Frontotemporal dementia (FTD) is a group of disorders categorized by the progressive loss of nerve cells within the brain’s frontal lobes (found directly behind the forehead) or temporal lobes (Areas behind the ears). When nerve cells are lost in these areas of the brain behavior and personality can begin to deteriorate along with changes to language and changes to muscle or motor functions.
There are three major types of FTD including behavior variant frontotemporal dementia (bvFTD), primary progressive aphasia (PPA), and disturbances of motor function. Behavior variant frontotemporal dementia mainly leads to changes in personality and behavior and generally occurs in individuals in their 50s and 60s. Nerve cell loss in bvFTD is mostly seen in the areas of the brain which controls conduct, judgment, empathy, foresight and other abilities.
Primary progressive aphasia largely affects language, speech, writing and comprehension. PPA can be separated into two areas: Semantic variant of PPA and nonfluent/agrammatic variant of PPA. In the semantic variant a person’s ability to understand or formulate words is lost. In nonfluent/agrammatic speaking is hesitant, labored and ungrammatical.
Lastly, disturbances of motor function consist of three disorders which are part of the frontotemporal degeneration spectrum which affects motor and muscle function. The three disorders are Amyotrophic lateral sclerosis (ALS), Corticobasal syndrome and Progressive supranuclear palsy (PSP).
People with demanding jobs may live longer after developing frontotemporal dementia
New findings suggest that individuals with demanding jobs they may live longer after developing FTD. Study author, Lauren Massimo, said, “”This study suggests that having a higher occupational level protects the brain from some of the effects of this disease, allowing people to live longer after developing the disease.”
The findings support the “cognitive reserve” theory which states that more education and higher occupation and mental activity build up connections in the brain which protects it from disease. Massimo added, “People with frontotemporal dementia typically live six to 10 years after the symptoms emerge, but little has been known about what factors contribute to this range.”
Researchers reviewed medical charts of 83 people who had an autopsy post-death in order to confirm diagnosis of FTD or Alzheimer’s disease – job history was also recorded. Occupations were ranked from low to high based on demand, the lowest being trade workers and sales associates and the highest was lawyers and engineers.
Thirty-four people had a diagnosis of FTD had had an average life span with it for seven years. Those with higher demanding and challenging jobs lived longer compared to those with the lowest demand.
Although the findings suggest a connection between occupation and FTD no association was seen between FTD and Alzheimer’s survival.
Causes of frontotemporal dementia
There is not one specific cause for FTD as it is a collection of many diseases which contribute to nerve cell loss. IN majority of cases of FTD a cause is never found but in 15 to 40 percent of cases hereditary has been found to play a role.
When genes are the cause of FTD is often involves a genetic mutation which causes the gene to act abnormally. Below is a list of genes which have been shown to play a role in the onset of FTD.
- PGRN gene – leads to lower production of progranulin which causes TDP-43 to act abnormally
- Tau gene (MAPT gene) – a mutation in the tau protein which tangles and destroys brain cells
- VCP, CHMP2B, TARDBP, and FUS genes – part of rare familial types of FTD
- C9ORF72 gene – common genetic mutation of familial FTD
Common symptoms of frontotemporal dementia
Symptoms of FTD can vary vastly from each person as it is dependant on what areas of the brain are affected. Typically, nerve loss in the frontal lobe of the brain affects behavior and nerve loss in the temporal lobe mainly affects language and emotions.
Common symptoms of frontotemporal dementia include:
- Problems with planning and executing, prioritizing, multitasking, and self-monitoring behavior
- Repeating activities or words
- Impulsive behavior and act out inappropriately for example laughing at a funeral
- Compulsive eating
- Impulsive behavior regarding reaching out and grabbing things in their line of vision
- Inability to understand or use words but speaking is normal (aphasia)
- Inability to speak properly but the message is normal (dysarthria)
- Emotional changes
- Changes to social interactions – inability to “read” social signals and understanding relationships
- Abnormal body part posture
- Abnormalities with walking and frequent falls
- Loss of coordination
- Muscle weakness and cramps
Key differences between FTD and Alzheimer’s disease
Although FTD and Alzheimer’s disease may share similar symptoms there are some key differences which distinguish one condition from the other. Differences between FTD and Alzheimer’s disease are:
- Age: FTD is commonly diagnosed between 40 to 60 years of age and Alzheimer’s diagnosis occurs more so with aging.
- Memory loss: Typically seen in early Alzheimer’s and not early FTD but can occur in later FTD.
- Behavior changes: More noticeable with bvFTD in all stages and may only be present in later Alzheimer’s disease.
- Speech problems: Alzheimer’s patients have less difficulty with speech even though they may have difficulty thinking of the word to say. In certain FTDs speech problems are more prominent.
- Hallucinations and delusion: More so common in Alzheimer’s disease, rarely seen in FTD.
Treatment and lifestyle changes for frontotemporal dementia
There is currently no cure for FTD but there are ways to manage symptoms with the help of a specialist team. The first part of managing symptoms is by managing behavior. Managing behavior may involve not arguing with a person, approaching them calmly and understand for yourself that it is the illness that has created a new behavior and it is of no fault of the individual.
Other tips are to ask close ended questions to avoid apathy for example instead of asking “what do you want to do today?” be more specific and ask, “do you want to go to the mall or to the movies?”
Treating speech and language problems may involve the help of a speech/language specialist. This individual can work with the patient to develop effective ways for them to communicate in the meantime helping them preserve their own language.
For movement and mobility symptoms exercise and physical therapy can be useful to combat stiffness and muscle cramps. The patient may even choose to move around with an assistive device like a can or walker in order to preserve balance and prevent falls.
Medications, too, can be useful in order to treat symptoms associated with FTD. Common medications prescribed to FTD patients include antidepressants and antipsychotics in order to better manage mood and behavior.
Until more research and understanding comes out in regards to frontotemporal dementia that is only when we can begin to work closer to a potential cure. In the meantime working with doctors, nurses and specialists can help ease symptoms and improve quality of life for the patient.
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