A doctor dismissed my husband’s dizziness as vertigo… four months later he died of mad cow disease

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

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

But it was the start of a rapid decline that soon robbed the 75-year-old of his ability to talk and walk – and led to his death just four months later.

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

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

‘He was always worried about his health so he was very active. He walked our dogs for two hours every day, he never smoked or drank alcohol. But he went downhill so quickly, it was so shocking. Within a few weeks he began to shuffle while walking, slurring his words and becoming increasingly confused.”

To many, CJD is best known as the human form of the 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 contaminated beef. Bob, a retired procurement executive, had a version called spontaneous CJD, which affects around 130 people a year in Britain and the cause is unknown.

In CJD, a common protein called a prion, which is found on the surface of brain cells and is thought to transmit messages between them, malfunctions.

This appears to trigger a cascade that causes other prion proteins to malfunction, causing brain cells to die. The disease causes holes in the brain, causing mental degeneration and eventually death. It is not known what triggers this process in spontaneous CJD, which leads to death on average four months after the onset of symptoms.

When Bob began to deteriorate, Maggie made an appointment with a private neurologist. “When we first visited in August, Bob was using a cane,” says Maggie, who was married to Bob for 47 years.

‘The neurologist said it was definitely not dizziness and arranged for an MRI scan.

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

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

After a fall at home in November, Bob went to the hospital, where a special type of MRI scan using a contrast dye showed changes in parts of his brain that indicated CJD.

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

“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,” says Maggie.

“After Bob went to the hospital, he couldn’t talk anymore,” she says. “He didn’t really indicate we were there, but he did seem to smile when his two grandchildren came in.” Bob died in hospital just before Christmas.

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

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

“The progression of spontaneous CJD can be quite baffling and surprising to families and doctors because they are not used to it,” he says. “Families tell us their relative was able to walk when they were admitted to the hospital, and when they speak to us two days later, they can’t walk or talk.”

Only two risk factors have been confirmed. One is aging, with people under 40 rarely developing the condition, and the other is a specific version of a gene carried by a third of people.

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

Research is underway in a number of promising areas that could tackle the disease, but the speed at which the disease spreads in the brain and the relatively late time at which it is diagnosed in humans are likely to complicate the trials, he adds .

‘There is nothing you can do to reduce your risk of developing spontaneous CJD,’ says Professor Knight. ‘But it is rare: one or two people in every million die from it every year in Britain.’