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From: TSS ()
Subject: GRIEF OF WIDOW WHOSE HUSBAND DIED OF CJD
Date: November 28, 2005 at 7:22 am PST

CJD WATCH MESSAGE BOARD
TSS
GRIEF OF WIDOW WHOSE HUSBAND DIED OF CJD
Mon Nov 28, 2005 09:28
68.238.109.15


GRIEF OF WIDOW WHOSE HUSBAND DIED OF CJD

16:05 - 24 November 2005

Redruth woman hopes death could aid research into cure Jonathan Carter reports

A Redruth widow told this week how she hoped the death of her husband from CJD could help scientists find a cure for the devastating disease.

Lynda Anderson said that she watched helplessly as her once-fit husband, Andy, succumbed to a disorder of the central nervous system that kills just 50 people in the UK each year.

"Andy was 6ft 2ins but when he died he only weighed three stone," she said.

"I saw my husband waste away before my eyes and there was nothing I could do."

Her nightmare began in April when Mr Anderson, an information technology tutor at Cornwall College, was referred to hospital by his GP suffering from breathing problems.

Only six months earlier, he had looked a picture of health as the couple enjoyed a Mediterranean cruise to celebrate his 60th birthday.

Mrs Anderson, aged 56, said: "Between April and June, Treliske Hospital did not know what was wrong with him.

"He was allowed home but was re-admitted 10 days later.

"His short-term memory went but he recognised photographs of his grandchildren, Lee, Chloe and Laura.

"His co-ordination had gone and he was unable to lift his head.

"He would play with a children's ball, a Rubic cube and a pack of cards for hours.

"At night, he would rip up a duvet cover into strips exactly the same size."

At the beginning of September, doctors told Mrs Anderson that her husband had fallen victim to sporadic Creutzfeldt-Jakob disease, the cause of which is unknown and for which there is no cure.

She said: "A week before he died, two doctors from the National CJD Surveillance Unit in Edinburgh came to the hospital.

"They wanted to know his background from birth, his eating and drinking habits, whether he was a gardener and even what washing powder he used.

"Until I was told that he had only two weeks to live, I thought he would get better.

"I think that Andy is the only person in Cornwall to have died from this disease.

"The last time he spoke, he looked at me and said: 'Lynda, I've always loved you'.

"I thought, I've got such a happy marriage and it's being taken away.

"He never spoke again. He was such a caring man. Why this disease?"

Following his funeral at Penmount, Truro, Mr Anderson's ashes were scattered in the shadow of a magnolia tree on their wedding anniversary, November 3.

Mrs Anderson, of Park Road, Redruth, added: "I decided that I wanted to donate his tissue and blood to help with research into finding a cure for CJD.

"Andy was the father of three girls, Sharon, Mandy and Marie.

"Two days ago, I was told that the CJD he suffered from was not a genetic strain, which was a great relief."

Earlier this month, the computer suite at All Saints' Community Centre, Tuckingmill, was named in memory of Mr Anderson.

Treve James, the centre's co-ordinator, said: "Andy was a very special volunteer who always gave his best for others."


http://www.thisiscornwall.co.uk/displayNode.jsp?nodeId=144290&command=displayContent&sourceNode=144118&home=yes&contentPK=13558216&localNewsNodeId=146877


sporadic CJD or nvCJD $$$


Coexistence of multiple PrPSc types in individuals with

Creutzfeldt-Jakob disease

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

Summary

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

classifications.


snip...


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?


http://neurology.thelancet.com Published online October 31, 2005

TSS



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