Primary progressive aphasia: a review of 112 cases

Primary progressive aphasia (PPA) was first recognized by Mesulam in 1982. Although dozens of cases have since been described, it has been difficult to place these cases into a coherent framework due to the wide variation in measures which have been reported. We review 170 contacts with 112 patients to provide a clinical, neuroanatomical, and neuropsychological profile of patients with the disorder. The progression of the disease is analyzed over a 10-year reporting period starting from symptom onset to show how progression affects five general linguistic skills: oral and written naming, reading, repetition, and general comprehension. The pattern of functional and neurological deficits in PPA is heterogeneous. Differences in the distribution of neurological anomalies between patients with bilateral and unilateral changes suggest that there may be two separate disease processes involved.