Although Alzheimer’s disease is most common cause of dementia in the elderly population, there are other types of dementia. Among these, Frontotemporal Lobar Degeneration is the second most frequent neurodegenerative disease in the elderly population, but is the first disease in people under 65 years of age.
Memory is almost preserved, but other problems, related to cognitive functions, occur. The prevalence of the disease is 10/15 affected for 100.000 subjects between 45-65 years of age and is equal among men and women.
Behavioral variant of frontotemporal dementia is the most frequent FTLD phenotype, characterized by behavioral alterations, such as disinhibition, overeating, and impulsiveness, and impairment of cognitive functions. Early and progressive changes in language functions represent the alternative presentation of FTLD: progressive loss of speech and complete mutism in the advanced stages of the disease are typical of primary non-fluent aphasia (PNFA) subtype. Semantic Dementia is instead characterized by loss of knowledge about words and objects, anomia and single-word comprehension deficits.
Mutations in several genes, transmitted with autosomal dominant manner, account for about 8% of cases affected by Frototemporal Lobar Degeneration, whereas the majority of cases is sporadic, likely caused by the interaction of genetic and environmental factors.