Everything You Need to Know About Frontotemporal Dementia
The onset of frontotemporal dementia (FTD) can be painful and confusing for the person dealing with it, as well as for their family and friends. Frontotemporal dementia refers to a group of related brain disorders that are similar to other types of dementia, but are less common, have some different symptom characteristics, and usually begin earlier in life than other forms of dementia. FTD generally begins when a person is between ages 45 and 64. FTD is often misdiagnosed as Alzheimer’s, Parkinson’s, or a psychiatric disorder.
This article focuses on multiple aspects of frontotemporal dementia to help those with the condition, as well as family, friends and caregivers, deal with the disease:
- Symptoms of frontotemporal dementia
- Causes of frontotemporal dementia
- Frontotemporal dementia vs. other illnesses
- Frontotemporal dementia stages and progression
- Frontotemporal dementia treatments
Symptoms of Frontotemporal Dementia
One reason FTD is often misdiagnosed is the wide range of symptoms through which it can manifest. There are a variety of symptoms associated with FTD, and different people have different symptom patterns. Researchers have identified three main categories of FTD symptom types: behavioral changes, speech and language problems, and movement disorders.
Behavioral Changes
Those who primarily experience behavioral changes have behavioral variant FTD (bvFTD), formerly called Pick’s disease or frontal variant FTD. People experiencing behavioral changes due to FTD often act in ways uncharacteristic of their personality or unusual for whatever situation they are in. Sometimes they do things others would consider inappropriate or embarrassing and seem unaware of social norms. They might act impulsively or without considering the feelings of others. Sometimes they engage in compulsive behavior, begin overeating, or even eat things that aren’t food. They might stop paying attention to personal hygiene. Their behavioral changes are often upsetting to friends and family, while they seem unphased. As the illness progresses, they often begin developing symptoms that fall under other categories, too.
Speech and language problems.
Doctors refer to the speech and language problems of FTD as primary progressive aphasia (PPA). There are three types of PPA: semantic PPA, agrammatic PPA, and logopenic PPA.
- Semantic PPA has to do with remembering words. People who have semantic PPA might forget the names of objects or people and point or use more general terms like “it” or “them” to refer to something or someone they can’t remember the name of. As the illness progresses, they might also have trouble recognizing people’s faces or remembering the purpose of everyday objects.
- People with agrammatic PPA can recognize and remember most words, but they have trouble saying them. They might speak in a distorted way, trying to say a word, but finding that it doesn’t come out right. They might omit words from sentences or omit syllables from longer words. With time, they might lose most of their ability to speak.
- People with logopenic PPA have trouble finding the right words when speaking. They understand and recall the meaning of words, but they might pause often while speaking, or give long descriptions in place of a word if they can’t place it.
Movement Disorders
Multiple movement disorders are associated with FTD, such as corticobasal syndrome (CBS), progressive supranuclear palsy (PSP), frontotemporal dementia with parkinsonism, and frontotemporal dementia with amyotrophic lateral sclerosis (FTD-ALS). Movement disorders are the least common forms of FTD and can manifest in multiple ways. Sometimes they cause muscle spasms or visible tremors and the person is unable to make steady movements without shaking. They might notice their coordination worsening and an increase in physical mistakes, such as dropping or spilling things. A person’s muscles might become weak, making it difficult for them to lift things or do normal tasks. Their muscles might also become stiff and rigid, which could affect their posture and gait. Others might notice that they walk differently or more slowly, or they might begin tripping and falling. Sometimes they have trouble swallowing or eating, or trouble looking in certain directions, often downward.
Doctors diagnose FTD as one of multiple specific types of the illness based on these symptoms, with each symptom cluster corresponding to formal diagnostic names. A person with FTD might have symptoms from more than one cluster, making it difficult to determine which cluster they fall into, or if they have FTD at all rather than another illness.
Causes of Frontotemporal Dementia
Frontotemporal dementia gets its name from that fact that it occurs when there are problems in the frontal and temporal lobes of the brain. Sometimes, these areas of the brain shrink or atrophy; in other cases, they suffer nerve damage. FTD used to be called Pick’s disease because some types of FTD are due to abnormal proteins in the brain called “Pick bodies.”
When FTD only occurs in one member of a family and does not appear to be hereditary, it is called sporadic FTD. Most cases of FTD are sporadic, with experts not knowing what caused them to appear. When FTD occurs in multiple generations of a family, it is called familial FTD. Up to 40% of FTD cases are familial, though experts cannot identify the gene associated with FTD in all cases.
However, researchers have identified multiple genes associated with FTD. When FTD can be traced to a specific gene, it is called genetic FTD. Only 10-20% of people with FTD have genetic FTD. Genetic FTD is passed down in what is called a dominant pattern, which means a person with an FTD-causing gene has a 40% chance of passing the gene on to each child. Virtually every person with an FTD-causing gene will develop FTD if they live until age 80. Genetic counseling is available at many dementia-focused medical centers for people experiencing FTD symptoms as well as for their offspring.
Frontotemporal Dementia vs. Other Illnesses
FTD is often misdiagnosed, especially in the early stages. The average person with FTD takes 3.6 years to obtain a correct diagnosis. FTD is often misdiagnosed as depression or other psychiatric disorders, Alzheimer’s, or Parkinson’s. Because FTD is less common than other types of dementia, some clinicians might not suspect it, or might not have experience diagnosing it.
An accurate diagnosis is important because different disorders have different treatments and outcomes. Those with FTD and their families want to make sure the correct diagnosis (or diagnoses) is obtained so they can best understand what is happening and prepare for the future. Also, because many medications have side effects, they want to ensure people are only on needed, useful medications. Common misdiagnoses include:
Depression and Psychiatric Disorders
Behavioral variant FTD (bvFTD) is often confused with depression, bipolar disorder, and other psychiatric disorders because of the changes in personality and behavior that accompany it. To make the situation even more confusing, depression and anxiety are often present in people with bvFTD, and could be risk factors for the disease.
A person with bvFTD might become withdrawn, change their eating habits, care less about other people, and pay less attention to their personal hygiene, which could look like depression or another mood or psychiatric disorder. They might even begin hoarding, engaging in risky or illegal behavior, or doing repetitive actions in which they repeat the same behavior again and again.
Depression or other psychiatric disorders can occur at any age, while bvFTD commonly begins showing symptoms in people ages 45 to 64 or older. After symptoms become more severe, people with bvFTD will likely have abnormal MRI scans. If you suspect someone might have bvFTD in addition to or instead of a psychiatric disorder, it’s best for them to see a neurologist who has experience with dementia, particularly those with FTD.
Alzheimer’s Disease
Although there is some symptom overlap, Alzheimer’s and frontotemporal dementia are distinct disorders with many differences. They both involve cognitive impairment and become progressively worse over time, but doctors familiar with types of dementia should be able to distinguish between them fairly easily. Brain scans can help doctors determine if a person has Alzheimer’s or FTD.
Alzheimer’s is the most common type of dementia and accounts for up to 80% of dementia cases. Alzheimer’s primarily occurs in people over 65, while FTD most commonly appears in people ages 45 – 64. That said, early-onset Alzheimer’s does exist, so Alzheimer’s can develop in people of the same age range as FTD. The older a person gets, however, the more likely they are to develop Alzheimer’s and the less likely they are to develop FTD.
The primary symptom of Alzheimer’s is memory impairment. Initially, a person developing Alzheimer’s will have trouble retaining new information. With time, they will begin forgetting information they might have known for years. People with primary progressive aphasia FTD often have trouble recalling words, which is why people might think they have Alzheimer’s. People with FTD can generally still learn new information and remember things they’ve known for years, especially in the early stages of the illness. The memory issues that come with FTD are primarily related to language and identification.
Parkinson’s Disease
Parkinson’s disease is a progressive disease of the nervous system that produces symptoms that overlap with FTD movement disorders. Tremors and muscle stiffness are common symptoms. Parkinson’s is more common than the rare FTD movement disorders, which is part of why they can be misdiagnosed. Parkinson’s is most likely to occur in people over age 60, while FTD generally appears between ages 45 and 64.
Parkinson’s can be accompanied by dementia, which is called Parkinson’s Disease Dementia (PDD) and is different from FTD. People with PDD have memory problems, difficulty planning things and problem solving, and difficulty understanding objects in space. People with Parkinson’s can also develop a separate diagnosis of Alzheimer’s. They are less likely to have language problems, which are common in people with FTD.
People can be given a diagnosis of FTD with parkinsonism, which can be confusing, but it isn’t the same thing as Parkinson’s disease. Parkinsonism is an umbrella term that refers to multiple neurological disorders with symptoms similar to Parkinson’s. Parkinson’s is one form of parkinsonism, and FTD with parkinsonism is another. A neurologist skilled in diagnosing and treating parkinsonism disorders will be able to help determine which disorder someone is developing.
Frontotemporal Dementia Stages and Progression
At this point in time, there is no known way to stop the progression of FTD. In most people, FTD progresses over a course of 2 – 20 years.
There aren’t distinct frontotemporal dementia stages like there are in some other disorders. Generally, an individual’s initial symptoms become worse over time and they develop additional symptoms. People diagnosed with one type of FTD often begin to develop symptoms from other symptom clusters as the disease progresses. For example, someone with behavioral variant FTD might begin the illness with personality changes and few other symptoms, then develop language troubles and even movement issues as the years go on.
People who have FTD have an average life expectancy of 7 – 13 years after development of the disease. Those with FTD do not die of FTD itself, but from some other related illness or injury. The most common cause of death for people with FTD is pneumonia, but they also might die from falling or developing another type of infection.
Frontotemporal Dementia Treatments
Unfortunately, there is not yet a cure for FTD. There are many ongoing research studies searching for a cure and effective treatments. Interested people may apply to participate in clinical trials related to FTD. There are treatments for FTD, but so far none of them are known to slow or stop the progression of the illness. There are also many medications commonly prescribed for other types of dementia that are not recommended for FTD due to potential negative side effects, which is another reason a correct diagnosis is so important.
However, there are many strategies for managing FTD and most involve some sort of caretaking. Establishing a routine can help friends and family members caring for someone with FTD to keep them as healthy as possible.
Many families of people with FTD choose to move the person into a supportive living environment with professional care where they can continue to pursue an exceptional life while receiving the assistance and treatment they need. Full-time care often becomes the only option for FTD management as the disease progresses.
The Brielle in Staten Island, New York, is one such supportive living environment, staffed with professionals experienced in improving the wellness of people with dementia through signature memory care programs. Contact us for more information about our memory care community and respite care program to see how we can help your friend or relative with FTD.