Frontotemporal dementia

Frontotemporal dementia (FTD) is an umbrella term reflecting the clinical syndromes of a range of diseases that cause frontotemporal lobar degeneration (FTLD), which is characterized by selective atrophy of the frontal and/or temporal cortices.

Terminology

Previously, Pick's disease has been used as an alternative term for frontotemporal dementia ². 

Epidemiology

Frontotemporal dementia is a major cause of young-onset dementia with most patients (60%) being diagnosed between 45-60 years and 10% being diagnosed <45 years old. Prevalence is estimated at between 1-26 cases per 100,000 population ². 

Clinical presentation

Patients with frontotemporal dementia present with changes in behavior, executive function and/or language ^1,2.

Pathology

The major clinical syndromes of frontotemporal dementia include (N.B. variable nomenclature) ^1-3:

• behavioral variant frontotemporal dementia
• primary progressive aphasia (a.k.a. language variant frontotemporal dementia)
  • semantic variant primary progressive aphasia
  • non-fluent variant primary progressive aphasia
• overlap syndromes
  • progressive supranuclear palsy
  • corticobasal syndrome
  • FTD with motor neuron disease

Radiographic features

Frontotemporal dementia is characterized by selective atrophy of the frontal and/or temporal cortices, with frontoinsular atrophy considered to be particularly indicative of the diagnosis ². Please see specific variant articles for further details. 

History and etymology

Frontotemporal dementia was first described by Arnold Pick (1851–1924), Czech psychiatrist, neurologist and neuropathologist, in 1892 ^3,4. 

Differential diagnosis

Clinically, behavior and language changes can mimic other diseases ²:

• psychiatric disorders, e.g. obsessive-compulsive disorder, bipolar disease, schizophrenia, personality disorders
• other neurodegenerative diseases, e.g. Alzheimer disease, dementia with Lewy bodies