Frontotemporal dementia is a term used to characterize diverse clinical and neuropathological conditions that can present with the same clinical phenotype. Frontotemporal dementia is still under-diagnosed and underestimated, partly because the individual components of the complex are considered separately or are considered "heterogeneous," a somewhat misleading adjective used in many descriptions. The discovery of mutations in the tau gene in frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) constitutes convincing evidence that tau proteins play a key role in the pathogenesis of neurodegenerative disorders. The clinical presentation in frontotemporal dementia (FTD) reflects the distribution of the pathologic changes rather than the exact histologic subtype of the disease. Three major clinical syndromes can be identified: 1) frontal variant FTD (dementia of frontal type) in which changes in social behavior and personality predominate, reflecting the orbitobasal frontal lobe focus of the pathology; 2) semantic dementia (progressive fluent aphasia) in which there is a breakdown in the conceptual database which underlies language production and comprehension, although deficits in nonverbal semantic knowledge can also be shown on neuropsychologic testing; 3) progressive nonfluent aphasia in which the phonologic and syntactic components of language are affected in association with left peri-Sylvian atrophy. However, a substantive overlap between the clinical syndromes of frontal lobe dementia (FLD), frontotemporal dementia (FTD), or primary progressive aphasia, and corticobasal degeneration syndrome has been demonstrated. Despite of all these considerations, FTD is a very common cause of early-onset dementia than previously recognized and appears to be more common in men. Also, FTD is the second most common form of cortical dementia in the presenium after Alzheimer's disease Dementia of frontal type and motor neuron disease inclusion dementia are the most frequent neuropathological subtypes of frontotemporal lobar degeneration. Neuropsychiatric features characterize frontotemporal dementia (FTD). They include loss of emotions and insight, selfishness, disinhibition, personal neglect, gluttony and sweet food preference, wandering, motor and verbal stereotypies, loss of pain, echolalia and mutism. Paroxetine addressed anxiety and repetitive, ritualistic behaviors. Depression was resistant to treatment. Valproic acid and quetiapine calmed agitated subjects without exacerbating Parkinsonism. Donepezil, Rivastigmine and Galantamine have not emerged as a beneficial medication for this group of subjects. Agitation and hallucinations are frequently treated with neuroleptic medication. However, these patients may be particularly sensitive to the extrapyramidal side effects of neuroleptics. Therefore, neuroleptics should be used cautiously in FTD and treatment should be started at low doses avoiding depot preparations until further prospective studies have been performed. In conclusion, FTD is a complex constalation of clinical subtypes. When the diagnosis of FTD is considered, the physician must considered the clinical, neuroradiologic, cognitive and neuropsychiatric aspects, in order to discriminate an acurate diagnosis. Unfortunetly, treatment is still far away from a rational outcome and further research it is necessary.
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