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.
Previously, Pick's disease has been used as an alternative term for frontotemporal dementia 2.
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.
Patients with frontotemporal dementia present with changes in behavior, executive function and/or language 1,2.
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)
- overlap syndromes
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.
Clinically, behavior and language changes can mimic other diseases 2:
- 1. Warren JD, Rohrer JD, Rossor MN. Clinical review. Frontotemporal dementia. (2013) BMJ (Clinical research ed.). 347: f4827. doi:10.1136/bmj.f4827 - Pubmed
- 2. Bang J, Spina S, Miller BL. Frontotemporal dementia. (2015) Lancet (London, England). 386 (10004): 1672-82. doi:10.1016/S0140-6736(15)00461-4 - Pubmed
- 3. Olney NT, Spina S, Miller BL. Frontotemporal Dementia. (2017) Neurologic clinics. 35 (2): 339-374. doi:10.1016/j.ncl.2017.01.008 - Pubmed
- 4. Mikol, J. (2018). History of Pick’s disease. Revue Neurologique, 174(10), 740–741. https://doi.org/10.1016/j.neurol.2018.09.009