Simone Pomati, MD, PhD, Centre for Research and Treatment on Cognitive Dysfunctions, Institute of Clinical Neurology, Department of Clinical Sciences, Luigi Sacco Hospital, Milan, Italy.
Francesca Clerici, MD, PhD, Centre for Research and Treatment on Cognitive Dysfunctions, Institute of Clinical Neurology, Department of Clinical Sciences, Luigi Sacco Hospital, Milan, Italy.
Stefano Defendi, MD, Centre for Research and Treatment on Cognitive Dysfunctions, Institute of Clinical Neurology, Department of Clinical Sciences, Luigi Sacco Hospital, Milan, Italy.
Silvia Bovo, MD, Centre for Research and Treatment on Cognitive Dysfunctions, Institute of Clinical Neurology, Department of Clinical Sciences, Luigi Sacco Hospital, Milan, Italy.
Claudio Mariani, MD, Chair of Clinical Neurology, University of Milan; Centre for Research and Treatment on Cognitive Dysfunctions, Institute of Clinical Neurology, Department of Clinical Sciences, Luigi Sacco Hospital, Milan, Italy.

Frontotemporal dementia (FTD) is a progressive condition characterized by atrophy of the frontal and/or temporal lobes. Three main clinical syndromes have been described (behavioural variant, progressive nonfluent aphasia, and semantic dementia). The symptoms reflect the anatomical distribution of the pathological changes rather than the precise histological subtype. Frontotemporal dementia is a genetically complex disorder with a strong likelihood of inheritance, mainly transmitted as an autosomal dominant trait. Mutations in microtubule associated tau protein and progranulin have been reported in several families affected by FTD. The treatment is directed to the control of the behavioural disturbances through pharmacological and nonpharmacological approaches.
Key words: frontotemporal dementia, semantic dementia, progressive nonfluent aphasia, neuropsychology, progranulin.