Frontotemporal dementia

Frontotemporal dementia
Brain MRI of a 65-year-old woman with frontotemporal dementia. Cortical and white matter atrophy of the frontal lobes is clear in all images.
Specialty	Psychiatry, neurology
Causes	frontotemporal lobar degeneration

Frontotemporal dementia (FTD), also called frontotemporal degeneration disease^[1] or frontotemporal neurocognitive disorder,^[2] encompasses several types of dementia involving the progressive degeneration of the brain's frontal and temporal lobes.^[3] FTD is the second most prevalent type of early onset dementia after Alzheimer's disease.^{[citation needed]} Men and women appear to be equally affected.^[1] FTD generally presents as a behavioral or language disorder with gradual onset.^[4] Signs and symptoms tend to appear in late adulthood, typically between the ages of 45 and 65, although it can affect people younger or older than this.^[1] Currently, no cure or approved symptomatic treatment for FTD exists, although some off-label drugs and behavioral methods are prescribed.^[1]

Features of FTD were first described by Arnold Pick between 1892 and 1906.^[5] The name Pick's disease was coined in 1922.^[6] This term is now reserved only for the behavioral variant of FTD, in which characteristic Pick bodies and Pick cells are present.^[7][8] These were first described by Alois Alzheimer in 1911.^[6] 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.^[9]

Each FTD subtype is relatively rare.^[10] FTDs are mostly early onset syndromes linked to frontotemporal lobar degeneration (FTLD),^[11] 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.^[12] 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).