Frontotemporal neurodegeneration can cause three typical clinical syndromes
: frontotemporal dementia (FTD), primary progressive aphasia (PPA) and sema
ntic dementia (SD), In the present paper we review these syndromes, highlig
hting FTD, four case examples are presented. At the early stage of FTD chan
ges of personality and social conduct are prominent, whereas cognitive func
tions are relatively well preserved. Since the usual dementia tests are not
sufficiently sensitive to disclose non-cognitive symptoms, clinical diagno
sis as well as differentiation from non-organic psychiatric disorders can b
e difficult. Detailed history, thorough clinical examination, and neuropsyc
hological testing are required to establish the diagnosis, EEG and function
al brain imaging may be helpful, The choice of therapeutic options for FTD
is extremely limited. Medications may be used to treat neuropsychiatric sym
ptoms, There is little experience with non-pharmacologic behaviour modifica
tion and milieu treatment approaches. The problems that FTD imposes on care
givers are dissimilar to those arising from Alzheimer's disease. Families r
eceive little or no support so that early nursing home admission of patient
s is common.