About 12–13% of people with bvFTD develop motor neuron disease.The Pick bodies in behavioral variant FTD are spherical inclusion bodies found in the cytoplasm of affected cells. They consist of tau fibrils as a major component together with a number of other protein products including ubiquitin and tubulin. Semantic dementia
Semantic dementia (SD) is characterized by the loss of semantic understanding, resulting in impaired word comprehension. However, speech remains fluent and grammatical. Progressive nonfluent aphasia
Progressive nonfluent aphasia (PNFA) is characterized by progressive difficulties in speech production. Neuronal intermediate filament inclusion disease
Neuronal intermediate filament inclusion disease (NIFID) is a rare distinct variant. The inclusion bodies that are present in NIFID are cytoplasmic and made up of type IV intermediate filaments. NIFID has an early age of onset between 23 and 56. Symptoms can include behavioural, and personality changes, memory and cognitive impairments, language difficulties, motor weakness, and extrapyramidal symptoms. NIFID is one of the FTLD-FUS proteopathies. Imaging commonly shows atrophy in the frontotemporal region, and in part of the striatum in the basal ganglia. Post-mortem studies show a marked reduction in the caudate nucleus of the striatum; frontal temporal gyri are narrowed with widened intervening sulci, and the lateral ventricles are enlarged. Basophilic inclusion body disease
Another rare FTD variant, also a FTLD-FUS proteopathy, is basophilic inclusion body disease (BIBD). Other characteristics
In later stages of FTD, the clinical phenotypes may overlap. People with FTD tend to struggle with binge eating and compulsive behaviors.
Currently, there is no cure for FTD, but there are treatments that help alleviate symptoms. Signs and symptoms
Frontotemporal dementia (FTD) is an early-onset disorder that mostly occurs before the age of 65, but can begin earlier, and in 20–25% of cases onset is later. It is the most common early presenting dementia.The International Classification of Diseases recognizes the disease as causative to disorder affecting mental and behavioural aspects of the human organism. Dissociation from family, extreme oniomania, vulgar speech characteristics, screaming, inability to control emotions, behavior, personality, and temperament are characteristic social display patterns. A gradual onset and progression of changes in behavior or language deficits are reported to have begun several years prior to presentation to a neurologist. Subtypes and related disorders
The main subtypes of frontotemporal dementia are behavioral variant FTD, semantic dementia, progressive nonfluent aphasia, and FTD associated with amyotrophic lateral sclerosis (FTD–ALS). Two distinct rare subtypes are neuronal intermediate filament inclusion disease, and basophilic inclusion body disease. Related disorders are corticobasal syndrome, and progressive supranuclear palsy. Behavioral variant frontotemporal dementia
Behavioral variant frontotemporal dementia (BvFTD) was previously known as Picks disease, and is the most common of the FTD types. BvFTD is diagnosed four times as often as the PPA variants. 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.