By Sonja Zimmer, Patient Services Director, The ALS Association Evergreen Chapter
The frontotemporal dementias (FTD) are a group of degenerative brain disorders that share many medical features. All result from progressive damage to cells in the anterior temporal and/or more frontal lobes of the brain. The disorders are also known as frontotemporal lobar degeneration (FTLD). The hallmark of FTD is a gradual, progressive decline in behavior and/or language. As the disease progresses, it becomes more difficult for people to plan or organize activities, behave appropriately in social or work settings, interact with others, and care for oneself, resulting in increasing dependency.
While estimates vary, it is now believed that approximately 20-30% of ALS patients have the signs and symptoms of frontotemporal dementia. Upwards of 50% of ALS patients have mild changes in their ability to concentrate and focus on multiple ideas and/or actions at one time, as well as make complex judgments, called “executive functioning”. It is important to know that the majority of ALS patients that have mild changes in behavior DO NOT have FTD
Difficulties with diagnosis – Is it FTD or Alzheimer’s disease?
Historically, physicians had a difficult time distinguishing FTD from Alzheimer’s disease, psychiatric problems or several other neurological disorders. Today, increased medical understanding and advanced brain imaging techniques help the physician to make an accurate diagnosis. FTD and Alzheimer disease (AD) are characterized by deterioration of the brain, and a gradual, progressive loss of brain function. Frontotemporal dementia however, is primarily a disease of behavior and language dysfunction, while the characteristic of Alzheimer Disease is loss of memory. FTD often begins earlier than Alzheimer’s disease, and affects areas in the brain that are more anterior or ‘forward’ than those affected by Alzheimer’s.
Damage to the brain’s frontal and temporal lobes affect complex thinking and reasoning abilities which can result in other symptoms commonly associated with FTD. Increased damage in “executive functions” affects a person’s ability to plan, organize and execute activities, while emotional changes impact relationships. Symptoms may include:
• Easily distracted and increasing difficulty staying on task mentally.
• Mental rigidity and inflexibility, insistence on having his or her own way, increasing difficulty adapting to new or changing circumstances.
• Planning and problem solving changes, abstract reasoning decreases. Examples of this would include difficulty coordinating the cooking of a meal, or making a shopping list and performing necessary errands.
• Poor financial judgment, impulsive spending or financial risk taking.
• Reduced initiative, lack of motivation, and an apparent loss of interest in previously enjoyed hobbies and social activities.
• Lack of insight into one’s behavior develops early; the patient does not recognize changes in his or her own behaviors and shows no concern for the effect of these behaviors on others, including loved ones.
• Mood changes that can be abrupt and frequent.
Behavioral changes are typically seen as changes in personality, emotional blunting or loss of empathy that result in increasingly inappropriate social behavior. People gradually become less involved in routine daily activities and withdraw emotionally from others. Unusual behaviors may include swearing, overeating or drinking, impulsivity, shoplifting, hypersexual behavior, and deterioration in personal hygiene habits.
The language deficits experienced by FTD patients are distinguished by two presenting issues: problems with expression of language or severe naming difficulty, and problems with word meaning.
The Caregiver’s Approach
There are three strategies: plan for success; old memories are the strongest; and structure is important. Develop an approach to care giving that maximizes strengths minimizes frustration.
Evidence from caregivers and physicians indicate that having a planned structure is very helpful for FTD patients who have trouble with apathy and initiation. It allows them to remain more engaged because they don’t have to make decisions about what to do. People with dis-inhibited behavior tend to benefit from greater activity because problem behaviors are worse when people are bored. Know that FTD progression is continual and inevitable. The more managed and structured the day is, the more you may enjoy being together, and the more you can feel in charge as a caregiver.
There is no cure for FTD, and in most cases its progression cannot be slowed. Although no medications have been proven effective for FTD, many clinicians look to the medications and treatment approaches used in other, similar disorders to develop a therapeutic approach.