Fronto-temporal lobar degeneration - advanced semantic variant primary progressive aphasia


This patient initially presented with symtoms suggestive of a behavioral variant fronto-temporal dementia, although word finding difficulty was his earliest clinical manifestation and a language-variant fronto-temporal dementia was considered within the differential diagnosis.

Over a follow-up period of 4 years, expressive and receptive language impairment emerged as the dominant phenotype and source of occupational dysfunction. A formal diagnosis of semantic demantia was strongly supported by neuropsychological testing, with clear asymmetric fronto-temporal lobar degeneration on both structural and functional neuroimaging.

This case illustrates the potential for symptom cross-over between different FTLD variants, especially early in the course of illness, with a final diagnosis emerging only in the setting of longitudinal multidisciplinary assessment.