The expert in neuropsychology, Cristina Troyano Jiménez, explains in this article what frontotemporal dementia is, its types, symptoms, causes, diagnosis, and treatment.

What is frontotemporal dementia?

Frontotemporal dementia (FTD) is a dementia with a lower incidence that affects younger people (40-65 years old). It is the second leading cause of presenile dementia, with the affected areas being the frontal and temporal lobes (which atrophy).

The most common symptoms are related to behavior and personality. Patients with frontotemporal dementia can become challenging to manage. They may cause numerous conflicts due to personality changes, even resulting in clashes with others, including their family members.

Types of frontotemporal dementia

Frontotemporal dementia affects the frontal and temporal lobes, leading to atrophy of these areas. Unlike other dementias, it causes changes in behavior, language and speech problems, and in some cases, movement disorders.

1. Frontotemporal dementia with behavioral problems

These patients experience personality changes. Their behavior is affected, showing emotional alterations that can range from being highly agitated to complete inhibition unless prompted to move.

Among the personality changes, we find disinhibition and increasingly inappropriate behaviors:

• Neglecting personal hygiene.
• Increased interest in sex, sometimes developing Klüver-Bucy syndrome (increased interest in sex, lip-smacking, and putting objects in the mouth).
• More frequent use of obscene language.
• Engaging in repetitive behaviors.
• Becoming more impulsive or compulsive.

2. Frontotemporal dementia with aphasia

In these cases, language is primarily affected, leading to difficulties in speaking, communicating, reading, writing, and understanding.

Language impairments begin with problems such as word-finding difficulties and articulation, progressing until communication becomes nonexistent. In some cases, language symptoms may be the only ones present for years in this type of dementia. However, the disease can progress more rapidly in other instances. As the condition advances, patients often speak less until they stop speaking altogether.

3. Frontotemporal dementia with movement disorders

There is impairment in movement, which can lead to frontotemporal dementia with parkinsonism or frontotemporal dementia with amyotrophic lateral sclerosis.

One type of frontotemporal dementia is Pick’s disease, characterized by severe atrophy, cell death, and the presence of abnormal Pick cells.

Symptoms of frontotemporal dementia

• The symptoms of frontotemporal dementia are progressive, but their evolution varies among patients,
• they affect personality, behavior, and language,
• while memory impairment is less prominent compared to other dementias,
• additional symptoms include difficulties in abstract thinking, attention, and recent memory,
• planning, organizing tasks, and developing ideas are also impaired,
• patients are easily distracted but often remain oriented and can carry out activities of daily living,
• other symptoms may include dysarthria (speech articulation difficulties),
• Those who have aphasia often experience weakness in the head and neck muscles, which results in swallowing difficulties.
• as well as anomic aphasia (failing to recognize objects) and prosopagnosia (failing to recognize faces).

Causes of frontotemporal dementia

The causes of frontotemporal dementia are not fully understood. However, patients with this condition often exhibit an abnormal accumulation of proteins called TAU and TDP-43 in their neurons, which leads to damage and subsequent cell death.

In some cases, there may be a genetic mutation associated with frontotemporal dementia, particularly in the behavioral variant. It is believed that between 10% and 30% of frontotemporal dementia patients have a genetic cause, especially when there is a family history of the condition.

Diagnosis of frontotemporal dementia

Diagnosing frontotemporal dementia is complex since its symptoms can be confused with other forms of dementia. It is not uncommon for patients to initially receive a misdiagnosis of Alzheimer’s disease due to its lower frequency and lesser known nature. In these cases, the prescribed medication may also be inappropriate.

This highlights the importance of an accurate diagnosis. Through many years of observation, I have witnessed cases where medication failed to alleviate symptoms, but once the correct diagnosis was made, the prescribed treatment led to improvement. Fortunately, we now have neuroimaging techniques available that can identify the specific brain areas affected, thus confirming the diagnosis.

Among the innovative neuroimaging techniques are brain magnetic resonance imaging (MRI) and cortical mean diffusivity, which provide more precise diagnoses.

Initially, the diagnosis of frontotemporal dementia is usually based on interviews with the patient and their family, complemented by neuroimaging tests. In cases where a hereditary condition is suspected, a genetic test can provide confirmation.

Unfortunately, a definitive confirmation is often only possible through postmortem brain autopsies, as is the case with most forms of dementia.

Treatments for frontotemporal dementia

1. Pharmacological treatment

Pharmacological treatments for frontotemporal dementia include the use of antipsychotics for patients with behavioral problems, particularly compulsive behaviors. Antidepressants are also prescribed, and medications targeting movement problems are used when necessary.

One of the new treatments that has been implemented involves the use of Sodium Selenate. This compound increases the enzyme protein phosphatase 2A, which breaks down the TAU protein. Patients with dementia exhibit significant accumulation of TAU protein in their neurons.

2. Non-pharmacological treatment

Non-pharmacological treatments for frontotemporal dementia vary depending on the specific symptoms and needs of each patient.

For patients with language problems, speech therapy is important.

Physiotherapy is recommended for patients with movement issues.

In general, occupational therapy and cognitive stimulation are beneficial. Although orientation and memory are less affected in comparison to other dementias at the initial stages, it is still important to work on all areas of the brain.

Establishing a daily routine is particularly important for these patients.

Progression of frontotemporal dementia

The progression of frontotemporal dementia differs from other dementias, such as Alzheimer’s disease. It is usually progressive and varies from patient to patient.

In some individuals, the disease progresses slowly, with a course of around ten years, while in others, it advances more rapidly, influenced by the type of lesion. For example, a patient with aphasia will eventually develop swallowing problems such as dysphagia, leading to a different disease course compared to a patient with mobility or behavioral problems.

The behavioral variant typically progresses more rapidly, while the aphasic variant has an intermediate course.

The average life expectancy for frontotemporal dementia is between 6 and 8 years, with a maximum of 15 years.

What is important in all dementias, apart from an early and accurate diagnosis, is dealing with it as soon as possible through different options: cognitive stimulation, physiotherapy, occupational therapy, etc.

I have been working in this field for many years and I have seen patients of all kinds. I have also seen how all these techniques have slowed down the progression of the disease. While it is true that in the last three years, during the pandemic, many patients have stopped going to day care centers and their condition has worsened more quickly. Healthcare has somewhat neglected specialties due to COVID-19 protocols, and many individuals have been diagnosed at advanced stages where both pharmacological and non-pharmacological treatments come late, resulting in highly advanced patients with behavioral problems. Working with them becomes complicated. These are some of the collateral problems of a pandemic that paralyzed everything.

Conclusions

Frontotemporal dementia, like many dementias, is a psychosocial disease because it not only affects the patient but also their environment, caregivers, and family members.

Perhaps we are now reaching the peak of this silent pandemic that increasingly affects more people due to the aging population we are witnessing. Much progress has been made in understanding the causes, medication, technology, diagnosis, and day-to-day management through various therapies.

There is still a long way to go. Undoubtedly, advances in genetic engineering and technology will help in the future for very early diagnosis and even for eradicating the disease someday, just as many diseases have been in the past. But for now, we continue to combat the disease day by day using the means available to us, striving to improve the quality of life and dignity of patients and their environment.

Alzheimer’s erases memory, not feelings.

Pascual Maragall

References

• Parkin, A. (2003). “Exploraciones en Neuropsicología,” Editorial Médica Panamericana.

• Carpintero H, Delius J, Fierro A, León Carrión A. (2001), “Neuropsicología Cognitiva, algunos problemas actuales.” Ediciones Aljibe.

• HYPERLINK “https://www.msdmanuals.com/es-es/hogar/authors/huang-juebin” Juebin Huang, MD, PhD, Department of Neurology, University of Mississippi Medical Center. Ultimo revisión completa mar. 2021.
• https://www.mayoclinic.org/es-es/diseases-conditions/frontotemporal-dementia/diagnosis-treatment/drc-20354741.2022
• https://www.alzheimers.gov/es/alzheimer-demencias/demencia-frontotemporal.2022

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