Primary Progressive Aphasia
OBJECTIVES
To present characteristic features of the syndrome of primary progressive aphasia (PPA).
To describe the diagnostic features of the three PPA phenotypes: progressive nonfluent aphasia, semantic dementia, and logopenic aphasia.
To name the most common etiologies associated with each of the phenotypes of the PPA syndrome.
VIGNETTE
Nine years earlier, this 72-year-old right-handed man consulted us because of difficulty “coming up with proper words.” Since then, he had progressive difficulties with several cognitive functions, most specifically forgetting names and nouns and having impaired reading. Most specific complaints consisted of his inability to name objects even though he could describe their meaning. He also frequently forgot names of relatives and friends.
CASE SUMMARY
Our patient was a Ph.D. research physiologist whom we followed clinically since 1997. Evaluation at that time indicated an anomic aphasia with normal comprehension, anterograde memory, executive function, and visual perception.
In 2000, he noted worsening ability to read and find words and to comprehend certain nouns. His wife also suspected changes on his recent memory. His judgment had also deteriorated as he engaged in risky ventures. Examination at that time demonstrated well-preserved social graces. Verbal output was largely fluent but showed worsening word-finding difficulties particularly with nouns. Speech was more empty, anomic, and circumlocutory. Occasional semantic paraphasias were also present in conversational speech.