Frontotemporal dementia

Frontotemporal dementia
Other namesFTD, Frontotemporal Degeneration, Frontal Lobe Dementia, Pick's disease
FTD primarily affects the Frontal and Temporal lobes of the brain.
SpecialtyPsychiatry, neurology
Usual onsetCommonly 45-64 years, but can occur earlier
Causesfrontotemporal lobar degeneration
Risk factorsFamily history of FTD or ALS; risk-associated mutations of GRN, MAPT, C9orf72 genes, and other less-common genes; ALS diagnosis; environmental risk factors currently unknown
Diagnostic methodClinical diagnosis of exclusion based on progressive behavioral, cognitive, communication, or movement-based symptoms, with no other explanation. Supportive evidence from neuroimaging or genetic testing. Confirmed diagnosis via brain autopsy.
Differential diagnosisAlzheimer's disease, Parkinsons' disease, Lewy body dementia, Vascular dementia, Mild cognitive impairment, Depression, Generalized anxiety disorder, other mental disorders
TreatmentAs no cures or disease-modifying treatments have been approved, symptom management is the primary focus for FTD. Person-centered care is often used to address heterogeneous symptoms that vary between people.
MedicationNo approved drugs exist, but medications may be prescribed to manage symptoms.
PrognosisLife expectancy is highly variable but is typically 7-13 years.
FrequencyIn 2011 in the United States, 50,000-60,000 [1]

Frontotemporal dementia (FTD), also called frontotemporal degeneration disease[2] or frontotemporal neurocognitive disorder,[3] encompasses several types of dementia involving the progressive degeneration of the brain's frontal and temporal lobes.[4] Men and women appear to be equally affected.[2] FTD generally presents as a behavioral or language disorder with gradual onset.[5] Signs and symptoms tend to appear in mid adulthood, typically between the ages of 45 and 65, although it can affect people younger or older than this.[2] There is currently no cure or approved symptomatic treatment for FTD, although some off-label drugs and behavioral methods are prescribed.[2]

Features of FTD were first described by Arnold Pick between 1892 and 1906.[6] The name Pick's disease was coined in 1922.[7] This term is now reserved only for the behavioral variant of FTD, in which characteristic Pick bodies and Pick cells are present.[8][9] These were first described by Alois Alzheimer in 1911.[7] Common signs and symptoms include significant changes in social and personal behavior, disinhibition, apathy, blunting and dysregulation of emotions, and deficits in both expressive and receptive language.[10]

Each FTD subtype is relatively rare.[11] FTDs are mostly early onset syndromes linked to frontotemporal lobar degeneration (FTLD),[12] which is characterized by progressive neuronal loss predominantly involving the frontal or temporal lobes, and a typical loss of more than 70% of spindle neurons, while other neuron types remain intact.[13] The three main subtypes or variant syndromes are a behavioral variant (bvFTD) previously known as Pick's disease, and two variants of primary progressive aphasia (PPA): semantic (svPPA) and nonfluent (nfvPPA). Two rare distinct subtypes of FTD are neuronal intermediate filament inclusion disease (NIFID) and basophilic inclusion body disease (BIBD). Other related disorders include corticobasal syndrome (CBS or CBD), and FTD with amyotrophic lateral sclerosis (ALS).

  1. ^ Knopman, David (2011-05-17). "Estimating the Number of Persons with Frontotemporal Lobar Degeneration in the US Population". Journal of Molecular Neuroscience. 45 (3): 330–335. doi:10.1007/s12031-011-9538-y. PMC 3208074. PMID 21584654.
  2. ^ a b c d Olney NT, Spina S, Miller BL (May 2017). "Frontotemporal dementia". Neurologic Clinics. 35 (2): 339–374. doi:10.1016/j.ncl.2017.01.008. PMC 5472209. PMID 28410663.
  3. ^ Diagnostic and statistical manual of mental disorders: DSM-5 (5th ed.). Arlington, Virginia: American Psychiatric Association. 2013. pp. 614–618. ISBN 978-0-89042-554-1.
  4. ^ "ICD-11 – Mortality and Morbidity Statistics". icd.who.int. World Health Organization. Archived from the original on 2018-08-01.
  5. ^ Sivasathiaseelan H, Marshall CR, Agustus JL, et al. (April 2019). "Frontotemporal Dementia: A Clinical Review". Seminars in Neurology. 39 (2): 251–263. doi:10.1055/s-0039-1683379. PMID 30925617. S2CID 88481297.
  6. ^ Cite error: The named reference Mikol2018 was invoked but never defined (see the help page).
  7. ^ a b Cite error: The named reference Pearce2003 was invoked but never defined (see the help page).
  8. ^ Ropper AH, Samuels MA, Klein JP (2019). Adams and Victor's Principles of Neurology (11th ed.). McGraw Hill. p. 1096. ISBN 978-0-07-184262-4.
  9. ^ Cite error: The named reference Cardarelli2010 was invoked but never defined (see the help page).
  10. ^ "Frontotemporal dementia". hopkinsmedicine.org. Johns Hopkins Medicine. 2024. Retrieved 23 March 2024.
  11. ^ Borroni B, Graff C, Hardiman O, et al. (March 2022). "FRONTotemporal dementia Incidence European Research Study-FRONTIERS: Rationale and design". Alzheimer's & Dementia. 18 (3): 498–506. doi:10.1002/alz.12414. PMC 9291221. PMID 34338439.
  12. ^ Cite error: The named reference Hofmann was invoked but never defined (see the help page).
  13. ^ Chen I (June 2009). "Brain Cells for Socializing". Smithsonian Magazine. Smithsonian. Retrieved 30 October 2015.
This article is issued from Wikipedia. The text is available under Creative Commons Attribution-Share Alike 4.0 unless otherwise noted. Additional terms may apply for the media files.