Bruce Willis, front -time dementia and because his brain is “yielding”

Bruce Willis “is very well” physically, but his brain “is giving up”, compromising his ability to communicate with the outside world: his wife Emma Heming revealed it in a recent interview with ABC. «Bruce still moves and is in good health. It is only his brain that is betraying him, “he specified Heming to Diane Sawyer during the special Emma & Bruce Willis: The Unexpected Journeyof which a preview has been transmitted.

The actor’s family, famous for the role of John McClane in Die Hardhe had announced in 2023 the diagnosis of a form of dementia that affects the personality and can cause changes in behavior: retreating from the scenes, then more specific was spoken of Frontotemporal dementia (FTD).

For Willis, 70 years old, the main symptom was the loss of language, explained his wife, author of the book The Unexpected Journey: Finding Strength, Hope, and Yourself on the caregiving Path: «Language leaves, but we have learned to adapt. Now we have a different way of communicating with him ». Just following the aggravation of his conditions, Bruce Willis lives today in a separate house with 24/7 assistance: The decision “was not easy, but that’s what Bruce would have liked for his daughters”

What is frontotemporal dementia (FTD)

There Frontotemporal dementia (FTD) – We read on Dementia Observatory – «It is one of the main causes of dementia a early debutand it is the second more common among people with less than 65 years “.

Over the past ten years, the concept of FTD has evolved: initially known as pick diseaseToday it is identified as a heterogeneous group of neurodegenerative disorders. These disorders affect i Frontal and temporal lobes of the brain, fundamental areas for the regulation of behavior, personality, emotions, language and understanding. The onset of working age often has an important impact on the personal, social and professional life of the patient. In particular, the behavioral variant can cause changes marked in judgment, social conduct and self -controlsometimes leading to legal problems even before a diagnosis is formulated ».

The main clinical forms of the FTD are:

• Behavioral variant (BVFTD): It manifests itself with apathy, disinhibition, impulsiveness and alterations of personality and social relationships.
• Semantic dementia: the language is fluent but without significant content; The patient gradually loses understanding the meaning of words.
• Progressive non -fluent aphasia: It is characterized by difficulties in the articulation of the word, in reading and writing, up to a possible mutism.
• FTD associated with Motoneurone disease: Includes neurological signs such as muscle weakness and atrophy.

Numbers, age, incidence

In the absence of a national official register, to estimate numbers in Italy, we are based on European data. According to the multicenter study Frustiersconducted between 2018 and 2019 in 13 specialized clinics (including Italy), theaverage annual incidence of the FTD in Europe is approximately 2.36 cases per 100,000 people. This is equivalent to About 12,000 new cases every year in Europe. In Italyconsidering precisely the European average and the population, it is estimated that The sick are a few thousandeven if the precise figure is not made official.

Average age of debut

One of the most distinctive aspects of frontotemporal dementia is the early age of onset: generally affects people among the 40 and 65 years. In this range it focuses about the 70% of cases. However, the study Frontiers (also reported in Italy by Omar) indicates that the incidence peaks around 71 yearsparticularly in men, which affects almost double compared to women.

Symptoms

As he declares to the site Omar The Dr. Giuseppe Di Fede, Head of the Genetics and Biochemical Laboratory of Dementia, Complex Structure of Neurology – 5 Neuropathology, Besta di Milano Institute«The manifestations with which front -time dementia can present themselves are classified in 3 categories: Cognitive, behavioral and motor symptoms. In the sphere of cognitive disorders, the Language changes they are the most represented and between them there is theaphasiawith which the FTD had the debut in the case of Bruce Willis. In a first phase, those who suffer from it has difficulty expressing themselves and do not find the right words to make themselves understood. Gradually, the impairment of language proceeds to reduce communication to a few incomprehensible syllables. Patients lose the ability to organize words within the sentences and incur problems of understanding verbal and written language ».

As for behavioral alterations, He continues of Fede, «patients with FTD can show apathy, disinterest, emotional detachment, lack of initiative, listlessness And depression, or on the contrary to manifest hyperactivity, irritability And aggression”. They often also highlight “alterations of food behaviorwith the tendency to take food in a disordered way. With the progress of the pathology, then, problems of personal neglect And disappearance of the inhibitory brakeswhich make the behavior of the patient not adequate to the social context ».

The alterations of the motor sphere “They include greater rigidity, slowness, hindrance in the movements And Difficulty in manipulating objectsas happens in individuals suffering from Parkinson’s disease “specifies of faith. «In addition, in the forms of FTD that associate with the Amiotrophic lateral sclerosis (SLA) Symptoms such as weakness, slimming and muscle fascications, spontaneous and rapid contractions of fiber groups can present themselves ».

Frontotemporal dementia: who is striking, how many cases in Italy and the differences between men and women

In the absence of a national official register, to estimate numbers in Italy, we are based on European data. Always according to the multicenter study Frontiersconducted between 2018 and 2019 in 13 specialized clinics (including Italy), theaverage annual incidence of the FTD in Europe is approximately 2.36 cases per 100,000 people. This is equivalent to About 12,000 new cases every year in Europe.

In Italy, considering the population and the European average, it is estimated that The sick are a few thousandeven if the precise figure is not made official.

Diagnosis

Distinguish With precision between the different forms of dementia it is one of the most complex challenges for a neurologist. Always according to Dr. of faith, the diagnostic path begins with theobservation of the signs of cognitive dysfunction and continue with the aim of differentiating the various forms of dementia. In support of the diagnosis, several instrumental tests are performed, including CT and magnetic resonance imagingwhich allow you to detect any areas of atrophy in the frontal and temporal lobes. The Cerebral pet/coach with fluorodesoxyglucose (FDG)a radiopharmaceutical that allows you to identify a possible slowdown in brain metabolism in the regions affected by the disease.

Such as therapies: drugs, rehabilitation and new approaches

At the moment for the FTD There are no care real, if not the stabilization: Today there are no drugs or treatments that have shown to prevent, treat or slow down the decline of these disorders. However, therapeutic paths include:

Symptomatic pharmacological therapies

In some cases, to manage behavioral symptoms are used Off-Label drugslike:

• SSRI antidepressants And atypical antipsychoticsto deal with agitation, disinhibition and compulsive behaviors.
• Benzodiazepine (eg Alprazolam, Diazepam): to be used with extreme caution for the risk of confusion and falls.
• Levodopa/carbidopain cases with Parkinsonian symptoms (limited efficacy).
• Psychostimulants Like Metilfenida, only in cases selected due to possible side effects.

Non -pharmacological and support therapies

• Environmental rehabilitation
  Creating safe, bright and structured environments (with watches, calendars, visual signals) helps improve daily management.
• Rehabilitation therapies
  Interventions such as speech therapy, employment therapy, behavioral support and psychoeducation for caregiver are fundamental for maintaining autonomy and quality of life.
• Non -invasive cerebral stimulation (Nibs)
  Experimental techniques such as the Transcranial magnetic stimulation (TMS) and the Transcranial electrical stimulation (TES) They are designed to improve language disorders in FTD patients.

Search and emerging therapies:

Scientific research is opening up new paths to face Frontotemporal Dementia (FTD): alongside traditional symptomatic therapies, innovative approaches – pharmacological and non – – aimed are emerging to slow down the progression of the disease and improve the quality of life of patients. Some of these treatments are already in the experimentation phase Clinic, offering new hopes for families and caregiver. Among these:

• Co -Ultrapealut molecule (endocannabinoids)
  An Italian study conducted by Santa Lucia IRCCS Foundation LinkHa highlighted cognitive and behavioral improvements in patients treated with co -ultrapealut compared to placebo. The treatment has also shown positive effects on daily autonomies and language.
• Nasal oxytocin spray for apathy
  A clinical study of the Western University He found that the daily administration of oxytocin for six weeks has reduced slightly but significantly the symptoms of apathy in patients with FTD.
• Experimental genical therapy
  Are underway Research on gene therapiesespecially in the hereditary forms of FTD, but these are long -term approaches and still preliminary.

More awareness is needed

In Italy, front -time dementia is recognized within the National Fund for Alzheimer and dementiamanaged byHigher Institute of Health and from Ministry of Health. Despite this, the awareness is still low And often the diagnosis he arrives latealso because of the young age of patients and behavioral symptoms that can be misunderstood. A recent Senate bill proposed the establishment of the National day for awareness on FTD, to make the disease known and strengthen support for patients and families.