PPA is a clinical condition that occurs as part of neurodegeneration of a large-scale, distributed, neuronal network for language. Almost every PPA patient has his/her own peculiarities, and the systematic study of this condition over the past years has led to the identification of different disease presentations, in a close connection with the recent discoveries in the field of imaging, genetic, and pathologic biomarkers. The study of the neuropsychiatric aspects associated with degenerative diseases, aside language and neuropsychological characterization, has been increasingly considered as playing a vital role in the diagnosis and management of such diseases. In a time where an accurate diagnosis can be reached in vivo through the use of advanced biochemical methods to assess the cerebrospinal fluid, as well as sophisticated brain imaging techniques with specific ligands to Ap or tau, both reflecting underlying pathology and orienting to precise treatment, the value of identification of behavior changes early in disease course may be placed in question.
As far as PPA is concerned, it is being increasingly acknowledged that the presence of behavioral ancillary features may further characterize each PPA variant. Changes in personality/behavior represent a specific clinical marker of typical frontal lobe syndromes. The introduction of a neuropsychiatric assessment, together with the performance on neuropsychological tests, may enhance the clinical diagnosis and help with the classification of PPA into subtypes, with implications in a probable underlying pathology targeted by specific drug agents. From a clinical standpoint, such symptoms that are often reported by caregivers may be the cause of major distress and often require an adequate, precise management which is nonetheless specific and may help to anticipate and program future care for these patients.