Second Round of CJD - Human Mad Cow - Disease Starts in Scotland

by Heidi Stevenson

18 December 2009

A 30 year old man, Grant Goodwin, has died of a new variant of Creutzfeldt-Jakob Disease (CJD) in Scotland. He died in January of this year. The cause of death and the genetic link have just been confirmed. The first death wave started in 1996 after officials had been telling the public that mad cow disease, which had been found to be rampant in cattle years earlier, could not be transmitted to humans. Goodwin is the first of a new wave of CJD deaths.

In an interview with BBC, Thomas Goodwin said that, "Grant was a very happy-go-lucky person. He was a bit of a jack-the-lad kind of person, lived life, was a bit of a party animal, loved the good life." He had settled in the Channel Islands with, as his father describes, "a smashing group of young chaps who we've come to know very well who came to see Grant when he was dying."

Thomas Goodwin said that his early symptoms were of a personality change, from his happy-go-lucky ways to depression and jealousy. He and his girlfriend saw a doctor, who misdiagnosed depression and prescribed medications for that.

Margaret Goodwin, Grant's mother, visited him in May 2008, and found him complaining of leg and head pain. On visiting him three months later, Thomas says that "the deterioration in three months was absolutely unbelievable. He couldn't support himself, he fell twice when he was walking and he had to be supported. My wife phoned me and told me that I must arrange a flight for Grant and get him home as soon as possible."

They thought he had a tumor. His short-term memory was so bad that, Grant's father said, "He couldn't remember what he had had for breakfast."

On meeting him at the airport on his return home, "When I saw him coming through arrivals a great knot came into my stomach. It was heart-breaking. He came through like a drunk man and the plan was for to bring him home, but when I saw him, we took him straight to hospital."

That was when the diagnosis of CJD was finally made. But he was so ill at that point that, "Grant couldn't take it in - because of his illness, five minutes after he was told, he had forgotten what he had been told."

The rest of his life, Grant "went to bed every night, expecting to die." Sadly, his sad end was determined on coming into contact with tainted beef during earliest childhood.

New Wave of CJD Deaths

A second wave of deaths among people of a genetic makeup different from the first victims had been expected for several years. Grant Goodwin has been confirmed to have had the gene.

Human susceptibility to CJD caused by prions from cattle with bovine spongiform encephalitis (BSE or mad cow disease) is related to which of three variations of a particular gene each person has. It can be coded to accept the amino acid valine alone (VV), methionine alone (MM), or both (MV).

All prior BSE-related CJD deaths have been in people with the MM variation. Goodwin had the MV type. It's believed that about 47% of the population carries the same gene, making anyone with the MV gene who ate, or possibly came into contact with, contaminated beef during the mad cow epidemic of the 1980s through early '90s a potential victim of CJD.

Professor John Collinge of the UK's National Prion Clinic, stated:

The majority of the UK population have potentially been exposed to BSE prions but the extent of clinically silent infection remains unclear.

In the first wave of UK deaths from CJD, associated with the MM gene variation, 166 people died. Scientists predict about 250 deaths from the second wave.

The truth, though, is that no one really knows. According to a January 2009 article in The Lancet:

A second wave of CJD with a longer incubation time might hit these shores, but we do not know if this will be a tidal wave or just an imperceptible ripple.

Whatever is going to happen, the first person to die in this second wave was Grant Goodwin, a young man who had just begun to settle into his life.