Bruce Willis suffers from frontotemporal dementia (FTD), a neurodegenerative disease, related to Alzheimer’s disease, announces his ex-wife Demi Moore on Instagram on February 16, 2023.
[Mis à jour le 17 février 2023 à 11h00] Suffering from aphasia, the state of health of Bruce Willis would have deteriorated. The 67-year-old actor would be with frontotemporal dementia (FTD or frontotemporal degeneration), a neurodegenerative disease similar to Alzheimer’s diseaseannounced his ex-wife Demi Moore on February 16, 2023 on his instagram account. “Since we announced Bruce’s aphasia diagnosis in the spring of 2022, Bruce’s condition has progressed and we now have a more accurate diagnosis: frontotemporal dementia. Unfortunately, communication problems are only a symptom of the disease that Bruce faces“, she wrote on her post. “FTD is a cruel disease that many of us have never heard of and can strike anyone. For people under 60, FTD is the most common form of dementia“, can we read on the website of the AFTD association (The Association for Frontotemporal Degeneration).
Definition: what is frontotemporal dementia?
Frontotemporal lobar degeneration (FTLD) or frontotemporal dementia (FTD) represent the second leading cause of presenile degenerative dementia after Alzheimer’s disease. “We speak more readily now of frontotemporal lobar degeneration (DLFT), the term “dementia” corresponding to an advanced stage of the disease and being difficult for patients and families” says Dr Isabelle le Ber, neurologist specializing in FTDs at the reference center for rare or early onset dementia at Pitié-Salpêtrière and researcher at the Brain and Spinal Cord Institute (ICM). It exists 3 main clinical forms :
- the most frequent form is characterized by inaugural behavioral disorders,
- two other rarer forms are characterized by language disorders hampering verbal communication from the onset of the disease. Behavioral disorders, language and cognitive disorders that are associated gradually evolve towards a loss of autonomy (corresponding to the stage of ‘dementia’).
The prevalence of frontotemporal dementia is 10 to 15 in 100,000 individuals. It is estimated that 6,000 to 10,000 people in France are affected.
What causes frontotemporal dementia?
Frontotemporal dementias are caused by the progressive alteration of certain areas of the brain (frontal areas at the front of the brain and temporal areas at the sides of the brain). “Three types of lesions can be seen in the brains of patients with FTD. They consist of three different proteins (TDP-43, TAU or rarely FUS) which accumulate in brain cells in each of these 3 forms“, says Dr le Ber. The cause of the disease and the constitution of these lesions are not known in the majority of cases, but sometimes a genetic cause can be identified. These genetic causes concern about 20% of patients, usually when other people in their family also have FTD. The majority of patients do not have a genetic form, in which case the cause remains unknown.
What are the differences with Alzheimer’s disease?
Frontotemporal dementias that begin around 60
Frontotemporal dementia and Alzheimer’s disease are so-called ‘related’ degenerative dementias because they are both characterized by cognitive disorders. However, they are actually very different diseases. Alzheimer’s disease, which is the leading cause of dementia generally begins later (around 70-80 years) than frontotemporal dementias which begin around 60. “The symptoms are different: Alzheimer’s disease generally begins with memory problems, whereas the first problems in FTD are behavioral and language disorders. Finally, the regions of the brain that are affected (frontal and temporal lobes in FTD; whereas it is the ‘hippocampal regions’ of memory that are primarily affected in Alzheimer’s disease) and the proteins that constitute the lesions in the brain are also different“describes the neurologist.
What are the symptoms of frontotemporal dementia?
Fronto-temporal dementias are manifested, at first, by behavioral and language disorders before later affecting other cognitive and neurological functions. The manifestations of the disease vary according to the person and according to the stage of evolution of the disease. The symptoms which generally begin between the ages of 50 and 65 and, in the most classic form, are represented by:
- an apathyloss of interest in usual activities, social isolation
- Behavioral disorders marked by inappropriate social behaviordisinhibition, irritability,
- Eating disorders: food rush, bulimia, change in food tastes…
- Disorders of emotional control: emotional instability, emotional blunting, inappropriate emotionality
- Behaviors repetitive Or stereotyped
- Language disorders (reduction of language) and cognitive (difficulties in concentration and attention, difficulties in reasoning, judgment, etc.) are associated and worsen with development. When the disease progresses, the sick person gradually loses their autonomy and becomes unable to carry out daily activities on their own.
Diagnosis: are there any tests to be done?
The diagnosis is suspected by a physician and made by a neurologist or medical specialist. It is based on a series of tests to assess the person’s cognitive functions. These cognitive tests also help to assess the stage of disease progression. “The diagnosis is confirmed by brain imaging examinations: brain MRI, often supplemented by an examination of brain metabolism by PET-FDG. These imaging tests may reveal abnormalities (‘atrophy’ or ‘hypometabolism’) of the frontal and/or temporal regions“adds Dr. Isabelle Le Ber. Biological examinations, a lumbar puncture or an electroencephalogram can be carried out in order to eliminate other pathologies, in particular Alzheimer’s disease.
What are the treatments to cure it?
No treatment can cure frontotemporal dementia.
No treatment can cure frontotemporal dementia. Nevertheless, treatments can improve the most bothersome symptoms (aggressiveness, wandering, etc.) such as certain anti-depressants. Support must be supplemented by a work of cognitive stimulation to maintain cognitive functions as much as possible, but also by social and human aids. The care of people with frontal dementia is multidisciplinary including doctors, psychiatrists, speech therapists, and must be supplemented by psychological care for the patient and support from those close to him. Help from patient associations is also important (France-DFT, France Alzheimer, etc.).
What are the complications?
People with frontotemporal dementia most commonly die from complications of the disease (falls, bedridden complications, miscarriages…)
What is the life expectancy in case of frontotemporal dementia?
Life expectancy from diagnosis averagesten years. Nevertheless, it is very variable according to the people and there can be longer evolutions. There are currently no factors that can give a precise idea of the speed of progression. In twenty years, important research advances have been made with improved diagnostic criteria and clinical classification of these diseases, identification of the proteins that constitute brain lesions, identification of the genetic causes and development of tools that improve differential diagnosis with other diseases such as Alzheimer’s disease. “Thus, we now know that there is not a DFT, but different DFTs with clinical presentations, and different lesions. We now know much better about these different forms of the disease, which makes it possible to better diagnose them and improve their management. Current research focuses on understanding their pathogenic mechanisms, identifying biomarkers for their diagnosis and monitoring disease progression, and of course finding treatments. In this area, ofInteresting therapeutic leads are being developed as part of research protocols, particularly in genetic forms of the disease” develops the neurologist.
Thanks to Dr Isabelle le Ber, neurologist specializing in FTDs at the reference center for rare or early onset dementia at Pitié-Salpêtrière and researcher at the Brain and Spine Institute (ICM)