After the 67-year-old American actor and producer ended his career in March 2022, Bruce Willis’s family announced Thursday that he has frontotemporal dementia. But what is this?
Frontotemporal degeneration (FTD), also called frontotemporal dementia or Pick’s disease, are rare neurodegenerative diseases, indicates the Brain Institute (ICM), a major research center located in Pitié-Salpetriere, in Paris.
FTDs are sometimes associated with Alzheimer’s disease, although in the latter it is memory that is affected, recalls the France-FTD association. In the case of DFTs, social behaviors and language are affected.
“The onset of the disease is insidious”
Because there are various forms of the disease, Isabelle le Ber, neurologist at the Pitié-Salpêtrière hospital and ICM researcher within the team of molecular bases, pathophysiology and treatment of neurodegenerative diseases, explains to BFMTV.com. “There are behavioral forms, which are the most common, and other linguistic ones. We speak then of primary progressive aphasia”.
The patient may thus present difficulties in pronouncing words, suffer loss of vocabulary, comprehension problems or difficulties in identifying objects or people. As was the case with Bruce Willis: last year, relatives of the star announced that he suffered from aphasia that affected his cognitive abilities.
Among the first signs: apathy, personality change, indifference to emotions and so on. “In the early stages of FTD, behavioral disturbances are discrete and can evoke the effects of fatigue, stress,” warns the ICM on a page dedicated to symptoms and diagnosis.
Most noticeable symptoms
The cause of the disease – dysfunction of the frontal and temporal regions of the brain. Regions that are involved in social behavior, but also take initiative or control emotions. “In these pathologies, the affected brain areas are reduced (atrophy) as a consequence of the death of neurons,” writes the ICM, which carries out various investigations on the disease.
“The onset of the disease is insidious”, abounds Isabelle le Ber. “When not all of the suggestive symptoms are met, there may be confusion with a diagnosis of depression or Alzheimer’s disease.”
Then other symptoms appear, this time more visible: behavior problems, great disinhibition or inability to respect social codes -with, for example, inappropriate reflexes-. “It can also be a great agitation or even aggressiveness, escapes or the development of obsessions”, details the ICM.
“The disease advances”
Patients may also act in strange ways, such as continually repeating the same gestures, becoming obsessed with certain habits or objects, or becoming compulsive collectors. Some patients also have an irrepressible urge to move and walk several kilometers without a specific goal.
Changes in eating behavior are also observed in many patients: some eat excessively and in a hurry, others have phases of bulimia or anorexia. It also happens that patients are neglected from the point of view of hygiene.
“Whatever the form, the disease progresses towards a similar clinical picture”, adds Isabelle le Ber. “The injuries give rise to symptoms that are the same. What distinguishes them is the brain localization of these symptoms.” And as the disease progresses, patients may become confused, their judgment impaired, and they find themselves unable to do simple things.
A difficult diagnosis
Family members are often the ones who suspect the disease when they identify changes in behavior, a change in personality or difficulties in expression. But the diagnosis is difficult to make.
Neurologist Isabelle le Ber acknowledges that patients sometimes find themselves in a diagnostic wandering, especially in cases of atypical forms. “But usually the diagnosis is made within two to three years of symptom onset, although more subtle and less specific reports are reported by families many years earlier.”
As for the speed at which the disease progresses to severe dementia, it varies from case to case. But the patient’s condition deteriorates over time.
“In general, the evolution of the disease lasts about ten years, although there are faster or slower forms,” specifies the neurologist. If the disease is not fatal, the complications are. “When the patient becomes bedridden, he may have trouble swallowing or have a stroke.”
an incurable disease
The disease is incurable. However, some treatments can alleviate behavioral disorders, such as antidepressants or anxiolytics that reduce agitation with an effect on appetite regulation or irritability. Patients are also usually followed in speech therapy or even in day hospitals to offer them adequate rehabilitation.
“There is hope with the research working on experimental protocols”, Isabelle le Ber qualifies. But it is not for now.
Frontotemporal degeneration affects both men and women and represents 10% of dementias. In 20% of cases, the origin of the disease is genetic. In France, between 15,000 and 20,000 patients are affected by this disease.
Source: BFM TV
