Frontotemporal dementia (FTD) is a progressive neurodegenerative disease especially sporadic. About 30–40% have positive family history, with an identifiable genetic mutation in a percentage of cases increasing. Although the FTD psychosis has been recognized for many years, it is not included in the clinical criteria.

To assess the prevalence and characteristics of psychotic symptoms in FTD, compare the presence of psychosis in FTD C9+ versus C9− and analyze the occurrence of wrong diagnoses in FTD with psychosis.

Literature review, using computerized databases (Pubmed®). Articles were selected based on the content of their abstract and their relevance.

It is frequently the presence of psychotic symptoms in FTD associated with C9+ versus C9−. These may arise as initial symptom often leading to a psychiatric diagnosis years before obtaining diagnosis of FTD. There is no conclusive evidence about the anatomical correlation of psychotic features in the FTD, although there is the possible association with the right brain degeneration.

The existence of psychotic symptoms do not argues against the diagnosis of FTD verifying a high frequency of psychosis in FTD – C9+. As can be the first symptom in FTD is critical to differentiate psychiatric disorders. Further studies are needed in order to obtain a better characterization of psychotic symptoms in FTD – C9+.

The authors have not supplied their declaration of competing interest.