formal language and nonverbal
cognitive tests in a case control design. Language fluency was
determined at the first and last follow up visits. Quantitation
of sulcal and ventricular atrophy on MRI was carried out in
46 PPA and 53 AD patients. Most PPA patients (57%) are relatively
fluent when first examined. Visuospatial and memory functions
are initially preserved. Aphemic, stuttering, “pure
motor” presentation, or agrammatic aphasia are seen less
frequently. Later most PPAs become logopenic and nonfluent,
even those with semantic aphasia (dementia). In contrast, AD
patients were more fluent and had relatively lower comprehension,
but better overall language performance. MRI showed significant
left sided atrophy in most PPA patients. Subsequent to PPA,
25 patients developed behavioral manifestations of frontotemporal
dementia and 15 the corticobasal degeneration syndrome, indicating
the substantial clinical overlap of these conditions. Language
testing, particularly fluency scores supported by neuroimaging
are helpful differentiating PPA from AD. The fluent–nonfluent
dichotomy in PPA is mostly stage related. The
aphemic-logopenic-agrammatic and semantic distinction is useful,
but the outcomes converge. (JINS, 2003, 9,
710–719.)