Frontotemporal dementia is dementia associated with progressive loss of nerve cells in two areas of the brain, which shrink – frontal (behind the forehead) and temporal (behind the temples). This leads to progressive change in personality and behavior, difficulties with language use and comprehension, and cognitive impairment consisting of trouble remembering, learning new things, concentrating, or making decisions that affect their everyday life (Sadock, et al., 2017).
Types and Symptoms of Frontotemporal Dementia
Frontotemporal dementia is also referred to as frontotemporal lobar degeneration. It is mainly of two types. They are as follows (Sadock, et al., 2017):
Behavioral-variant Frontotemporal Dementia (bv-FTD)
In this, there are dramatic changes in the patient’s personality and behavior. They display socially inappropriate behavior with no manners or decorum. They may begin to take impulsive, rash or careless decisions and actions. Apathy or inertia can also be seen. They may show a lack of sympathy or empathy, with diminished responsiveness to other’s needs or feelings, or diminished social interest and interrelatedness, or personal warmth. They may show less support to a spouse’s health problems.
Stereotyped, compulsive or ritualistic behaviors can also be seen, ranging from simple repetitive movements to more complex or compulsive behaviors, like collecting specific objects, or hoarding. Meaningless speech, as well as mental rigidity can also be seen. Individuals may display insertion of inappropriate objects in the mouth or dietary changes, such as eating foods that are just one color, or binge eating.
Neuropsychological testing reveals deficits of planning, organizing and completing tasks, with relative sparing of memory and visual orientation in space. They often have very limited insight into their alterations of behavior. About 20 percent of individuals with the behavioral variant of frontotemporal dementia also show signs of parkinsonism, with rigidity and tremor. Five to ten percent of individuals with this dementia have co-occurring motor neuron disease as well, with symptoms including muscle wasting, weakness, and twitching.
Language Predominant Frontotemporal Dementia
There are three varieties of this – semantic variant, nonfluent/agrammatic variant, and logopenic variant.
In the semantic variant, they lose the ability to understand or formulate words in a spoken sentence. They may not recognize objects and have difficulty with recognition of irregular sounding words, like yacht, and writing will be incoherent. They have prominent difficulty naming things (anomia) and may replace a specific word with a more general word such as “it” for pen or “food” for “apples”. They may also lose knowledge of word meaning (Mayo Clinic Staff, 2016). As the disease progresses, speech becomes empty of content. Those who have right-sided predominant shrinkage of brain, may show some early behavioral features, including emotional detachment or lack of empathy, or may also have impairments in facial recognition.
In nonfluent/agrammatic variant, a person’s speaking is very slow, hesitant, labored and ungrammatical. Speech may sound telegraphic with misuse of pronouns and errors in sentence construction (Mayo Clinic Staff, 2016). They will not comprehend complex sentences. Single-word comprehension and object knowledge are spared. They have more insight into their deficits and maintain social norms; if shrinkage of nerve cells progresses to the right frontal regions, behavioral symptoms are likely to follow.
In the logopenic variant, people may have trouble finding the right word to use in speech or naming objects. Speech is slow due to word-finding problems. Single-word comprehension and speech articulation are spared.
Frontotemporal dementia invariably gets worse over time and the speed of decline varies from person to person. On average, survival is 8 years from symptom onset and 4 to 5 years from diagnosis. For many years, these individuals show muscle weakness and coordination problems, leaving them needing a wheelchair or becoming bedbound. These muscle issues can cause problems with swallowing, chewing, moving and controlling bladder and/or bowels. Eventually they die because of the physical changes that can cause skin, urinary tract and/or lung infections (Alzheimer’s Association, n.d.).
Causes of Frontotemporal Dementia
Frontotemporal dementia is said to be caused by clumps of abnormal protein (tau and TDP-43) forming inside brain cells. These are thought to damage the cells and stop them from working properly. The proteins mainly build up in the frontal and temporal lobes of the brain that are important for controlling language, behavior, and the ability to plan, organize and carry out tasks (NHS UK, 2017).
Only less than half the people who develop frontotemporal dementia have a family history of dementia, which is the only known risk factor (Mayo Clinic Staff, 2016).
Diagnosis of Frontotemporal Dementia (NHS UK, 2017)
There’s no single test that can lead to the diagnosis of frontotemporal dementia. The following may be needed to make a diagnosis:
- Symptoms assessment – A spouse or close relative or caregiver is the best person to tell the symptoms since the person himself may be unaware of the changes.
- Mental abilities assessment– this will usually involve a neuropsychological assessment by having the patient do a number of tasks and answer questions.
- Do blood tests – to rule out conditions with similar symptoms.
- Do brain scans– An MRI, CT or PET scan can detect signs of dementia and help rule out other problems with the brain.
- Carry out a lumbar puncture – to test the spinal fluid; this may be useful to rule out Alzheimer’s disease.
Treatment of Frontotemporal Dementia (Mayo Clinic Staff, 2016) (NHS UK, 2017)
As of now, there is no cure for frontotemporal dementia. Treatment mainly involves the management of symptoms so that the patient can have a better quality of life because you cannot slow down or stop the disease progression.
- Antidepressants. Some antidepressants, such as trazodone or SSRIs (sertraline [Zoloft] or fluvoxamine [Luvox]), may reduce the behavioral problems associated with frontotemporal dementia.
- Antipsychotics. Antipsychotics, such as olanzapine (Zyprexa) or quetiapine (Seroquel), can be used to combat the behavioral problems of frontotemporal dementia. However, these medications carry an increased risk of death in dementia patients, so use caution.
Speech and language therapy can help improve any communication or swallowing problems. Physiotherapy can help with movement difficulties. Relaxation techniques like massage or music therapy can be of help.
Lifestyle and Home Remedies
You’ll eventually need caregivers to assist you with daily life activities, maintain your safety, provide transportation and help with finances. Exercise may help improve your mood and thinking skills. Minor adjustments at home, such as removing rugs or raising toilets, will make daily living tasks easier and reduce your chance of injuries.
Caregivers can help by:
- Anticipating needs and meeting them promptly
- Maintaining a calm environment
- Avoiding events or activities that trigger the undesirable behavior
- Providing structured routines and simplifying daily tasks
- Using humor
Caring for someone with frontotemporal dementia can be challenging and stressful because of the extreme personality changes and behavioral problems that often develop. Caregivers need assistance from family members, friends and support groups, or respite care provided by home health care agencies. Caregivers should exercise, eat a healthy diet and manage their stress.
End of Life and Legal Issues
You might want to make arrangements for your care that take into account the decline in your mental abilities. This may include ensuring your wishes are upheld if you’re not able to make decisions for yourself. This may involve:
- Drawing up an Advance Directive – making your treatment preferences known in case you’re unable to do this in the future
- Specifying where you want to receive treatment as your condition becomes more advanced
- Giving a relative lasting power of attorney
Alzheimer’s Association, n.d. Frontotemporal Dementia. [Online]
Available at: https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/frontotemporal-dementia
[Accessed 19 Sep 2019].
Mayo Clinic Staff, 2016. Frontotemporal dementia. [Online]
Available at: https://www.mayoclinic.org/diseases-conditions/frontotemporal-dementia/symptoms-causes/syc-20354737
[Accessed 19 Sep 2019].
NHS UK, 2017. Frontotemporal Dementia. [Online]
Available at: https://www.nhs.uk/conditions/frontotemporal-dementia/
[Accessed 19 Sep 2019].
Sadock, B. J., Sadock, V. A. & Ruiz, P., 2017. Kaplan and Sadock’s Comprehensive Textbook of Psychiatry. 10th ed. Philadelphia: Wolters Kluwer.