Pick's Disease: Behavioral Variant Frontotemporal Dementia

Behavioral variant frontotemporal dementia (bvFTD) is one of several types of dementias that fall into the "frontotemporal degeneration" category. This category affects approximately 50,000 to 60,000 Americans. As the name indicates, this kind of dementia primarily affects the frontal and temporal lobes of the brain. The most common frontotemporal dementias are of the behavioral-variant type. Behavioral-variant frontotemporal dementia is sometimes referred to as Pick's disease.

Pick's disease is thought to be caused by abnormal clustering of tau proteins in the frontal and temporal lobes of the brain. These protein clumps are known as Pick bodies. As they gather in these lobes, brain cells begin to die, causing emotional, behavioral and cognitive changes in your functioning. 

The most common symptoms of bvFTD are behavioral or emotional challenges, including compulsively overeating, having an emotionally-detached demeanor, making socially inappropriate responses, and displaying hypersexual conversation and actions, along with irritability, agitation, apathy, and selfishness.

People with bvFTD might have difficulty keeping a job, display a decline in their hygiene, hoard certain items, make poor financial choices, and distance family members and friends through their insensitive comments and behaviors.

Some individuals with bvFTD also experience movement difficulties similar to Parkinson’s disease, including decreased facial expressions, muscle stiffness, weakness, and rigidity.

Unlike Alzheimer's, memory loss does not usually develop in bvFTD, and if it does, it's not until the disease is its later stages. As a caregiver, you may then notice some memory difficulties, as well as challenges with planning or attention (related to executive functioning).

In severe bvFTD, language also is affected, making communication very difficult.

Behavioral variant frontotemporal dementia (Pick's disease) has some overlapping symptoms with Alzheimer's disease since both diseases typically affect cognition, emotion and behaviors. The earlier symptoms of Pick's disease, however, consist primarily of changes in emotion, judgment, executive functioning, and behavior, while in Alzheimer's disease, memory, orientation, and communication deficits are more common initially. 

Diagnosis of BvFTD is often delayed because initially it may be thought that an affected person is just being rude, selfish, depressed or acting out of character. As symptoms progress, however, families usually seek a clinician's assistance for diagnosis and treatment. Diagnosis depends on the presence of multiple symptoms that are outlined above, as well as the ability to rule out other dementias such as Alzheimer's or vascular dementia. Reviewing the official criteria for behavioral variant FTD may be helpful for family members if BvFTD is diagnosed or being considered by the physician.

An MRI can also assist in diagnosing bvFTD because it will often show some atrophy (shrinkage) of the frontal lobe of the brain. As the disease progresses, the atrophy will increase and spread to the temporal areas, as well as the basal ganglia.

Average life expectancy from the onset of symptoms is approximately eight to nine years, though some people may live 20 years or so with the disease. As with any dementia, it can be a challenging disease for caregivers, as well as for those experiencing it.

Treatment may consist of attempting to use non-drug behavior strategies to curb specific behaviors. Some physicians prescribe antidepressants called selective serotonin reuptake inhibitors (SSRIs), which may help with some of the obsessive-compulsive behaviors such as hoarding or overeating.

It's normal to feel overwhelmed at times as you cope with the challenges of behavioral variant frontotemporal dementia. Ignore the temptation to try to do it all yourself; instead, reach out for support to those around you, whether that's through a community support program, an online discussion about BvFTD or a friend who's willing to take you out for coffee or sit at home with your loved one while you take a nap.

Additionally, The Association for Frontotemporal Dementia lists local support groups as well as online resources and a phone line for help.