To describe a case report of a patient who had been diagnosed Behavioral Variant Frontotemporal Dementia (bvFTD) in our department, and literature review.

Internet-based literature search using PubMed, Google Scholar and UpToDate databases. The search terms included were: “Frontotemporal Dementia”, “Behavioral Variant Frontotemporal Dementia”, 'Frontotemporal Lobar Degeneration”.

52 years-old female patient without previous psychiatric history. 2 years before the psychiatric interview is described a progressive change of behavior, including apathy, inertia, dependency of her husband to perform domestic work, decreased social engagement and emotional detachment. About 2 weeks before the clinical interview, and with associated urinary complaints, it is referred cleaning rituals causing significant distress and interfering seriously with daily life, hypochondriacal concerns surrounding the urinary complaints, food restriction and food fads, and mental rigidity. Finally, 1 week before the interview, paranoid delusions and intensification of the impulsive behavior with verbal and physical aggression to strangers and her husband.

Frontotemporal dementia (FTD) is the second most common young-onset dementia and is clinically characterised by disturbances in behavior, personality, executive functions and/or language. FTD represents a heterogeneous group of different clinical syndromes. The bvFTD is the most common presentation of FTD, with progressive change in personality and behavior. The common differential diagnoses to take into consideration are the psychiatric disorders: depression, obsessive-compulsive disorder and bipolar disorders. The diagnosis is made primarily on the basis of clinical features. Pharmacologic and nonpharmacologic therapy is aimed to control behavioral symptoms’, once there’s no effective disease modifying treatment.