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From: TSS ()
Subject: Our dad's CJD hell Dec 7 2005
Date: December 7, 2005 at 6:36 am PST

Our dad's CJD hell Dec 7 2005
Wed Dec 7, 2005 08:43

North Wales

Our dad's CJD hell Dec 7 2005

By David Greenwood, Daily Post

DEVASTATED relatives last night told how a North Wales dad-of-five was struck down by the deadly brain disease CJD.

Tests have been carried out on grandfather Thomas Llewelyn Jones, who died at Ysbyty Gwynedd, Bangor.

They will establish whether he died of new variant CJD, which is linked to eating BSE-infected meat, or the more common sporadic type.

His family last night told how Mr Jones loved eating steaks, beefburgers and sausages.

The 56-year-old joiner, of Dwyran, Anglesey, was a keen sea angler who cared for his 90-year- old widowed mother before he was taken ill seven months ago.

His 57-year-old brother John, who lives next door, last night said: "Thomas started with headaches and couldn't sleep. It went on for months.

"In the beginning doctors didn't have a clue what it was. They thought it was lack of sleep or depression.

"It wasn't that at all. In the end it was all down to the illness."

He said Mr Jones had difficulty putting sentences together, but did manage to tell him he knew what he wanted to say, but the words got jumbled up.

"He became confused and on a couple of occasions he walked out of the hospital.

"At one time two nurses followed him and managed to help him back to the ward.They were brilliant and I would like to thank them for what they did for Thomas. They didn't know what was wrong with him at that time.

"When he was admitted to hospital they carried all sorts of tests." The hospital could not pinpoint the problem, so they called in experts from the Creutzfeldt-Jakob disease unit in Edinburgh who, after carrying out their own tests, took a fluid sample from the bottom of Mr Jones' spine.

"Those samples were analysed and showed he had CJD. That was about four weeks ago and now he is dead. We just can't come to terms with it. His condition deteriorated very quickly," said John.

"The doctors don't really know what brought on the CJD."

He added: "The whole family and his friends are just devastated. Thomas was a wonderful man.

He enjoyed the outdoor life and would help anybody.

"His hobby was angling and he was a member of the Brynsiencyn Sea Angling Club. We would often go fishing in my boat along the Menai Strait."

John's wife Megan, 54, added: "It was terrible to see what happened to Thomas. It was awful. Before he died he was in hospital for six weeks.

"He couldn't speak and he didn't know us. You could see he was dying a little bit every day. It happened very quickly. We will have to wait for six weeks for the result of the postmortem and we have been told an inquest will be held sometime next year."

Thomas, who was born in the village, cared for his mother Margaret, of Lime Grove.

She said: "He was a loving son. He would go out of his way to help people. Nobody ever had a bad word about him."

His eldest daughter, 27-year-old Linda Jones who lives in Brynsciencyn, said: "We just don't know what kind of CJD it was. We will have to wait for the results and we are not expecting to hear anything until the New Year."

And in a moving tribute his 25-year-old mechanic son, John, added: "He never did anything wrong to anybody. Whatever you wanted he would do it for you."

Mr Jones' funeral took place at the weekend. Donations in lieu of flowers can be made to Tegid Ward, Ysbyty Gwynedd and to the CJD Research Fund.

A spokeswoman for district coroner Dewi Pritchard-Jones last night said Mr Jones' death was suspected CJD but they were awaiting the results of the post-mortem examination.

"That could take between five or six weeks and an inquest will not be held into the circumstances of Mr Jones' death until sometime in the New Year," she added.

CJD... the four types identified

GENETIC CJD A very rare illness caused by an abnormal gene, therefore not "caught" in any way. No causal relationship with BSE.

IATROGENIC CJD Also very rare, it is accidentally transmitted during medical or surgical procedures. The most important example in the UK relates to CJD transmitted via human growth hormone treatment in childhood.

SPORADIC CJD Numerically the most common, it has been found in every country where it has been looked for. Around 50 to 60 deaths per year are due to sporadic CJD in UK. The cause remains uncertain.

VARIANT CJD First reported in 1996, and confined to the UK apart from six cases in France, one in Ireland, one in Italy and one in the USA. It is believed to transmitted from BSE in cattle to humans via meat.

Death toll rises

Since 1990 a total of 2,022 referrals have been made to the CJD surveillance unit in Edinburgh, and 1,084 deaths have been recorded up to November 5 this year Published online October 31, 2005

Coexistence of multiple PrPSc types in individuals with

Creutzfeldt-Jakob disease

Magdalini Polymenidou, Katharina Stoeck, Markus Glatzel, Martin Vey, Anne Bellon, and Adriano Aguzzi


Background The molecular typing of sporadic Creutzfeldt-Jakob disease (CJD) is based on the size and glycoform

ratio of protease-resistant prion protein (PrPSc), and on PRNP haplotype. On digestion with proteinase K, type 1 and

type 2 PrPSc display unglycosylated core fragments of 21 kDa and 19 kDa, resulting from cleavage around amino

acids 82 and 97, respectively.

Methods We generated anti-PrP monoclonal antibodies to epitopes immediately preceding the differential proteinase

K cleavage sites. These antibodies, which were designated POM2 and POM12, recognise type 1, but not type 2, PrPSc.

Findings We studied 114 brain samples from 70 patients with sporadic CJD and three patients with variant CJD.

Every patient classified as CJD type 2, and all variant CJD patients, showed POM2/POM12 reactivity in the

cerebellum and other PrPSc-rich brain areas, with a typical PrPSc type 1 migration pattern.

Interpretation The regular coexistence of multiple PrPSc types in patients with CJD casts doubts on the validity of

electrophoretic PrPSc mobilities as surrogates for prion strains, and questions the rational basis of current CJD



The above results set the existing CJD classifications

into debate and introduce interesting questions about

human CJD types. For example, do human prion types

exist in a dynamic equilibrium in the brains of affected

individuals? Do they coexist in most or even all CJD

cases? Is the biochemically identified PrPSc type simply

the dominant type, and not the only PrPSc species? Published online October 31, 2005

what i been saying for years, that the diagnostic criteria differentiating between the nvCJD (i.e. 'the chosen ones') and the sCJD (i.e. 'the forgotten ones') has been terribly flawed from the beginning. .... full text html at bottom of this email, if you want the full text pdf with all the bells and whistles, please email me and i will send pdf....tss

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