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What progressive aphasia says about its neuropathology

The primary progressive aphasia (PPA) syndromes are heterogeneous neurodegenerative disorders. The 3 major PPA syndromes are not identical to the usual aphasia syndromes from strokes. Progressive nonfluent aphasia (PNFA) involves effortful speech with agrammatism and frequent apraxia of speech. The semantic variant or semantic dementia (SemD) involves fluent speech with loss of word and object meaning. Logopenic progressive aphasia (LPA), the third and most recently described PPA, involves word-finding pauses, moderate anomia, and impaired repetition of sentences. Each PPA syndrome correlates with a specific patterns of focal atrophy, but has a less robust relationship with the underlying neuropathology. An important reason for clarifying the relationship between clinical syndrome and neuropathology is that it facilitates clinical trials with disease-modifying therapies.

In this issue of Neurology ®, Deramecourt et al.1 have decreased the gap between the clinical syndromes of PPA and their neuropathology. This clinicopathologic study investigated 18 patients followed in the Lille Memory Clinic over a 15-year period. These patients presented with progressive …