spongiform encephalopathy in young humans

[Vasconcelos Costa 385]

> From: "A.J.Cann" <nna at le.ac.uk>
> To: "bionet.virology mail newsgroup" <bionet-news at dl.ac.uk>
> 
> mremingt at UMABNET.AB.UMD.EDU ("Mary P. Remington") wrote:
> >Two "Letters to the Editor" appeared in the Oct. 28, 1995 Lancet.  CJD 
> >was histologically confirmed in an 18 yr.old male in the UK without 
> >family history of CJD.  A 16 yr.old girlwas also confirmed histologically 
> >to have CJD.
> 
> Hmm... I wonder if these numbers are statistically significant?   ;-)
> 
> Dr Alan J. Cann  PhD,   Department of Microbiology & Immunology,
> University of Leicester,  P.O. Box 138,  Medical Sciences Building,
> University Road, Leicester LE1 9HN,  UK.
> Email: nna at le.ac.uk     http://www-micro.msb.le.ac.uk/AJC/nna.html
> 
> 
The incidence of CJD is around 1-2 cases/year/million. But the unusual coin-
cidence here is of two cases in very young people. CJD is typically a disease
of people between 50 and 70 years old. Does someone know what is the proba-
bility of the disease afecting people younger than 20?
I think it would be very interesting to get and sequence a PCR product of
the PRN gene from those unfortunate young people. Maybe the lesion affects
a different residue than those frequently found mutated in the classical
adult disease. I remeber that a similar disease, Gerstmann-Straussler syndrome,
has an earlier age incidence than CJD and shows some differences in the fre-
quency of mutations.

Joao V. Costa

               ---------------------------------------------------------------
I  GGG   CCC    Dr. JOAO V. COSTA                     e-mail:
I G     C       Laboratorio de Virologia II              jcosta at gulbenkian.pt
I G  GG C       Instituto Gulbenkian de Ciencia       phone: +351 (1) 4435750
I G   G C       Apartado 14, R. Quinta Grande, 6      fax:   +351 (1) 4435625
I  GGG   CCC    P-2781 OEIRAS Codex, PORTUGAL
               ---------------------------------------------------------------