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Frontotemporal dementia: Clinical features and diagnosis

Suzee E Lee, MD
Bruce L Miller, MD
Section Editor
Steven T DeKosky, MD, FAAN, FACP, FANA
Deputy Editor
Janet L Wilterdink, MD


Frontotemporal dementias (FTD) are a group of clinically and neuropathologically heterogeneous neurodegenerative disorders characterized by prominent changes in social behavior and personality or aphasia accompanied by degeneration of the frontal and/or temporal lobes. Some patients with FTD also develop a concomitant motor syndrome such as parkinsonism or motor neuron disease. FTD is one of the more common causes of early-onset dementia, with an average age of symptom onset in the sixth decade.

This topic will review the clinical features and diagnosis of the main clinical syndromes of FTD. The treatment of FTD and the genetics, pathology, and pathogenesis of FTD are discussed separately. (See "Frontotemporal dementia: Treatment" and "Frontotemporal dementia: Epidemiology, pathology, and pathogenesis".)


Terminology for frontotemporal dementia (FTD) has evolved over time. In 1892, Arnold Pick first described a patient with progressive aphasia and focal frontotemporal atrophy [1]. Alois Alzheimer later characterized the disorder as Pick disease, defined by distinctive round, silver-staining inclusions, called Pick bodies [2]. Subsequent clinicopathological studies determined that many patients diagnosed clinically with Pick disease did not have Pick neuropathology at autopsy, and thus Pick disease now designates a pathological diagnosis, rather than a clinical syndrome.

The term FTD has gained favor as an umbrella term for three clinical presentations: behavioral variant FTD (bvFTD) and two forms of primary progressive aphasia (PPA), the nonfluent and semantic variants. Historically, bvFTD has also been called “FTD” or “frontal variant FTD,” while semantic variant PPA has been called “temporal variant” FTD and semantic dementia. Nonfluent variant PPA is also referred to as agrammatic PPA. The third variant of PPA, the logopenic variant, is typically associated with Alzheimer pathology and is not included as one of the three clinical FTD syndromes.

The pathology underlying clinically-defined FTD is heterogeneous. The term frontotemporal lobar degeneration (FTLD) refers to this group of neuropathological diagnoses, further categorized by abnormal protein inclusions due to tau, TDP-43 (transactive response [Tar, abbreviated T] DNA binding protein 43 kDa), and other proteins. A clear distinction between clinical and neuropathological terminology is important in these disorders, since clinicopathological series have determined that clinical syndromes often fail to predict underlying pathology.

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Literature review current through: Nov 2017. | This topic last updated: Jan 06, 2016.
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