BEHAVIORAL VARIANT FRONTOTEMPORAL DEMENTIA (BVFTD)

Perelman School of Medicine

Frontotemporal degeneration (FTD) refers to a family of disorders characterized by the progressive loss of neurons (brain cells) in the frontal and temporal regions of the brain.

Patients with FTD can present with different clinical symptoms, ranging from behavioral impairments to language or motor dysfunction. Although the precise cause may not be known, Frontotemporal degeneration is thought to result from abnormal accumulation of misfolded proteins, which disrupt, and eventually lead to degeneration of brain cells.

As FTD progresses, it can slowly deprive a person of their cognitive abilities, personality and eventually their independence. 

Below are the typical symptoms of behavioral variant Frontotemporal degeneration:

• Behavioral disinhibition: Disinhibition is the hallmark feature of bvFTD and can manifest as socially inappropriate behavior (e.g., inappropriately approaching or touching strangers), loss of manners or decorum (e.g., violation of personal space, rude or offensive remarks), or impulsive, rash or careless actions (e.g., reckless buying or selling).
• Apathy/Inertia: Patients with bvFTD also present with a general loss of interest, drive or motivation. In extreme cases, patients may need prompts to initiate or continue basic activities (e.g., bathing, dressing). 
• Loss of empathy: bvFTD patients may seem emotionally cold or detached and can exhibit an overt disregard for others pain or distress.
• Perseverative/Compulsive behaviors: These can range from simple repetitive behaviors such as tapping, scratching or picking, to complex compulsive behaviors such as ordering, cleaning or collecting.
• Changes in eating habits: Dietary changes can range from altered food preferences or “food fads” (e.g., only eating a particular type of food) to indiscriminate binge eating and weight gain.
• Executive dysfunction: Due to cell loss in their frontal lobes, bvFTD patients exhibit deficits in complex thinking or “executive functions” such as planning, organizing, mental flexibility and generation of ideas.

Diagnosis challenges – Given the gradual behavioral and personality changes typical of bvFTD, diagnosis of the syndrome can be challenging. Many patients endure multiple physician and mental-health referrals before their disease is recognized as a neurodegenerative disorder. Even when patients are identified as suffering from a neurological problem, they are often misdiagnosed with Alzheimer’s disease or other forms of dementia. Early and accurate diagnosis of bvFTD is crucial, as it allows for appropriate care and counseling of patients and their families. Most importantly, accurate diagnosis of behavioral variant Frontotemporal degeneration will identify patients likely to benefit from new medications, once these become available.