Objective: To evaluate the frequency and types of change in "self" seen in
frontotemporal dementia (FTD) and to determine the relative involvement of
the nondominant and dominant frontal and temporal brain regions in FTD pati
ents with or without changes in a sense of self using neuropsychology tests
and neuroimaging. Background: The self has been defined as "the total, ess
ential, or particular being of a person" involving "the essential qualities
distinguishing one person from another." Some suggest that the frontal lob
es play a dominant role in maintaining the self FTD affects anterior fronta
l and temporal areas and can be associated with a loss of self. Methods: Se
venty-two consecutive FTD patients were evaluated with neuropsychiatric, ne
uropsychologic, and behavioral measures. Patients were imaged with MRI and
SPECT. Charts were reviewed by a social psychologist to determine patients
who exhibited a dramatic change in their self as defined by changes in poli
tical, social, or religious values. The brain areas with the most severe at
rophy or hypoperfusion on neuroimaging were noted. Results: Seven of 72 pat
ients exhibited a dramatic change in self. In six of the seven, the selecti
ve dysfunction involved the nondominant frontal region. In contrast, only o
ne of the other 65 patients without selective nondominant frontal dysfuncti
on showed a change in self. Conclusions: FTD patients with asymmetric loss
of function in the nondominant frontal lobe often exhibit a diminished main
tenance of previously learned self-concepts despite intact memory and langu
age. Normal nondominant frontal function is important for the maintenance o
f the self.