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The Facts About bvFTD

The following information is copied with permission from the Association for Frontotemporal Dementia (AFTD) web site. For additional information and support please visit them at: http://www.ftd-picks.org

Note: As of February 2011-
The AFTD Board of Directors has unanimously voted to change our organization’s name to The Association for Frontotemporal Degeneration. To read a letter from Board Chair Beth Walter explaining the change, go here. It is still the same great organization.

Behavioral Variant FTD (bvFTD)

Behavioral variant FTD (bvFTD) is frontotemporal dementia characterized by early and progressive changes in personality, emotional blunting and/or loss of empathy. It is characterized by difficulty in modulating behavior that often results in inappropriate responses or activities. Impairment of language may also occur, but is less prominent and would appear as a word-finding problem.

Onset typically occurs sometime in the 50’s, though it can occur as early as age 20 or as late as age 80. As with all FTDs, the course of bvFTD will vary from one person to another. Not every symptom will be experienced by every person, nor will these symptoms develop in a pre-ordained sequence.
Key Clinical Features

The hallmark of behavioral variant FTD is a progressive deterioration in a person’s ability to control or adjust his or her behavior in different social contexts that results in the embarrassing, inappropriate social situations that can be one of the most disturbing facets of FTD. The patient typically does not recognize the changes in his or her own behaviors, nor do they exhibit awareness or concern for the effect these behaviors have on the people around them.

Behavioral symptoms

Hyperoral behaviors include overeating, dietary compulsions, in which the person restricts himself to eating only specific foods (such as a certain flavor of Lifesaver, or eating food only from one fast food restaurant), or attempts to consume inedible objects. Patients may consume excessive amounts of liquids, alcohol and cigarettes.

Stereotyped and/or repetitive behaviors can include re-reading the same book multiple times, hand rubbing and clapping, humming one tune repeatedly, or walking to the same location day after day.

Personal hygiene habits deteriorate early in the disease progression, as the person fails to perform everyday tasks of bathing, grooming, and appropriate dressing.

Hyperactive behavior is exhibited by some patients, and can include agitation, pacing, wandering, outbursts of frustration, and aggression.

Hypersexual behavior can range from a preoccupation with sexual jokes to compulsive masturbation.

Impulsive acts can include shoplifting, impulsive buying, and grabbing food off of another person’s plate.

Emotional Symptoms

Apathy or indifference toward events and the surrounding environment can be marked by reduced initiative and lack of motivation.

Lack of insight into the person’s own behavior develops early. The patient typically does not recognize the changes in his or her own behaviors, nor do they exhibit awareness or concern for the effect these behaviors have on the people around them, including loved ones.

Emotional blunting develops early in the course of the disorder, and is manifested as a loss of emotional warmth, empathy, and sympathy, and development of what appears to be indifference toward other people, including loved ones.

Mood changes can be abrupt and frequent.

Neurological Symptoms

Symptoms similar to those seen in Parkinson disease. In FTD patients the term “parkinsonism” is used to distinguish the fact that they do not have Parkinson disease, though they do exhibit some of these symptoms. Symptoms include: decreased facial expression, bradykinesia (slowness of movements), rigidity (resistance to imposed movement), and postural instability.
Key Pathological Features

No specific pathology has been associated with bvFTD to date. In some patients it may be related to abnormal collections of the protein tau. In other patients it may be related to accumulations of the protein TDP-43. More cases seem to be connected with TDP-43 pathology rather than than tau pathology. Research is continuing to better understand the causes of this disorder.

The majority of bvFTD cases are not hereditary. A subset of patients has a family history and genetic mutations have been identified in some of these families.

As with all forms of FTD, there is no cure for bvFTD, and in most cases its progression cannot be slowed. Although no medications have been proven effective specifically in FTD, many clinicians look to the medications and treatment approaches that target behavioral disturbances as needed.

For instance, some FTD patients benefit from using selective serotonin reuptake inhibitors (SSRIs) to treat obsessive-compulsive behaviors, such as hoarding or craving sweets. Clinicians may also recommend antioxidants, such as coenzyme Q10, which are known to slow the progression of damage to brain cells in general, but there is little evidence to support this in Pick’s disease.

Management and Prognosis

The progression of behavioral variant FTD can range from 3 to 17 years, with a mean course of 8 years from the time of diagnosis to death. During this time, the patient is typically increasingly dependent on others for activities of daily living.

The unusual behaviors and emotional changes that patients develop are a manifestation of the disease. Recognizing these changes as outside of the patient’s control makes it easier for family and friends to adjust their expectations, daily routines and roles to help the patient stay as actively engaged as possible. The patient will not be aware of the impact of behaviors that others find disruptive, annoying or embarrassing. Logical reasoning and trying to convince the person to stop will not work. In fact, pressing the person will likely increase symptoms and agitation. When others can adapt their approach to the patient, a more positive outcome can be expected.

Caregivers may decide to accept some behaviors that are odd, but not harmful. Other behaviors can be reduced by changes in the home environment, such as limited access to food if overeating or hoarding are issues. When confronted with persistent behavior, redirecting the person’s attention to a different activity will be most effective. Direct intervention by others is necessary if aggressive behavior or poor judgment causes the person’s safety or that of another to be at risk. For more tips, see Caregiving Challenges in the Support and Resources section

It is important for caregivers and families to think about long-term management issues and identify a team of experts who can help with difficult medical, financial and emotional challenges. It is imperative to have a physician who is knowledgeable about FTD. Other medical specialists who may be helpful include: speech and language pathologists, occupational and physical therapists, neuropsychologists, nurses (especially home-care nursing), and genetic counselors.

Death in bvFTD is generally caused by pneumonia or other complications of severe debility, such as sepsis (an infection throughout the body) or pulmonary embolism (a blood clot that blocks a major blood vessel in the lung)