factors Frontotemporal dementia identifies a diverse band of circumstances that collectively certainly are a main reason behind young starting point dementia Frontotemporal dementia makes selective human brain atrophy relating to the frontal and temporal lobes requiring human brain magnetic resonance imaging for accurate medical diagnosis Clinically these illnesses present chiefly seeing that progressive aphasia or seeing that disintegration of character and behaviour which may be misdiagnosed being a psychiatric disorder Up to around 25 % of situations arise from dominant mutations in another of three main causative genes Frontotemporal dementia is often connected with other neurological impairment specifically parkinsonism or electric motor neurone disease Treatment remains to be supportive but sufferers Crizotinib and families want extensive counselling potential preparation and involvement of social Crizotinib LDOC1L antibody and mental health services Frontotemporal 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. extensive counselling future planning and involvement of interpersonal and mental health services Frontotemporal dementia (FTD) is usually 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.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. 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. FTD is Crizotinib usually substantially less common than Alzheimer’s disease with estimates of populace prevalence ranging from four to 15 per 100?000 before Crizotinib age 65 years in European and US epidemiological studies.1 However Crizotinib this disease group is of disproportionate importance as a cause of young onset dementia and all the attendant socioeconomic and human costs that entails. 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. Here we provide a general overview of FTD emphasising clinical aspects and highlighting recent progress and prospects. Physique 1?1 provides a schematic overview of FTD showing major clinical and neuroanatomical syndromes and diseases and the relations between them. Fig 1?Molecular pathologies and phenotypic correlations in frontotemporal dementia. The schematic shows major genes causing frontotemporal dementia histopathological substrates and clinical phenotypes. Neuroanatomical profiles are shown as coronal … Sources and selection criteria We examined recent literature on frontotemporal dementia targeting full text English language studies published since 1990. We selected articles on the basis of our personal knowledge and searches of the Medline database using the terms “frontotemporal dementia ” “frontotemporal lobar degeneration ” “progressive nonfluent aphasia ” “semantic dementia ” and “logopenic aphasia ” and each of these terms in conjunction with “diagnosis ” “treatment ” and “therapy.” The final selection of recommendations was based on our judgment of relevance completeness and compatibility with recent clinical pathological and genetic criteria. What are the different syndromes of frontotemporal dementia? There are three main clinical syndromes of FTD defined on the basis of leading features at presentation. About half of cases present with behavioural change (behavioural variant frontotemporal dementia) and the remainder present with language decline (primary progressive aphasia) characterised either by impaired speech production (progressive non-fluent aphasia) or by impaired word comprehension and semantic memory (that is memory for meaning) (semantic dementia). There is variable overlap clinically between the syndromes and atypical parkinsonism and motor neurone disease. New consensus diagnostic criteria for FTD5 and the progressive aphasias6 have recently been formulated but they are likely to be refined as more specific information about disease pathophysiology arises and neuroimaging and other techniques that can capture pathophysiological changes become available. Methods for bedside assessment of behavioural variant frontotemporal dementia and the progressive aphasias are presented in the table?table and figure 2?2 respectively. It is particularly important for the nonspecialist to have a workable framework for suspecting FTD as diagnosis particularly early in the course of the disease is usually often challenging. In contrast to Alzheimer’s disease (the most common cause of dementia in later life) FTD often presents in middle life and memory and navigational skills and Crizotinib other aspects of general intellect are often well maintained initially. Behavioural or personality changes may at first suggest a primary psychiatric disorder particularly if accompanied by psychotic features: clues that such features are harbingers of FTD may include a lack of any prior psychiatric history and emergence of certain specific symptoms such as changes in eating behaviour or interpersonal faux pas. Isolated language disturbances.