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Neurol Clin. 2017 May;35(2):339-374. doi: 10.1016/j.ncl.2017.01.008.

Frontotemporal Dementia.

Author information

1
Department of Neurology, UCSF Memory and Aging Center, San Francisco, CA, USA. Electronic address: Nicholas.Olney@ucsf.edu.
2
Department of Neurology, UCSF Memory and Aging Center, San Francisco, CA, USA.
3
Department of Neurology, UCSF Memory and Aging Center, San Francisco, CA, USA; UCSF School of Medicine, San Francisco, CA, USA.

Abstract

Frontotemporal dementia (FTD) is a heterogeneous disorder with distinct clinical phenotypes associated with multiple neuropathologic entities. Presently, the term FTD encompasses clinical disorders that include changes in behavior, language, executive control, and often motor symptoms. The core FTD spectrum disorders include behavioral variant FTD, nonfluent/agrammatic variant primary progressive aphasia, and semantic variant PPA. Related FTD disorders include frontotemporal dementia with motor neuron disease, progressive supranuclear palsy syndrome, and corticobasal syndrome. In this article, the authors discuss the clinical presentation, diagnostic criteria, neuropathology, genetics, and treatments of these disorders.

KEYWORDS:

Corticobasal syndrome (CBS); Frontotemporal dementia (FTD); Motor neuron disease; Nonfluent PPA; Primary progressive aphasia; Progressive supranuclear palsy (PSP); Semantic PPA

PMID:
28410663
PMCID:
PMC5472209
DOI:
10.1016/j.ncl.2017.01.008
[Indexed for MEDLINE]
Free PMC Article

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