Beastie Boy John Berry died of frontal lobe dementia – but what is it?

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Jan Oyebode, University of Bradford

John Berry, a founder member of the Beastie Boys, has died of dementia at the age of 52. Berry’s father told Rolling Stone magazine that his son died from frontal lobe dementia, a rare form of dementia more properly referred to as fronto-temporal dementia.

Symptoms of fronto-temporal dementia usually begin in late middle age. Because the disease is not very well known, people with fronto-temporal dementia often have delays in receiving a diagnosis and may find the services they need are not available.

A tricky term

The terms used for this dementia are confusing. The changes in the brain are referred to as “fronto-temporal lobar degeneration”. These do not initially have any effect on people’s behaviour. Once signs and symptoms show up, it is possible to diagnose the condition as one of the “fronto-temporal dementias”.

When the frontal lobes – the parts of the brain lying immediately behind the forehead – are mainly affected, there are changes in behaviour or personality, resulting in “behavioural variant fronto-temporal dementia”. When the temporal lobes – parts of the brain near the temples – are mainly affected, dementia shows up through changes in language, of which there are two types: semantic dementia and progressive non-fluent aphasia. Whichever type of fronto-temporal dementia people have, they do not come to the doctor complaining of the sort of problems with memory loss that most of us think of as being signs of dementia.

We don’t know the specific symptoms that John Berry had as each case of fronto-temporal dementia is different, but about four to 15 people in every 100,000 have fronto-temporal dementias – and there are some common symptoms.

Losing the supervisor

There is huge variation in how fronto-temporal dementia progresses. But over time, it usually affects more and more aspects of thinking and functioning. It is a condition that shortens life, with people living about three to ten years after diagnosis.

So what happens during the earlier stages of behavioural variant fronto-temporal dementia – the type that is sited in the frontal lobes? One way of thinking of this area of the brain is to imagine it as the supervisor of complex activities and social behaviour. When the supervisor starts to do its job poorly, people develop trouble with complicated tasks. They may not be able to get started, so they may seem apathetic and lacking in energy. When they get started they may get stuck in a groove.

Fronto-temporal dementia is sometimes confused with depression.
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One carer we spoke with described how his brother would drive the car late into the night until it ran out of petrol even though he knew, on one level, that he ought to stop to fill up.

Sometimes people repeat an action over and over in exactly the same way, perhaps arranging objects very precisely or following a set daily routine according to a strict timetable. As the frontal lobe overseer loosens control, people often become uninhibited. They may become blunt and tactless. They may act on appetites and urges in ways that are quite out of character: touching people, swearing profusely when irritated and eating excessive amounts of sweet foods.

A particular difficulty for friends and family is that people with this dementia lose their ability to empathise. They may no longer offer comfort if someone is in tears and may seem very self-centred.

As not many people are familiar with the condition, it is often mistaken for other more common conditions. People may put the changes down to mid-life crisis, stress at work, depression or the menopause. It is possible that the condition is often misdiagnosed.

On average, it takes four years to diagnosis after symptom onset for younger people with dementia, twice as long as for those over 65 years of age, by which time, relationships may have broken down. People with fronto-temporal dementia are often at a stage of life where they still have children – and sometimes parents – who depend on them. So this, coupled with their increasing needs for support can be very stressful for everyone. Yet a recent national survey, currently in press, found there was a lack of provision of appropriate care across most of the country.

Biomedical research is making strides in identifying many of the proteins that accumulate as plaques in the brains of the people affected. The genetic aspects which affect about one in every five to ten cases are also now understood. However, with a cure still a long way off, research into how to support and assist people to manage their day-to-day lives is also very important. In our research we have taken detailed accounts of the experiences of those affected and we will be using these to develop and test ways of helping people and their families to manage and live better with the condition.The Conversation

Jan Oyebode, Professor of Dementia Care, University of Bradford

This article was originally published on The Conversation. Read the original article.

Frontal lobe paradox: where people have brain damage but don’t know it

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Sam Gilbert, UCL and Melanie George, Canterbury Christ Church University

Humans have big brains and our frontal lobes, just behind the forehead, are particularly huge. Injuries to this part of the brain often happen after blows to the head or a stroke. Paradoxically, some people with frontal lobe injuries can seem unaffected – until they’ve been carefully evaluated.

The frontal lobes are sometimes described as the executives of the brain, or conductors of the orchestra. Among other things, they control and organise our thinking and decision-making processes. You rely on your frontal lobes when you do things like make plans, switch from one activity to another, or resist temptation.

Some people with frontal lobe injuries seem completely normal in short one-to-one conversations, but they actually have great difficulty with everyday tasks, such as cooking, organising their paperwork or remembering to take medication. This is called the frontal lobe paradox because, even though these people seem unimpaired when assessed, they have significant difficulties in everyday life.

Without specialist expertise in acquired brain injuries, it can be almost impossible to spot frontal lobe paradox because, in many cases, people will still be able to speak normally and seem remarkably unimpaired. They may be unaware of their difficulties and deny that they need any help or support.

Insight issues

People affected by the condition are not lying when they say they don’t need help or support. Instead, they may lack knowledge of their own condition because areas of the frontal lobes that are responsible for self-monitoring and developing insight have been affected by their brain damage.

A second reason for the frontal lobe paradox is that the skills needed for an assessment interview are different from those needed in everyday life. The structure and routine of an environment, such as a rehabilitation ward, can, in effect, play the role of someone’s frontal lobes. This can mask the difficulties people experience in less structured, open-ended environments. For this reason, a person’s level of ability needs to be assessed in a situation that resembles everyday life. A seemingly simple task, such as going shopping, can reveal difficulties in people who appear unimpaired on standard tests of memory and attention, and have normal intelligence.

People with frontal lobe paradox may need help with things like cooking.
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Lack of specialist training

Neuroscientists and doctors have known about the frontal lobe paradox for at least 50 years, but it is not always understood by non-specialists. This situation can lead to people not receiving help they desperately need.

For example, in England and Wales, social workers and care managers are usually responsible for deciding whether a person has the capacity (under the Mental Capacity Act 2005) to decline support or care. These are hardworking professionals who are motivated to act in the best interests of those under their care, but many receive little or no specialist training in brain injury.

These professionals tend to base their decision about a person’s mental capacity on a short face-to-face interview. This is exactly the situation that can lead to people with frontal lobe damage being denied the care that they need.

The assessment provides the support needed for a person to sound competent and able, but only for the duration of the assessment. In one example, a woman persuaded a series of professionals that she could safely live alone after a significant brain injury. In reality, she could not make meals for herself or remember to take her lifesaving medication. Sadly, she died at home shortly afterwards.

Support needed

We don’t know exactly how common the condition is, but the frontal lobe paradox is probably found in a much higher number of people than you might first imagine. As well as those who have suffered blows to the head and strokes, it can affect people with certain infections, some forms of dementia and even poorly controlled diabetes.

It is vital that social workers and care managers are trained on brain injury to protect the interests of people with frontal lobe injuries. People with these injuries are in particular need of support, but they are often the least likely to receive it.The Conversation

Sam Gilbert, Associate Professor, Institute of Cognitive Neuroscience, UCL and Melanie George, Consultant Clinical Neuropsychologist, Canterbury Christ Church University

This article was originally published on The Conversation. Read the original article.