Bob Deller was a fit and healthy grand­father, who spent his time gardening, playing golf and going to yoga when he developed a nagging headache and started feeling dizzy last summer.

His GP initially told him it was vertigo, a common condition that affects balance and generally improves on its own.

But it was the beginning of a rapid deterioration that swiftly robbed the 75-year-old of his ability to talk and walk – and led to his death only four months later.

Shortly before Bob died in December, doctors discovered the keen drummer had the ‘one-in-a-million’ fatal brain condition Creutzfeldt-Jakob disease (CJD).

‘When he first complained of headaches and dizziness, I wasn’t too worried as the GP said it would improve,’ says his widow Maggie, 75, a retired nurse who lives near Sevenoaks, Kent.

Bob Deller, pictured, was a healthy grandfather who spent his time playing golf, gardening and going to yoga before he started feeling dizzy last summer

‘He had always worried about his health, so was very active. He walked our dogs for two hours every day, he never smoked or drank alcohol. But he went downhill so fast, it was so shocking. Within a few weeks he started shuffling when he walked, slurring his words and became more and more confused.’

For many, CJD is best known as the human form of so-called ‘mad cow’ disease which came to prominence in the 1990s.

But the neurological disease comes in four types, only one of which – called variant CJD – is associated with eating infected beef. Bob, a retired procurement officer, had a version called spontaneous CJD, which strikes about 130 people a year in the UK and whose cause is unknown. 

In CJD, a common protein called a prion, which sits on the surface of brain cells and is believed to carry messages between them, malfunctions.

This seems to start a cascade that makes other prion proteins malfunction, leading to brain cells dying. The disease causes holes to develop in the brain, which triggers mental degeneration and, ultimately, death. It is not known what prompts this process to start in spontaneous CJD, which on average leads to death four months after symptoms start.

A blood sample for Creutzfeldt-Jakob disease. There are no treatment options for the illness

When Bob started to deteriorate, Maggie made an appointment with a private neurologist. ‘When we first went to see him in August, Bob was using a walking stick,’ says Maggie, who was married to Bob for 47 years.

‘The neurologist said it definitely wasn’t vertigo and arranged an MRI scan.

‘When we went to get the results a month later, Bob was in a wheelchair. You could see him getting worse almost day by day.’

Along with increasing confusion, Bob’s arms and legs started to jerk violently. Doctors ran through many scans and tests trying to find out what was behind the rapid decline of Bob, who was stepfather to Maggie’s son Kevin. Alzheimer’s, ­Parkinson’s disease and a brain tumour were all ruled out.

After a fall at home in November, Bob went into hospital where a special type of MRI scan with a contrast dye showed up changes in parts of his brain that suggested CJD.

Experts at the National CJD Research and Surveillance Unit at the University of Edinburgh tested fluid drawn from his spine to confirm he had the condition in November.

‘We spoke to a specialist before the spinal fluid test and he told us that if it was CJD he would be dead within a month, which was obviously shocking for me and Kevin to hear,’ Maggie says.

‘After Bob went into hospital, he couldn’t speak any more,’ she says. ‘He didn’t really give any signs that he was aware we were there, but he did seem to smile when his two grandchildren came in to see him.’ Bob died in hospital just before Christmas.

‘He was just so unlucky to get such a rare disease,’ Maggie says. ‘Doctors at the hospital had never even seen it before. We miss him so much, he was always keen to know what the grandchildren were up to and quick-witted and funny. He loved jazz and soul and it was always on in the background, the house is quiet without his music.’

There are no treatment options for CJD, which can advance with shocking swiftness, says Richard Knight, professor of clinical neurology at the National CJD Research and Surveillance Unit.

‘The progression of spontaneous CJD can be quite staggering and surprising to families and to doctors, as they are not used to it,’ he says. ‘Families tell us their relative could walk when they were admitted to hospital, and when they speak to us two days later, they can’t walk or speak.’

There are only two risk factors that have been confirmed. One is getting older, with people under 40 rarely developing the condition, and the other is a particular version of a gene carried by a third of the

UK population, Professor Knight says. Rates are increasing slowly around the world but this is most likely due to improvements in diagnosis, he says.

Research is looking at some promising areas that could tackle the disease, but the speed of its advance through the brain, and the relatively late point that people are diagnosed, is likely to make trials difficult, he adds.

‘There is nothing you can do to reduce your risk of developing spontaneous CJD,’ Professor Knight says. ‘But it is rare, with one or two per million people dying of it each year in the UK.’