Frontotemporal dementia (FTD) was diagnosed in 74 outpatients with a standa
rdized assessment including neuropsychological tests, behavioural scale, st
ructural and functional imaging. Clinical characteristics were consistent w
ith the literature data. The cohort was followed for 2-6 years to determine
the reliable variable for evaluating the progression of FTD, Every fourth
patient died after a mean duration of 7 years. At first, FTD manifests itse
lf in behavioural changes with relatively stable global cognition although
language, verbal fluency and memory tests were reliable tools to follow the
progression of the disease. Below 18 of Mini-Mental State Examination, mut
ism and apathy prevented from neuropsychological testing within the next 6
months, Behavioural disorders evolved with time but restlessness and hypero
rality were long-lasting, Imaging showed the progression of a consistent pa
ttern of anterior abnormalities with frequent leukoaraiosis.