However, over time, these ambiguities fade away as this dementia progresses and defining symptoms of apathy, unique to FTD, start to appear.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. Six distinct clinical features have been identified as symptoms of bvFTD. Disinhibition
Apathy/Inertia
Loss of Sympathy/Empathy
Perseverative/compulsive behaviors
Hyperorality
Dysexecutive neuropsychological profileOf the six features, three must be present in a patient to diagnose one with possible bvFTD. Similar to standard FTD, the primary diagnosis stems from clinical trials that identify the associated symptoms, instead of imaging studies. The above criteria are used to distinguish bvFTD from disorders such as Alzheimers and other causes of dementia. In addition, the new criteria allow for a diagnostic hierarchy distinguished possible, probable, and definite bvFTD based on the number of symptoms present.A 2021 study, led by researchers at the University of Pennsylvania, determined that using cerebrospinal fluid (CSF) biomarkers of pathologic amyloid plaques, tangles, and neurodegeneration, collectively called ATN, can be useful in diagnosing FTD. Neuropsychological tests
The progression of the degeneration caused by bvFTD may follow a predictable course. The degeneration begins in the orbitofrontal cortex and medial aspects such as ventromedial cortex. In later stages, it gradually expands its area to the dorsolateral cortex and the temporal lobe. Thus, the detection of dysfunction of the orbitofrontal cortex and ventromedial cortex is important in the detection of early stage bvFTD.
The first research criteria for FTD “Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups,” was developed in 1994. The clinical diagnostic criteria were revised in the late 1990s, when the FTD spectrum was divided into a behavioral variant, a nonfluent aphasia variant and a semantic dementia variant. The most recent revision of the clinical research criteria was by  International Behavioural Variant FTD Criteria Consortium (FTDC) in 2011.

Notable cases
Notable cases of bvFTD when mostly were referred to as Picks disease. John Berry (1963-2016), American hardcore punk musician and founding member of the Beastie Boys. Don Cardwell (1935–2008), Major League Baseball pitcher
Charmian Carr (1942–2016), who played Liesl, from the Sound of Music, born Charmian Anne Farnon. Jerry Corbetta (1947–2016), frontman, organist and keyboardist of American psychedelic rock band Sugarloaf
Ted Darling (1935–1996), Buffalo Sabres television announcer
Robert W. Floyd (1936–2001), computer scientist
Lee Holloway (born 1981), co-founder of Cloudflare
Colleen Howe (1933–2009), sports agent and hockey team manager, known as "Mrs. Hockey"
Kazi Nazrul Islam (1899–1976), notable Bengali poet and National Poet of Bangladesh
Ralph Klein (1942–2013), former premier of Alberta, Canada
Terry Jones (1942-2020), Welsh comedian (Monty Python) and director.