This disease is characterized by severe and progressive cognitive impairment, mainly affecting behavior and personality, as well as language and other functions. It represents 5% – 10% of dementia cases worldwide.

Generally, when memory disturbances are evident, people are automatically diagnosed with senile dementia, because in the imagination of much of the population, this term is the ideal one to encompass all types of dementia. However, it is important to note that there are only two: on the one hand, degenerative dementia (the most common are Alzheimer’s disease and frontotemporal dementia) and, on the other hand, vascular dementia (predominantly ischemic and hemorrhagic).

Alzheimer’s disease is probably the best-known neurodegenerative disease, accounting for 60%-70% of dementia cases with significant cognitive, motor, functional, emotional, and social consequences, according to the World Health Organization (WHO). However, in recent weeks, frontotemporal dementia has been in the media spotlight following the unfortunate news that the renowned actor Bruce Willis, 67, suffers from this little-known condition.

While it is true that last year the star of the film “Die Hard” announced his retirement from acting after being diagnosed with aphasia, however, this disease characterized by language impairment is ultimately what all patients with some form of dementia will suffer from at some point, said Manuel Moquillaza Valle, clinical and endovascular neurologist, and coordinator of the neurology service of the Clinica Ricardo Palma to Bienestar de El Comercio.

To learn more about frontotemporal dementia, the neurologist developed some important points that we have to keep in mind regarding this terrible disease.

What is frontotemporal dementia?

It is a degenerative disease in which there is an accelerated aging of brain neurons, mainly in the frontal lobe (social skills, movement and verbal expression) and temporal lobe (short-, medium- and long-term memory, olfaction, hearing and language comprehension area). Generally, its onset is early and accelerated, as there is a progressive process of deterioration in people over 60 years of age.

What are the causes of frontotemporal dementia?

The causes are not determined because, being a degenerative disease, there is a genetic predisposition. However, the risk factors are low mental activity, alcohol consumption, sedentary lifestyle, obesity, and lack of some vitamins.

What are the types of frontotemporal dementia?

Basically, there are two main groups: the behavioral variant, in which behavioral alterations predominate, and primary progressive aphasia, characterized by language alterations, i.e., the patient has problems in expressing himself, reading or writing.

What are the signs and symptoms?

The most common are speech and language problems, behavioral changes, such as disinhibition, as the patient presents inappropriate behaviors in public, apathy, hyperorality and repetitive and compulsive behaviors. In addition, there is a possibility that 15% to 20% of patients with behavioral variant develop motor neuron disease, specifically amyotrophic lateral sclerosis.

How many stages does frontotemporal dementia have?

Unlike Alzheimer’s disease, frontotemporal dementia presents in the first stage changes in behavior and later in memory. It should be noted that there are stages according to the degree: mild, the symptoms are almost imperceptible, moderate, 70% of cases are usually diagnosed and severe, where the disease is presented at its maximum expression.

How is a proper diagnosis made?

First, it must be determined whether there is a diagnosis of cognitive impairment -mild, moderate, or severe- before talking about dementia. It is worth mentioning that dementia is considered when the impairment is severe, so then the cause must be sought, since cognitive impairment can be primary or secondary. For example, those of degenerative origin, such as Alzheimer’s and frontotemporal dementia are primary, while vascular impairments are classified as secondary.

In short, the diagnosis is complex, since not only a neurological evaluation and some laboratory tests must be performed, but also brain imaging tests, such as dementia protocols, specifically, a spectroscopy with which an increase of the metabolite myoinositol and a decrease of the metabolite N-acetyl-L-aspartate are evidenced in these patients, as well as a hippocampal volumetry, with which a level below the average is usually observed.

What does the treatment consist of?

Frontotemporal dementia is a disease that still has no cure, although it is addressed with pharmacological treatment based on brain stimulants, such as memantine, an NMDA receptor antagonist, which has shown some degree of efficacy in all types of dementia, especially vascular dementia. Donepezil, an acetylcholinesterase inhibitor, is also used and is recommended for patients with degenerative dementias. In addition, psychological treatment is provided, since a person with dementia requires emotional support, as well as psychiatric support, especially if behavioral disturbances are present. Likewise, it is essential to aid, since this group of patients requires care to maintain their safety, hygiene, and nutrition, in other words, quality of life.

How long can a person with frontotemporal dementia live?

Undoubtedly, much depends on the care that the patient receives, because thanks to this, life expectancy can be longer than the average of approximately 5 to 10 years. Indeed, this is since mental functions are compromised, and the patient is dependent on others, so that if proper care is not provided, his/her health may deteriorate more rapidly or even die from adjacent complications.

Is there any way to prevent frontotemporal dementia?

The more mental activity, the lower the probability of developing some type of dementia. For this reason, it is key to perform exercises of mathematical ability, psychomotor, verbal reasoning, psychotechnical, i.e., Sudoku, checkers, chess, alphabet soup, learning languages, among others.

Likewise, a healthy diet rich in vitamin E is essential, since it is an antioxidant that favors the preservation of memory and can be found in fruits, green leafy vegetables, peanuts, broccoli, pecans, raisins, hazelnuts, avocado, etc.

Finally, Moquillaza stressed that it is important for the population to know that in the face of unusual behavioral changes and progressive memory loss they should see a neurologist, since people are often underdiagnosed with senile dementia, especially in Peru, and tend to be satisfied with this reality.

However, with an accurate and early diagnosis, an adequate treatment can be carried out and a prognosis can be determined.

Dr. Manuel Moquillaza
Clinical neurologist, endovascular and
Coordinator of the Neurology Service of the Clinica Ricardo Palma

Source: elcomercio.pe