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Frontotemporal dementia

Last updated: February 23, 2021

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Frontotemporal dementia (FTD) is a progressive neurodegenerative disease of the frontal and/or temporal lobe generally caused by mutations to proteins in the brain (e.g., Tau, progranulin). Pick disease, formerly used synonymously with FTD, is actually a specific subtype of FTD that can only be diagnosed pathologically; therefore, the two terms are not synonymous. Onset usually occurs in middle-aged individuals (40–60 years). Early symptoms are characterized by inappropriate social behavior (e.g., disinhibition, apathy), but patients typically continue to exhibit normal intelligence and orientation. As the disease progresses, patients may develop motor deficits and akinetic parkinsonism as an end‑stage syndrome. Diagnosis of FTD is based on a combination of factors, including the clinical criteria of dementia and possible frontotemporal brain atrophy on CT or MRI. Further diagnostic procedures may be considered to rule out other possible causes of dementia. No effective causal treatment for FTD exists and symptomatic treatment is limited to associated symptoms (e.g., depression). The disease usually has a fatal outcome within six years.

  • Heterogeneous group of syndromes that involve degeneration of the frontal, insular, and/or temporal cortices and manifest with a number of symptoms of dementia.
  • FTD is sometimes still referred to as Pick disease, but these terms are not synonymous and should not be confused with one another.
  • FTD syndromes are often considered special types of Alzheimer disease (however, initial symptoms are typically not memory‑related).

References:[1]

  • Age of onset: usually between 40 and 60 years; (typically younger than in Alzheimer disease)
  • Prevalence: 3–4/100,000 in patients ≤ 65 years

Epidemiological data refers to the US, unless otherwise specified.

References:[1]

  • Behavioral variant frontotemporal dementia (bvFTD): most frequent subtype (∼ 50% of FTD cases)
  • Two variants of primary progressive aphasia (PPA):
    • Progressive nonfluent aphasia (PNFA; also known as nonfluent agrammatic variant primary progressive aphasia)
    • Semantic dementia (SD; fluent type, also known as semantic variant primary progressive aphasia)
  • Posterior cortical atrophy (PCA)
  • Corticobasal syndrome (CBS)

General features of FTD

  • Early changes in personality and behavior inability to observe social etiquette
    • Apathy
    • Disinhibition (e.g., hypersexual behavior)
    • Exaggerated emotional display
    • Irritability
    • Binge eating (particularly of sweet foods)
  • Changes in cognitive functioning
  • Motor deficits

Patients with FTD display changes of personality and social behavior, but their memory generally remains intact.

Characteristic features FTD syndromes

  • bvFTD: : early changes in personality; apathy
  • PNFA: language impairment (e.g., grammatical errors, apraxia)
  • SD: language impairment (specifically comprehension)
  • PCA: visual disorders (e.g., blurred vision)
  • CBS: asymmetric kinetic movement disorder

The diagnosis of FTD syndromes is based on a combination of factors:

Pathological changes in FTD are commonly referred to as frontotemporal lobar degeneration (FTLD).

Macroscopic

  • Atrophy of the temporal and/or frontal lobes
  • Often beginning on one side and subsequently affecting both hemispheres

Microscopic

References:[1]

See “Differential diagnosis of dementia subtypes.”

The differential diagnoses listed here are not exhaustive.

At the moment, no curative treatment is available.

  • Patients with FTD survive an average of six years after symptom onset (progression is usually faster than in Alzheimer disease).
  • A common cause of death is intercurrent infection (e.g., pneumonia).

References:[3]

  1. Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. McGraw-Hill Education ; 2015
  2. Manoochehri M, Huey ED. Diagnosis and management of behavioral issues in frontotemporal dementia. Curr Neurol Neurosci Rep. 2012; 12 (5): p.528-536. doi: 10.1007/s11910-012-0302-7. . | Open in Read by QxMD
  3. Onyike CU, Diehl-Schmid J. The epidemiology of frontotemporal dementia. Int Rev Psychiatry. 2013; 25 (2): p.130-137. doi: 10.3109/09540261.2013.776523 . | Open in Read by QxMD