| Autor: |
Antonioni A; Unit of Clinical Neurology, Neurosciences and Rehabilitation Department, University of Ferrara, 44121 Ferrara, Italy.; Doctoral Program in Translational Neurosciences and Neurotechnologies, University of Ferrara, 44121 Ferrara, Italy., Raho EM; Unit of Clinical Neurology, Neurosciences and Rehabilitation Department, University of Ferrara, 44121 Ferrara, Italy., Lopriore P; Neurological Institute, Department of Clinical and Experimental Medicine, University of Pisa, 56126 Pisa, Italy., Pace AP; Institute of Radiology, Department of Medicine, University of Udine, University Hospital S. Maria della Misericordia, Azienda Sanitaria-Universitaria Friuli Centrale, 33100 Udine, Italy., Latino RR; Complex Structure of Neurology, Emergency Department, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Casa Sollievo della Sofferenza, 71013 San Giovanni Rotondo, Italy., Assogna M; Centro Demenze, Policlinico Tor Vergata, University of Rome 'Tor Vergata', 00133 Rome, Italy.; Non Invasive Brain Stimulation Unit, Istituto di Ricovero e Cura a Carattere Scientifico Santa Lucia, 00179 Rome, Italy., Mancuso M; Neurological Institute, Department of Clinical and Experimental Medicine, University of Pisa, 56126 Pisa, Italy., Gragnaniello D; Nuerology Unit, Neurosciences and Rehabilitation Department, Ferrara University Hospital, 44124 Ferrara, Italy., Granieri E; Unit of Clinical Neurology, Neurosciences and Rehabilitation Department, University of Ferrara, 44121 Ferrara, Italy., Pugliatti M; Unit of Clinical Neurology, Neurosciences and Rehabilitation Department, University of Ferrara, 44121 Ferrara, Italy., Di Lorenzo F; Non Invasive Brain Stimulation Unit, Istituto di Ricovero e Cura a Carattere Scientifico Santa Lucia, 00179 Rome, Italy., Koch G; Non Invasive Brain Stimulation Unit, Istituto di Ricovero e Cura a Carattere Scientifico Santa Lucia, 00179 Rome, Italy.; Iit@Unife Center for Translational Neurophysiology, Istituto Italiano di Tecnologia, 44121 Ferrara, Italy.; Section of Human Physiology, Neurosciences and Rehabilitation Department, University of Ferrara, 44121 Ferrara, Italy. |
| Abstrakt: |
Frontotemporal dementia (FTD) is a neurodegenerative disease of growing interest, since it accounts for up to 10% of middle-age-onset dementias and entails a social, economic, and emotional burden for the patients and caregivers. It is characterised by a (at least initially) selective degeneration of the frontal and/or temporal lobe, generally leading to behavioural alterations, speech disorders, and psychiatric symptoms. Despite the recent advances, given its extreme heterogeneity, an overview that can bring together all the data currently available is still lacking. Here, we aim to provide a state of the art on the pathogenesis of this disease, starting with established findings and integrating them with more recent ones. In particular, advances in the genetics field will be examined, assessing them in relation to both the clinical manifestations and histopathological findings, as well as considering the link with other diseases, such as amyotrophic lateral sclerosis (ALS). Furthermore, the current diagnostic criteria will be explored, including neuroimaging methods, nuclear medicine investigations, and biomarkers on biological fluids. Of note, the promising information provided by neurophysiological investigations, i.e., electroencephalography and non-invasive brain stimulation techniques, concerning the alterations in brain networks and neurotransmitter systems will be reviewed. Finally, current and experimental therapies will be considered. |
