INFAD (May 2018)
Frontotemporal dementia: diagnostic borders.
Abstract
Frontotemporal dementia (FTD) has a lower prevalence than Alzheimer Disease (AD), but its age of onset takes place earlier, between 50 and 60 years. For this reason, it supposes a great overload for caregivers and an increasing dependence for patients over a long period of time. FTD comprises a set of neurodegenerative diseases that affect different cerebral cortex areas and the nature of symptoms on each clinical syndrome will depend on the areas affected during each of the different stages of the disease. Thus, a behavioral variant, predominantly frontal and three primary progressive aphasia variants, named non-fluent, semantic and logopenic, all of them predominantly temporal, are described. Their study is particularly relevant in the field of neuropsychology because initially these dementias affect the neural networks supporting cognition and social-adjustment, language and semantic knowledge of the world. The great variety of symptoms along with its heterogenic nature determine that at the onset of the disease and during its evolution, FTD can be confused with AD or other pathologies. The aim of this work is to review its main characteristics and diagnosis boundaries in order to provide useful tools for differential diagnosis. FTD etiology is related to structural changes in the proteins responsible for maintaining the cytoskeleton of neurons, gene expression regulation and cell growth. Recently, new advances in the study of biomarkers, genetics and neuroimage diagnosis of FTD have made it possible to clarify the diagnostic criteria of the different FTD subtypes, are helping to define the boundaries with AD and are allowing to establish a neuropathological relationship with other clinical syndromes including atypical parkinsonism spectrum, amyotrophic lateral sclerosis and apraxia of speech.
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