Pick's disease

Frontotemporal dementia (FTD), frontotemporal degeneration disease, or frontotemporal neurocognitive disorder encompasses several types of dementia involving the progressive degeneration of the brain's frontal and temporal lobes. FTDs broadly present as behavioral or language disorders with gradual onsets.

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. 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. Men and women appear to be equally affected. FTD is the second most prevalent type of early onset dementia after Alzheimer's disease. Currently, there is no cure and no approved treatments to alleviate symptoms, although some off-label drugs and behavioral methods are prescribed.

Each FTD subtype is relatively rare. FTDs are mostly early onset syndromes linked to frontotemporal lobar degeneration (FTLD), 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. 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).

Features of FTD were first described by Arnold Pick between 1892 and 1906. The name Pick's disease was coined in 1922. This term is now reserved only for the behavioral variant of FTD which shows the presence of the characteristic Pick bodies and Pick cells first described by Alois Alzheimer in 1911.

Source: Wikipedia