Frontotemporal dementia, the neurodegenerative disease masquerading as depression

But this person actually suffers from a frontotemporal dementia (called frontotemporal lobar degeneration, FTD), a disease characterized by the progressive degeneration of the frontal and temporal lobes of the brain. This is a minority disease, which in Spain affects about a thousand people a year. It is less frequent than Alzheimer’s disease, but just as frequent as amyotrophic lateral sclerosis (ALS). Its incidence is 22 cases per 100,000 inhabitants, but physicians warn that there is a underdiagnosis because, and here’s the drama, it is often Is mistaken for depression or anxiety. It often takes years to be diagnosed and is often doubt of the story of the family who go in desperation to seek medical help.

In Spain, the Acensa association gives support to the families of patients with FTD. Based in Andalusia, its members include 3,000 people, calling for patients to be cared for “earlier” and that there is “a good triage” of the disease, in the words of its president, Rosa Barrios. “The Hospital de Sant Pau is the model to copy,” he insists.

Sant Pau, the benchmark

The Memory Unit of the Hospital de la Santa Creu i Sant Pau, at Barcelona, has a lot of experience in this neurodegenerative disease and even receives patients from other communities. “The media of diagnosis of frontotemporal dementia is two to four years. They are diseases relatively rare, described not long ago and not routinely seen by neurologists. No specific training [como sí hay en EEUU]”, explains Ignacio Illán-Gala, neurologist at the Sant Pau Memory Unit.

Frontotemporal dementia is characterized by an progressive personality change, which stands out above the possible cognitive changes, which explains why most patients preserve their mental faculties and are fully independent for activities of daily living. The person becomes socially isolated and appears cold and distant with loved ones. It is often confused with major depression, but not alone.

“It can also be confused with the bipolar disorder, characterized by a phase of depression and a phase of mania. Patients with both diseases want to make many plans thoughtlessly. Also with the disease of Alzheimer’s disease. And more rarely it may be confused with personality disorders or with an schizoaffective disorder.” Illán-Gala points out. proof determines that what this person has is frontotemporal dementia? The problem, says this neurologist, is that, as with most neurodegenerative diseases, there is “no single test” to detect it. Hence, the difficulty of diagnosis.

That is why it is important to “assess the the clinical picture as a whole and to obtain key information from the family”, and to give veracity to its account. “The environment helps to identify the chronology of changes and allows you to identify and reconstruct the patient’s personality change. But the existence of a personality change or a behavioral syndrome is not enough. Then there are others a lot of evidence that can be done, and it is the combination of all of them that makes it possible to increase the certainty of the diagnosis of frontotemporal dementia,” says this neurologist. A magnetic resonance imaging brain allows us to see if there is a volume loss frontal or temporal brain, consequence of the disease. The genetic testing also help to see if the patient is a carrier. Between 10% and 20% of cases are caused by a genetic mutation, but the rest are not.

No cure but with treatment

Although it is a disease without a cure, there is a symptomatic treatment for frontotemporal dementia that improves the quality of life of patients and families. That is why diagnosis is important. Treatments are also beginning to be tested potentially curative in some genetic cases.

Based on his own experience and in epidemiological studies, many experts consider this disease to be underdiagnosed, although this underdiagnosis is not “dramatic”. The percentage is unknown. But, in any case, it is “frequent”. delay in diagnosis, as is the case with diseases such as early-onset Alzheimer’s disease. (before the age of 65 years), which may be characterized by symptoms such as anxiety, low mood, nervousness, sleep problems, or difficulty concentrating or performing at work.

Personality change

“They are diseases that Are mistaken for anxiety or depression and may be the home of a neurodegenerative disease,” Illán-Gala notes. But there is a difference between FTD and Alzheimer’s disease: the former affects the specific neurons in the frontal lobe that make people “social beings”, That is to say, that make them “function in society”. That is why this dementia directly alters the personality of the sufferer. The patients begin to do things without thinking of the consequences (e.g., compulsive shopping), they suffer from apathy and begin to lack empathy: stop caring about the people around them, become cold and distant.

“Anyone can develop an anxious or depressive disorder at any time in life. The important thing is see if he or she responds well to medications. and if he begins to show a change in personality. These are the two characteristics that make us think of FTD,” he says. Javier Pagonabarraga, neurologist at the Movement Disorders Unit of Sant Pau, who works together with the Illán-Gala Memory Unit and the ALS unit for the diagnosis of patients with FTD. Both work with a “common agenda” thanks to which they have managed to have a “referral psychiatrist” with whom they discuss the most complex cases.

The clinical neuropsychologist at the Movement Disorders Unit of Sant Pau, Saül Martínez-Horta, insists strongly on “contemplate” always other symptoms so that diagnoses are not limited to anxiety or depression, since behind them there may be serious brain problems. He therefore calls for interventions to be multidisciplinary. “When you explore the cognition of people with diseases such as FTD you see abnormalities that are not consistent with the most essential models of depression, for example,” Martínez-Horta explains. For example, there are sometimes memory lapses or language problems that already indicate a “neuropsychological profile” different from the initial one. “Hence the need for the neuropsychological approach to see if there are atypical symptoms that indicate something else is going on,” he concludes.