“Bruce disease has progressed and we now have a more accurate diagnosis: frontotemporal dementia. On Thursday, February 16, Bruce Willis’ family released a statement providing information on the actor’s condition. Last March, the American star had ended his career due to aphasia. In one year, his disease progressed.
Frontotemporal degeneration (FTD), also known as frontotemporal dementia, belongs to the family of cognitive and behavioral neurodegenerative diseases. They are said to be related to Alzheimer’s disease because they lead to cognitive disorders, but affect different regions of the brain, mainly the frontal and temporal lobes.
Generally, the first symptoms appear between the ages of 55 and 65. “FTD manifests as behavioral disturbances such as apathy, personality change, social behavioral disturbances, familiarity, sometimes disinhibited, obsessive or stereotyped behaviors, emotional change. Eating behavior is often modified with a tendency to gluttony, and changes in food tastes, “explains Dr. Isabelle Le Ber, neurologist at AP-HP (Paris). In other patients, this disease begins with language difficulties, we then speak of primary progressive aphasia. Unlike Alzheimer’s disease, memory is little affected, and cognitive disorders mainly affect executive functions, leading to problems with attention, organization, planning, etc.
There are two forms of frontotemporal degeneration, as the neurologist explains: “In approximately 60% of cases, the disease is linked to the aggregation of an abnormal protein, the protein TDP43, in the cells of the brain. In 30% of cases, another protein, the tau protein, is involved. These lesions are different from those observed in Alzheimer’s disease. The causes of this disease are not known, apart from genetic causes which are found in approximately 20% of patients.
Frontotemporal degeneration evolves gradually over ten years. The diagnosis is evoked in front of the progressive installation of behavioral modifications or of the language, and rests on examinations of cerebral imagery, tests evaluating the cognition and the language, biological analyzes. “Today, there is no treatment to cure it, but medico-social care is offered to patients. It is based, among other things, on symptomatic treatments to improve certain behavioral disorders, approaches to cognitive stimulation, language (speech therapy), psychological support for patients and their caregivers, social aid”, explains Dr Le Ber .
Before concluding: “The research is progressing. Currently, research on therapeutic trials is beginning in certain genetic forms. Even though these are experimental trials at the moment, this is an important change and hope,” underlines Dr. Le Ber. About 15,000 to 20,000 French people suffer from this form of the disease.
