Frequently asked questions of frontotemporal dementia

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What causes frontotemporal dementia?

Frontotemporal dementia is a neurodegenerative disease. The loss of neurons is due to the pathological accumulation of certain proteins in the brain. The most common are tau protein and TDP-43 protein. When these proteins present alterations, they accumulate in neurons and prevent them from functioning properly. In most cases, it is not known what causes these proteins to alter.

Which part of the brain is affected?

As the name suggests, in frontotemporal dementia neurodegeneration occurs mainly in the frontal and temporal lobes of the brain. These parts of the brain are responsible for managing social conduct, behaviour and language.

Is it a common disease?

No. Frontotemporal dementia is considered a rare disease. It is estimated to affect around 200 in every million people.

What are the initial symptoms of the disease?

Frontotemporal dementia can present with very different symptoms depending on which areas of the brain are most affected in each case. The most common initial symptoms are changes in behaviour. This includes apathy, lowered inhibitions, loss of social behaviour, loss of empathy, compulsive actions and changes in dietary preferences. If the left side of the brain is affected by neurodegeneration, the disease may start with language problems such as difficulty speaking or understanding.

What types of frontotemporal dementia are there?

Three types of frontotemporal dementia can be distinguished, depending on the initial symptomatology. Firstly, in cases where the initial symptom is behavioural impairment, we talk about behavioural variant frontotemporal dementia. If the initial symptom is language impairment, it is known as primary progressive aphasia. Depending on the type of language impairment, primary progressive aphasia can either be agrammatic or semantic.

At what age do the first symptoms appear?

The average age of onset of frontotemporal dementia is around 60 years, but the disease can begin at a wide range of ages, from 40 to 80 years. Frontotemporal dementia is the second leading cause of dementia in people under 65 years of age (the leading cause is Alzheimer's disease).

Are there also motor symptoms?

Yes. Motor symptoms are common in the disease and often become very debilitating. These may include tremors, stiffness, weakness in the limbs, falls and issues with walking.

What is the relationship between frontotemporal dementia and amyotrophic lateral sclerosis?

In recent years, scientific research has found evidence that there is a close link between frontotemporal dementia and amyotrophic lateral sclerosis (ALS). Many patients with frontotemporal dementia eventually develop ALS, while many patients with ALS develop frontotemporal dementia. The two diseases share molecular and genetic links.

What tests are performed to diagnose frontotemporal dementia?

The diagnosis of frontotemporal dementia is clinical. This means that the doctor makes the diagnosis based on interviews with the patient and their caregiver, as well as neurological examinations. However, it is common to perform additional examinations to improve diagnostic confidence. The most common tests are neuropsychological assessment, brain MRI, positron emission tomography and lumbar puncture.

How is the disease treated?

There is no cure for the disease. However, there are treatments that can help to improve the symptoms of the disease. In cases of language impairment, speech therapy can be used to preserve communication skills.

How can I better cope with behavioural impairments?

It can be difficult to manage behavioural disturbances in frontotemporal dementia. There are strategies to help improve the patient's behaviour. An occupational therapist can help to find useful strategies. There are also treatments that can help to control these behaviours.

What does speech therapy consist of?

Speech therapy has two objectives in patients with language impairment: on the one hand, it helps them to maintain their preserved linguistic mechanisms and, on the other hand, it provides tools to compensate for lost linguistic skills.

What is the prognosis of the disease?

Frontotemporal dementia is a progressive neurodegenerative disease. Dementia worsens as time passes, affecting other cognitive functions until it reaches the stage of severe dementia.

Can the patient choose their treatment?

In many cases, the disease leads to changes in behaviour and psychological capacities that make it difficult for the patient to make decisions about their future. In these cases, it is essential to designate a legal representative who will be the point of contact with the healthcare team. When the patient is able to make decisions about their future medical actions, it is highly recommended that they be reflected in an Advanced Directive Document.

What are the legal impacts of the actions of a patient with frontotemporal dementia?

Patients with frontotemporal dementia may perform impulsive actions without thinking about the consequences. Sometimes these actions are against the law, and may have legal repercussions. For this reason, it is important that the competent authorities assess the legal capacity of each patient. If you live in Catalonia, head to Legal Guidance Service for more information. This is a free public service, through which a face-to-face visit can be arranged and general guidance in various specialities can be provided. The Municipal Institute of Social Services also provides free legal advice to users of the Social Services Centres.

Can a person with frontotemporal dementia drive?

Driving a car is a complex activity that involves several cognitive functions that are impaired in patients with frontotemporal dementia. For this reason, patients with frontotemporal dementia should not drive.

Is frontotemporal dementia hereditary?

The majority of cases of frontotemporal dementia are not hereditary. However, in a significant minority of cases (around 3 in 10) the disease is due to a genetic mutation that can be inherited from parents. These mutations can be found in different genes. The genes C9orf72, GRN and MAPT are the most common.

There is someone with frontotemporal dementia in my family. How can I find out whether I will also develop the disease?

If only one family member is affected, it is very unlikely that it is a heritable case. When frontotemporal dementia is caused by an inheritable genetic mutation, there is usually more than one case in the family. Nevertheless, doctors can offer you genetic counselling, in which they inform you about the chances of the disease being genetic.

I don’t know anybody in the same situation. Who can I share my experiences with?

As frontotemporal dementia is a relatively rare disease, relatives often do not know anyone in the same situation with whom they can share their experiences. It may be useful to know that there are associations for relatives of patients with this condition.

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