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Disease pathophysiology:
Primary progressive aphasia (PPA) is caused by degeneration in the parts of the brain that control speech and language (the left, or ‘dominant,’ side of the brain in the frontal, temporal and parietal regions). This type of aphasia begins gradually, with speech or language symptoms that will vary depending on the brain areas affected by the disease.
Disease statistics:
Our series comprised nine females and seven males, with ages varying between 39 and 83 years (average 62.8 years) and an educational level between two and 20 years (average 10.7 years). Six patients spoke other languages besides Portuguese. Performance in language tests, repeated after a one to two year interval in seven cases.
Disease treatment:
There are no treatments available at present for PPA. Bromocriptine was used in a double-blind placebo controlled trial in six subjects with PNFA and showed a mild improvement in the mean length of utterances. There is one case report of PPA showing some improvement with the use of oral steroids. The improvement was mild and the patient received steroids for only three months.
Major research on disease:
The definition and the subtyping of PPA continue to evolve but refinement in clinical and pathological characterization is required for better diagnosis of the different clinical syndromes. In future besides structural and functional neuroimaging, cerebrospinal fluid and molecular markers may be available for better diagnosis during life.