What is frontotemporal dementia, the brain disorder Bruce Willis has been diagnosed with?

“Today there are no treatments for the disease, a reality that we hope can change in the years ahead. As Bruce’s condition advances, we hope that any media attention can be focused on shining a light on this disease that needs far more awareness and research,” the family said, expressing their gratitude for “continued compassion, understanding, and respect” shown by the fans.

 

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What is frontotemporal dementia?

It is a group of heterogeneous neurodegenerative disorders characterised by prominent changes in social behaviour and personality or aphasia (language difficulties) accompanied by degeneration of the frontal and/or temporal lobes. “The term ‘FTD’ serves as an umbrella term for three clinical presentations: behavioural variant FTD (bvFTD) and two forms of a primary progressive aphasia (PPA), the nonfluent and semantic variants. Some patients with FTD also develop a concomitant motor syndrome such as parkinsonism or motor neuron disease (MND),” according to Dr Mohan Krishna J, Consultant Neurologist, Yashoda Hospitals, Hyderabad.

Dr Pavan Pai, Consultant Interventional Neurologist, Wockhardt Hospitals, Mira Road, Mumbai added that those with frontotemporal dementia will notice that his/her portions of their lobes tend to shrink. “Those people detected with this condition will become aggressive, impulsive, and emotional. However, there is significantly a low awareness of this condition. This condition can be often misdiagnosed and even go unreported. It is the need of the hour to know about its symptoms and seek timely intervention,” he said.

Symptoms

Dr Mohan shared the following common symptoms associated with this disorder.

*Disinhibition – Socially inappropriate behaviours include touching or kissing strangers, public urination, flatulence without concern, making offensive remarks or invading others’ personal space, and utilisation behaviours, like tampering with nearby objects or stealing people’s possessions.

*Apathy and loss of empathy – Apathy shows up as a loss of interest in and/or drive for social interactions and activities.

 

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“Most patients are unaware of the distress their families are going through as a result of their behavioural changes. They are mistaken frequently for depression, and patients are often referred for psychiatric treatment early in the disease course,” he said.

Dr Pai added that the symptoms of this condition need to be identified at the right time as they can worsen over time. “Do not delay the treatment once you notice the symptoms.”

What are the causes?

Experts say that genetic mutations and family history can be major causes behind the occurrence of this condition. “30 to 50 per cent of patients with FTD have at least one relative with dementia, particularly when associated with motor neuron disease,” Dr Mohan shared.

He added: “Microtubule-associated protein tau (MAPT), granulin precursor (GRN), and a hexanucleotide expansion in chromosome 9 open reading frame 72 are the genes most frequently mutated to cause disease (C9orf72).”

Prevention and treatment

Dr Pai said that those with frontotemporal dementia must do physical exercise, solve puzzles, and get a good night’s sleep on a daily basis, apart from staying in regular touch with their concerned doctors.

Sharing that “frontotemporal dementia is currently without viable disease-modifying therapies”, Dr Mohan listed the following treatment methods for this condition:

*Drug and non-drug therapies are both intended to reduce symptoms, especially the behavioural signs of FTD.
*Drug side effects can include paradoxical behavioural responses, extrapyramidal side effects, disorientation, and drowsiness in patients with FTD.
*It is advised to begin treatment with the lowest effective dose, gradually increase the dosage, and monitor side effects often.

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