New Victims: One new case v-CJD per week
Who gets v-CJD?
Can humans get scrapie from eating sheep?
How common is scrapie in UK?
Another 155 cows slaughtered in France

New Victims: Rate 1 new case v-CJD per week now

Adapted from a Correspondence
17 July 1996

The info about the 23 cases of v-CJD [or CJD2] came from Prof. Dealler's rumours/gossip section. Note he is sponsoring a free Internet BSE journal and conference.

The information is probably high grade info but there is no backup info. He may of heard it from Prof. L:acey who hears it from clinicians.

The additional cases are practically certainly new cases with the appropriate symptoms who are still alive, when they die they perform an autopsy and they go into the official count if CJD2 is confirmed. The rate of the new variant of CJD looks like steady at about 1 case per week.


Who can get v-CJD?

Lancet. May 11, 1996, vol 347 p 1332/3
by Diringer H., Koch-Institut, Nordufer 20, D 13353, Berlin, Germany
[The human prion gene is polymorphic at position 129. That is, both methionine and valine can occur seemingly normally. From looking at other species, methionine is probably the historic amino acid; for some reason valine has become fairly prevalent in humans. It is not known whether this results from selective pressure or just genetic drift.

since there ios a copy of this autosomal gene on both chromosomes, there are three genetic possibilities: met/met, met/val/, and val/val. The new variant CJD has been found to occur only in the met/met homozygotes, who consitute 48% of the English population. Sporadic CJd, non-familial but otherwise mysterious, is also enhanced in met/met homozygotes. -- webmaster]

Per Cent of investigated cases

met/met met/val val/val # examined:
Normal population 48 42 10 1397
Sporadic CJD 78 12 10 73
CJD2 100 0 0 8

Can humans get scrapie from eating sheep?

Adapted from Listserve item ... 17 July 1996

"In Italy, people commonly eat sheep"s brain. Lo-Russo et al. (1980) point out that the distribution of CJD corresponds with that of sheep-keeping in Central and South Italy. As 6 of the 8 cases are women, they suspect that the agent passes over during the process of preparing food, more than by eating.

In France, where brains are also very popular, the first case of V-CJD out of GB was duly confirmed. Besides, the CJD correlates with the population density. Perhaps in big towns such delicacies are eaten preferably?

In Slovaky, in a sheep area, the accumulation of CJD is particularly striking (Mayer, 1977). The authors report that scrapie wasn't noticed there, but perhaps scrapie is just a well kept secret there as in GB since the 19th century; the reports on scrapie are contradictory.

In GB, scrapie appeared in the 18th century for the first time, first in South England (Stamp, 1958) and spread afterwards. It would be interesting to know if it happened to be Kent where the first cases of BSE and after that the clusters of CJD were found?

As far as the Libyan Jews are concerned, they are a small community. Possibly, they make use of their own breeding of sheep with scrapie-infected sheep. Perhaps other ethnic groups with equal eating habits have got scrapie-free sheep? [CJD here has been attributed to a codon 200 mutation -- webmaster]

Whoever states the genetic constellation as proof to the contrary should also explain why frequently sections of the population involved in sheep breeding and eating are affected by CJD. I think the genetic peculiarities deliver the disposition, but they aren't the cause.

 
References
 
Lo-Russo, F., Neri, G., Figa-Talamanca, L.: Creutzfeldt-Jakob disease and
sheep brain. A report from Central an Southern Italy. Italian J. Neurol.
Sciences 1980, 1(3), p. 171
 
Mayer, V., Orolin, D., Mitrova, E.: Cluster of Creutzfeldt-Jakob disease and
presenile Dementia. Lancet, July 30, 1977
 
J. T. Stamp: Scrapie Disease of Sheep. Vet. Rec., January 18th, 1958

UK Sheep with Scrapie

Year Number sheep
1978-80 30,388
1984 34,802
1985 35,628
1986 37,016
1987 38,701
1988 40,942
1989 42,885
9 years 260,202

Another herd slaughtered in France

11 July 96 ... AP A herd of 155 cows in the Cantal region of central France was slaughtered yesterday after one was found to have BSE. The animal was born in 1991, after the ban on livestock feed imported from Britain came into effect. It was the ninth case in France this year and the 22nd since 1990, officials said.