RESUMO
BACKGROUND: Frontotemporal dementia is the third or fourth most common form of dementia in the 45-65 years age group. It causes significant morbidity as well as a six to eightfold increase in mortality risk. OBJECTIVE: This article provides an overview of the pathophysiology of frontal lobe function and the genetics of frontotemporal dementia. It also summarises the clinical features, diagnosis and management of frontotemporal dementia. DISCUSSION: While the clinical presentation of frontotemporal dementia was described as early as the nineteenth century, recent advances in genetics have resulted in greater understanding of the pathophysiology of this disease. While imaging may support the diagnosis of frontotemporal dementia, it is essentially a clinical diagnosis based on the presence of typical clinical features and the findings of neuropsychological tests. Clinical management of frontotemporal dementia remains a challenge and is largely centred on behavioural management. Pharmacological agents such as selective serotonin reuptake inhibitors and antipsychotics may be helpful, although evidence to support their use is minimal.