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Frontotemporal Degeneration

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Abstract

The frontotemporal degenerations (FTDs) are a group of neurodegenerative disorders causing dementia with or without other neurological symptoms (ALS and parkinsonism). The most common presentations are behavioral variant frontotemporal dementia with personality/mood changes and primary progressive aphasia. Approximately 40 % of FTD cases have some family history of neurological disease, with about 10 % having an autosomal dominant pattern. The 3 most common FTD genes are MAPT, GRN, and C9orf72. The age of onset and presentation of the FTDs are highly variable even within families. Capacity to consent and family dysfunction add to the complexity of counseling for these disorders.

Keywords

  • Amyotrophic Lateral Sclerosis
  • Progressive Supranuclear Palsy
  • Progressive Supranuclear Palsy
  • Primary Progressive Aphasia
  • Presymptomatic Testing

These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Fig. 8.1

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Correspondence to Jill S. Goldman .

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Frontotemporal degeneration (FTD) part 1 (MOV 296915 kb)

Frontotemporal degeneration (FTD) part 2 (MOV 371601 kb)

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Goldman, J.S. (2015). Frontotemporal Degeneration. In: Goldman, J. (eds) Genetic Counseling for Adult Neurogenetic Disease. Springer, Boston, MA. https://doi.org/10.1007/978-1-4899-7482-2_8

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