![]() Neuroanatomical profiles are shown as coronal magnetic resonance imaging sections (left hemisphere displayed on the right) abutting the corresponding pathological substrates, with regions of predominant regional atrophy demarcated by white rectangles. The schematic shows major genes causing frontotemporal dementia, histopathological substrates, and clinical phenotypes. Figure 1 ⇓ provides a schematic overview of FTD showing major clinical and neuroanatomical syndromes and diseases, and the relations between them.įig 1 Molecular pathologies and phenotypic correlations in frontotemporal dementia. Here we provide a general overview of FTD, emphasising clinical aspects and highlighting recent progress and prospects. Although onset is typically in the sixth decade of life, it may begin as early as the third or as late as the ninth decade, and the prevalence of FTD in older age groups has almost certainly been underestimated. 1 However, this disease group is of disproportionate importance as a cause of young onset dementia and all the attendant socioeconomic and human costs that entails. FTD is substantially less common than Alzheimer’s disease, with estimates of population prevalence ranging from four to 15 per 100 000 before age 65 years in European and US epidemiological studies. Only in the past three decades, however, has the clinical and pathological complexity of these diseases and their unique status as examples of selective brain degeneration been fully appreciated. 1 2 3 4 Cases of FTD have been described since the late 19th century, initially most comprehensively by Arnold Pick, who lent his name to the historical designation of the entire FTD spectrum as Pick’s disease. Treatment remains supportive, but patients and families need extensive counselling, future planning, and involvement of social and mental health servicesįrontotemporal dementia (FTD) is a clinically and pathologically heterogeneous group of non-Alzheimer dementias characterised collectively by relatively selective, progressive atrophy involving the frontal or temporal lobes, or both. Up to around a quarter of cases arise from dominant mutations in one of three major causative genesįrontotemporal dementia is commonly associated with other neurological impairment, in particular parkinsonism or motor neurone disease Frontotemporal dementia refers to a diverse group of conditions that collectively are a major cause of young onset dementiaįrontotemporal dementia produces selective brain atrophy involving the frontal and temporal lobes, requiring brain magnetic resonance imaging for accurate diagnosisĬlinically, these diseases present chiefly as progressive aphasia or as disintegration of personality and behaviour that may be misdiagnosed as a psychiatric disorder ![]()
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