RESUMEN
BACKGROUND AND SIGNIFICANCE: PPA is the clinical syndrome that reveals a marked, progressive loss of language functions over time with relative preservation of non-linguistic cognitive functions. Patients with this syndrome eventually develop a general dementia, but the natural history, neuropathology, and etiology are poorly understood. We report a patient who suffered from progressive aphasia unaccompanied with prominent cognitive deficits for 5 years. CASE: A 64-year-old man presented with an at least a 5-year history of a progressive language disorder. The initial symptoms were a disability to speak in complete sentences, word-finding difficulty, verbal hesitancy, and resultant social withdrawal. Over the next 5-years, his symptoms slowly worsened, but non-verbal cognitive functions such as memory, visuospatial skill, and daily living activities were preserved. Eventually, the patient was mutistic, incommunicable, and impaired in general intelligence parallel with personality and mood changes like emotional lability, impersistence, or agitation. The imaging of brain revealed generalized atrophy with more prominent changes in the left temporal lobe and the degree of atrophy progressed on the follow-up study 4 years later. The brain SPECT showed hypoperfusion in the left frontotemporal area. Initially, the patient scored 25 points with Mini-Mental State Examination but worsened to 3 points 4 years later. Language examination at the onset showed mildly decreased fluency, naming, and reading. Recently, our patient has become mutistic and alexic, but has preserved some comprehension. COMMENT: Non-dementic patients with progressive language disturbances as a result of the left focal temporal atrophy from 2-year of onset, have the possibility of being diagnosed with PPA, frontotemporal degeneration, Pick disease, or Alzheimer disease. But we suggest that characteristic isolated linguistic dysfunction with preserved cognitive function in PPA may be a distinct from other degenerative diseases of dementia.