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Clinical Review

Frontotemporal dementia

BMJ 2013; 347 doi: https://doi.org/10.1136/bmj.f4827 (Published 06 August 2013) Cite this as: BMJ 2013;347:f4827
  1. Jason D Warren, Wellcome Trust senior clinical fellow and reader in neurology,
  2. Jonathan D Rohrer, clinical lecturer,
  3. Martin N Rossor, senior investigator and director
  1. 1Dementia Research Centre, UCL Institute of Neurology, University College London, London WC1N 3BG, UK
  1. Correspondence to: J D Warren jason.warren{at}ucl.ac.uk

Summary points

  • Frontotemporal dementia refers to a diverse group of conditions that collectively are a major cause of young onset dementia

  • Frontotemporal dementia produces selective brain atrophy involving the frontal and temporal lobes, requiring brain magnetic resonance imaging for accurate diagnosis

  • Clinically, these diseases present chiefly as progressive aphasia or as disintegration of personality and behaviour that may be misdiagnosed as a psychiatric disorder

  • Up to around a quarter of cases arise from dominant mutations in one of three major causative genes

  • Frontotemporal dementia is commonly associated with other neurological impairment, in particular parkinsonism or motor neurone disease

  • Treatment remains supportive, but patients and families need extensive counselling, future planning, and involvement of social 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.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 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.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. 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 …

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