Specialty Psychiatry , neurology Causes frontotemporal lobar degeneration Frontotemporal dementia ( FTD ), or frontotemporal neurocognitive disorder [1] encompasses several types of dementia involving the frontal and temporal lobes . [2] FTDs are broadly presented as behavioral or language disorders. [3] The three main subtypes or variant syndromes are a behavioral variant (bvFTD) previously known as Pick's disease , [4] [5] and two variants of primary progressive aphasia – semantic variant (svPPA), and nonfluent variant (nfvPPA). [3] [5] Two rare distinct subtypes of FTD are neuronal intermediate filament inclusion disease (NIFID), and basophilic inclusion body disease . Other related disorders include corticobasal syndrome and FTD with amyotrophic lateral sclerosis (ALS) FTD-ALS also called FTD- MND . [6] Frontotemporal dementias are mostly early-onset syndromes that are linked to frontotemporal lobar degeneration (FTLD), [7] which is characterized by progressive neuronal loss predominantly involving the frontal or temporal lobes , and a typical loss of over 70% of spindle neurons , while other neuron types remain intact. [8] FTD was first described by Arnold Pick in 1892 and was originally called Pick's disease , a term now reserved only for behavioral variant FTD which shows the presence of Pick bodies and Pick cells. [9] [4] [10] Second only to Alzheimer's disease (AD) in prevalence , FTD accounts for 20% of early-onset dementia cases. [11] Signs and symptoms typically manifest in late adulthood, more commonly between the ages of 45 and 65, approximately equally affecting men and women. [11] Common signs and symptoms include significant changes in social and personal behavior, apathy, blunting of emotions, and deficits in both expressive and receptive language. ... Related disorders are corticobasal syndrome, and progressive supranuclear palsy. [6] Behavioral variant frontotemporal dementia [ edit ] Behavioral variant frontotemporal dementia (BvFTD) was previously known as Pick's disease , and is the most common of the FTD types. [4] BvFTD is diagnosed four times more than the PPA variants. [13] Behavior can change in BvFTD in either of two ways — it can change to being impulsive and disinhibited, acting in socially unacceptable ways; or it can change to being listless and apathetic. [14] [15] Semantic dementia [ edit ] Semantic dementia (SD) is characterized by the loss of semantic understanding, resulting in impaired word comprehension. ... External links [ edit ] OMIM on Frontotemporal Dementia and/or Amyotrophic Lateral Sclerosis and Chromosome 9 Open Reading Frame 72; C9ORF72 Classification D ICD - 10 : G31.0 ICD - 9-CM : 331.19 OMIM : 600274 105550 614260 MeSH : D003704 DiseasesDB : 10034 External resources Patient UK : Frontotemporal dementia GeneReviews : MAPT-Related Disorders v t e Mental and behavioral disorders Adult personality and behavior Gender dysphoria Ego-dystonic sexual orientation Paraphilia Fetishism Voyeurism Sexual maturation disorder Sexual relationship disorder Other Factitious disorder Munchausen syndrome Intermittent explosive disorder Dermatillomania Kleptomania Pyromania Trichotillomania Personality disorder Childhood and learning Emotional and behavioral ADHD Conduct disorder ODD Emotional and behavioral disorders Separation anxiety disorder Movement disorders Stereotypic Social functioning DAD RAD Selective mutism Speech Stuttering Cluttering Tic disorder Tourette syndrome Intellectual 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