Wednesday 17 January 2018

Form of dementia suffered by Monty Python star Terry Jones is often missed by doctors

Terry Jones has been diagnosed with dementia
Terry Jones has been diagnosed with dementia

The news that Terry Jones has dementia is very sad. He, along with the other Pythons, has become a household name - at least for those of us over the age of 50.

His part as the mother of Brian in Life of Brian will be amusingly remembered. Now the news that he cannot speak any more must be heartbreaking and frustrating for a man who lived by the spoken word, by quips and by wit. And now he is silent.

The news of this broke when his family arranged an interview to publicise the condition of frontotemporal dementia (FTD) from which he suffers, and about which the public know very little. His long-standing buddy Michael Palin was also interviewed.

Most people associate dementia with Alzheimer's disease and while it is the most common, FTD is often a missed diagnosis as it is not thought about.

Terry Jones, Eric Idle, Michael Palin, Terry Gilliam and John Cleese of Monty Python
Terry Jones, Eric Idle, Michael Palin, Terry Gilliam and John Cleese of Monty Python

In the US, about 60,000 are diagnosed with it every year. It is a condition that effects a somewhat younger age group than Alzheimer's, most commonly in their Fifties and Sixties. FTD is an umbrella term for those who lose the functioning in the frontal and/or temporal lobes of the brain, and the specific symptoms can vary with the exact part that is affected. These particular areas of the brain are large, located in the front and side of that organ, and are concerned with behaviour, language and personality. There is shrinkage of the lobes due to damage to certain groups of neurones.

Previously it was called Pick's disease, after Arnold Pick, a psychiatrist from Prague who discovered it in 1892 when he retrospectively examined the brain tissue of several deceased patients who had dementia. He identified a protein mass in the neurones which have become known as Pick bodies and characterise the disease when examined under a microscope.

Now, Pick's disease is used to depict a specific constellation of symptoms in the broader frontotemporal group.

Some people with FTD undergo dramatic changes in their personality and become socially inappropriate, impulsive or emotionally indifferent, while others lose the ability to use language, as Jones has. Some cannot find words for common objects, referring to "it" or "the thing" while others speak in a robotic, telegrammatic style.

Those who were once kind and loving become indifferent to the feelings of their loved ones. One of the FTD websites describes a man with the condition who telephoned his wife while in hospital undergoing chemotherapy and demanded that she come hope to cook for him. He had previously been a most caring, gentle and considerate man. People who abhorred coarse language may suddenly start telling risqué jokes or dressing inappropriately. Compulsive buying (oniomania) is another feature as is restlessness and lack of motivation. Compulsive overeating occurs in the later stages.

While sufferers experience difficulties performing complex behavioural tasks, memory for past events, known as episodic memory, is generally maintained at least until the late stages of the disease. So, unlike Alzheimer's disease, recognition of people is maintained in FTD.

When it presents initially, the changes are often mistaken for psychiatric illness such as hypomania (because of the disinhibition and compulsive buying), schizophrenia (because of the change in how language is used), or depressive illness (because of agitation and poor motivation). There may be a genetic component, although there is debate about this and it applies to all the subtypes.

What is sadly lacking are specific treatments - and some of the memory-enhancing agents used to help those with Alzheimer's dementia simply do not work in frontotemporal dementia as these target neurochemicals not affected in the latter.

SSRI antidepressants are used to treat the impulsivity, aggression and overeating. Practical measures to ensure that the home is made safe so as to minimise impulsive wandering and reduce the risk of dangerous behaviour is a priority.

It goes without saying that driving is unsafe. Antipsychotic agents, sometimes used to reduce the risk of wandering in elderly people, especially in nursing homes, should not be used as they may worsen the tremor and stiffness that some experience.

Jones has brought much joy to millions of people and his illness is a huge burden for him and for his family to bear. He and his family should be in our thoughts and in our prayers. Yet he has not lost his wit despite his suffering. He showed remarkable humour and incisiveness when first diagnosed, and he'd tell people: "I've got dementia. My frontal lobe has absconded."

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