Primary Progressive Aphasia in Nondementing Adults

Primary progressive aphasia (PPA) is a behavioral syndrome characterized by gradual deterioration of speech and language functions in a setting of initially preserved intellectual and social skills. There is considerable heterogeneity in the language manifestations, but an early progressive dysnomia that may be more pronounced in spontaneous speech than on measures of confrontation naming is typically present. The pattern of language breakdown in patients with PPA does not generally conform to any of the traditional aphasia syndromes associated with focal cerebrovascular lesions, such as Broca's or Wernicke's aphasia. There are no specific laboratory or imaging markers, but asymmetric atrophy involving the perisylvian language areas on MRI is a characteristic feature. No current pharmacological treatments are effective for patients with PPA. The course is variable, with some patients surviving more than 10 years after the initial diagnosis.