Fronto-Temporal Dementia : Who is Affected, How it Affects the Body, Causes, Symptoms, Treatment & Life Expectancy

Fronto-temporal dementia is a term used for a group of diseases which involve the deterioration of the frontal and temporal lobes of the brain.^(1,2) As these regions deteriorate, the patient loses the ability in the body regions, which are controlled by them. Patients suffering from Fronto-temporal dementia or Fronto-temporal dementia commonly do not have any control of their behavior and lose their ability to understand and speak the spoken language.

People suffering from fronto-temporal dementia are categorized under one of these three common groups of symptoms; of which two of these are the subtypes of PPA or primary progressive aphasia, which is a degenerative brain disease. The three common groups of symptoms are:

1. Behavioral-variant FTD (bvFTD).
2. Semantic-variant primary progressive aphasia (svPPA).
3. Nonfluent/agrammatic primary progressive aphasia (nfvPPA).

Fronto-temporal dementia is primarily an age-related condition; however, it can develop sooner than other age-related brain conditions.⁽³⁾ Many patients develop fronto-temporal dementia between the ages of 50 and 80, with 58 being the average age when this develops.

Fronto-temporal dementia affects females and males equally. This condition is also hereditary where patients having a family history are at risk to get affected by this condition. Around 40% of people who are affected by this condition have a family history of fronto-temporal dementia.⁽³⁾

This is a rare condition; however, it fairly known. According to experts, this condition affects 15 to 22 people in every 100,000.⁽⁷⁾

This condition affects the frontal and temporal lobes of the brain in the early and middle stages of Fronto-temporal dementia. As the lobes continue to get affected, the patient loses control of certain abilities as the neurons in these affected regions stop working.^(1,2)

The frontal lobe, which is situated behind the forehead, controls the following: decision-making and planning; movement; reasoning and judgment; spoken language and social skills; the ability to distinguish over the right and wrong speech; and control over the right and wrong actions.

The temporal lobes are situated on the sides of the brain and just below and behind the frontal lobe and are responsible for the following: understanding spoken language; hearing; emotional expression, processing and memory.

Deterioration of neurons is the primary cause of fronto-temporal dementia and this occurs when there is a problem in how some proteins are created by the body.^(3,6)

The shape of the proteins is one of the important aspects in determining how they work. This can be compared to how if a regular key is not the right shape, then it will not fit into the lock. Similarly the cells are unable to use certain proteins if they are not of the right shape. The cells also are unable to break these defective proteins to get rid of them. These misshapen proteins then accumulate and get tangled together. As time goes, these defective proteins collect in and around your neurons and destruct the cells till they cannot function at all.

Experts have associated these defective proteins with fronto-temporal dementia and its associated diseases like Pick’s disease. These misshaped proteins also play a role in diseases such as Alzheimer’s disease.

Certain DNA mutations make this protein malfunction more prone to happen.⁽⁷⁾ The cells follow whatever instructions are there on the DNA and even if there are small changes, then it can cause many problems.

There are certain mutations of DNA, which run in families, and this is why around 40% of fronto-temporal dementia cases are due to family history of this condition. These mutations can also occur spontaneously, which means that it was not inherited from the parents.

Other than this, there are two factors which contribute to the risk of this disease. First is having a history of head injury, which triples the risk of developing fronto-temporal dementia. The second is thyroid disease which increases the risk of fronto-temporal dementia about 2.5 times more.

The symptoms of this condition depend on the part of the brain which is affected. Most of the symptoms are similar, however, they can commonly occur in different combinations or they can more or less be acute in different patients.

The symptoms of bvFTD are categorized as:⁽⁵⁾

1. Loss of Inhibitions:

Inhibition is when the conscious part of the brain tells you something, which should or shouldn’t be done. With the frontal lobe deterioration, loss of inhibitions displays symptoms such as:

• Hurtful Speech: Where a person speaks without any “filter” which can be hurtful and offensive and this can seem like a major personality change in some people.
• Disrespecting Others: This involves ignoring an individual’s personal space and touching someone inappropriately and also making inappropriate sexual actions or comments.
• Impulsive Behaviors and Actions: This consists of risky behaviors, like reckless spending or gambling and criminal behaviors such as shoplifting.

2. Apathy:

The symptoms of apathy consist of: Social isolation, de-motivation and ignoring self-care and hygiene.

3. Loss Of Empathy:

Individuals who do not feel empathy towards others have difficulty in understanding what the other person is feeling or going through emotionally.

4. Compulsive Behaviors

The behavior of individuals suffering from fronto-temporal dementia greatly differs from normal people. The changes in the behavior can be small or big. Some of the examples of behavioral changes in patients suffering from fronto-temporal dementia are:

• Repetitive motions, such as tapping their feet or clapping their hands, etc.
• Complex or ritual-type of behaviors, such as reading the same books or watching the same movies over and over again.
• Speech repetition, such as repeating the same phrases, words or sounds.

5. Changes In Diet Or Mouth-Centered Behaviors

Patients having fronto-temporal dementia usually have a symptom, which is referred to as “hyperorality,” where the patient overeats or consumes things which do not belong to the food category.⁽¹⁰⁾ They can also have compulsive behaviors, which are related to mouth, such as smoking or using their mouth to explore things.

6. Loss of Executive Function Without Losing Other Abilities

Executive function is the ability of planning and solving problems, motivating yourself, staying organized etc. Individuals suffering from fronto-temporal dementia have difficulty in executive functions; however, their other abilities, such as the way they process what they see and their memory does not get affected until the last stages of this condition.

This is not a contagious condition and cannot be passed from person to person.⁽⁷⁾ However, if there is a family history of this disease, it increases the chances of you also developing it.

A specialized doctor, such as a neurologist will be able to diagnose this condition based on medical history and a neurological and physical exam. Lab tests and imaging tests are also done where they show the parts of the brain where deterioration is occurring.⁽⁹⁾

Other than neurological examination, neuro-cognitive assessment is also done where the patient is told to perform certain tasks or answer certain questions.⁽⁹⁾ Depending on the performance of this test, diagnosis is made with regards to any problems present in certain parts of the brain and this can help in narrowing down or excluding if a person suffers from fronto-temporal dementia.⁽¹⁰⁾

The common tests done to diagnose this condition are: CT scan, MRI scan, PET scan, spinal tap, electroencephalography and blood tests.

This is no cure for this condition and there is no direct treatment also.⁽⁸⁾ Medications and speech therapy helps in slowing the progression of this disease. Symptoms can be treated or managed; however, the severity of the symptoms differs from patient to patient and the best way to treat and manage this condition is a doctor specializing in treating such conditions.

You cannot predict when this condition can develop and so there is no definite way to prevent this condition. However, one can reduce the risk of developing this disease.

Avoiding head injuries is the primary way to cut down the risk of developing fronto-temporal dementia. Patients with a past history of head injury have thrice the risk of developing this condition. To avoid head injuries, one should always use safety equipment, such as safety restraints and helmets whenever possible, especially in moving vehicles.

Fronto-temporal dementia is a degenerative brain disease, which means there is worsening of this condition as time passes. Memory loss occurs in a later stage of this disease; however, other symptoms continue to develop and worsen with time.

Anosognosia, which means “lack of insight,” leading to problems in the way the brain processes symptoms where the patient is not aware of suffering from this condition. The average life expectancy of patient suffering from this condition after diagnosis is about 7.5 years.⁽¹¹⁾

As this condition gradually worsens, there is deterioration of the ability to control one’s actions and communication, many of the patients cannot live independently and need 24/7 skilled medical care.⁽¹²⁾

Fronto-Temporal Dementia (FTD) is a permanent condition, which lasts lifelong. Fronto-temporal dementia is not life threatening on its own, but it causes other problems, which are serious and can be fatal. One problem, which commonly develops with worsening of this condition, is trouble swallowing (dysphagia). Dysphagia causes problems with drinking, eating, and speaking and also increases the chances of developing respiratory failure or pneumonia.

After having a diagnosis of fronto-temporal dementia, patient should talk to their doctor and family members to entrust them to make important decisions for you. Having these kinds of discussions is vital, as it helps ensure that caregivers are honoring the wishes of the patient when they are not able to choose for themselves in the future.

Having these conversations can be difficult and it is better to have them sooner instead of later to avoid any confusion and to provide clarity about what the patient wants in the future. This will also mean that the family members can confidently move forward with the decision making that this is what the patient wants instead of guessing what the patient would have wanted.

Other than this, it is also recommended that the patient puts their decisions and wishes in writing, which means preparing documents pertaining to legal issues and also what will happen if the patient is not able to look after oneself regarding self-care and other things.

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