ABSTRACT
In this chapter we describe the demographic, historical, clinical, genetic, and pathological features
of frontotemporal dementia (FTD). This group of disorders have received increasing attention over
the past few years, in part triggered by the discovery of genetic cases caused by mutations in the tau
gene. The FTD field is still young and only in the past few years have there been systematic efforts
to characterize patients with this disorder. Many issues still remain related to etiology and
nomenclature that should be clarified as more clinically characterized cases come to pathology and
as the genetic basis for FTD is better defined. FTD remains a clinically fascinating disorder, often
diagnosed as a psychiatric disturbance before it is recognized as a dementia. These disorders offer
many insights into how slow and selective loss of function in the frontal and anterior temporal lobes
modifies the psychological and psychiatric well-being of a previously normal individual. As careful
clinical and imaging studies emerge, FTD should help to understand many aspects of self-reflection,
emotion, social pragmatics, language, and executive functions.
