[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.
[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.
Other related disorders include corticobasal syndrome (CBS or CBD), and FTD with amyotrophic lateral sclerosis (ALS).
[17] 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.
[15] Behavioral variant frontotemporal dementia (BvFTD) was previously known as Pick's disease, and is the most common of the FTD types.
They consist of tau fibrils as a major component together with a number of other protein products including ubiquitin and tubulin.
Post-mortem studies show a marked reduction in the caudate nucleus of the striatum; frontotemporal gyri are narrowed, with widened intervening sulci, and the lateral ventricles are enlarged.
Recent findings from structural MRI research have indicated that eating changes in FTD are associated with atrophy (wasting) in the right ventral insula, striatum, and orbitofrontal cortex.
[citation needed] In rare cases, FTD can occur in people with amyotrophic lateral sclerosis (ALS), a motor neuron disease.
In rare cases, patients with clinical FTD were found to have changes consistent with Alzheimer's disease on autopsy.
Many research groups have begun using techniques such as magnetic resonance spectroscopy, functional imaging, and cortical thickness measurements in an attempt to offer an earlier diagnosis to the FTD patient.
Meta-analyses based on imaging methods have shown that frontotemporal dementia mainly affects a frontomedial network discussed in the context of social cognition or "theory of mind".
[45] This is entirely in keeping with the notion that on the basis of cognitive neuropsychological evidence, the ventromedial prefrontal cortex is a major locus of dysfunction early on in the course of the behavioural variant of frontotemporal degeneration.
[46] The language subtypes of FTLD (semantic dementia and progressive nonfluent aphasia) can be regionally dissociated by imaging approaches in vivo.
There are distinct differences in the behavioral and emotional symptoms of the two dementias, notably, the blunting of affect seen in FTD patients.
However, over time, these ambiguities fade away as this dementia progresses and defining symptoms of apathy, unique to FTD, start to appear.
[citation needed] Recent studies over several years have developed new criteria for the diagnosis of behavioral variant frontotemporal dementia (bvFTD).
The confirmatory diagnosis is made by brain biopsy, but other tests can be used to help, such as MRI, EEG, CT, and physical examination and history.
Similar to standard FTD, the primary diagnosis stems from clinical trials that identify the associated symptoms, instead of imaging studies.
In addition, the criteria allow for a diagnostic hierarchy distinguished possible, probable, and definite bvFTD based on the number of symptoms present.
[50] A 2021 study, determined that using cerebrospinal fluid (CSF) biomarkers of pathologic amyloid plaques, tangles, and neurodegeneration, collectively called ATN, can be useful in diagnosing FTD.
It is suggested that people with orbitofrontal cortex dysfunction show a tendency to make social blunders due to a deficit in self-monitoring.
This hypothesis argues that when people have to make complex uncertain decisions, they employ both cognitive and emotional processes to assess the values of the choices available to them.
Each time a person makes a decision, both physiological signals and evoked emotion (somatic markers) are associated with their outcomes, and this accumulates as experience.
People tend to choose the choice which might produce the outcome reinforced with positive stimuli; thus it biases decision-making towards certain behaviors while avoiding others.
Rehabilitiation services supporting every day functioning have demonstrated some positive results, in particular the Tailored Activity Programme, which is occupational therapy based.