spongiform encephalopathy in young humans

[Ian A. York]

In article <47pvuk$ouj at ipgate.le.ac.uk>, A.J.Cann <nna at le.ac.uk> wrote:
>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?   ;-)

As you say ... of course, the question is probably important enough to
start looking very closely for warning signs.  Apparently CJD has an
incidence of approximately .5-1 new cases per million population per year
[1] - I presume this includes both familial and spontaneous cases, and the
former seem more common; but even so, two cases in Britain doesn't seem
way out of line. 

I don't know what age spontaneous CJD usually hits at.  Is there a 
preference?  Were these cases younger than the usual?  I have a vague 
memory (so typical, alas, of all my memories) that spontaneous CJD 
usually affects middle-aged and older people.

Ian

[1]  Chipps E.  Paulson G.
Creutzfeldt-Jakob disease: a review.
Journal of Neuroscience Nursing.  26(4):219-23, 1994
-- 
Ian York   (iayork at panix.com)
Dana-Farber Cancer Institute, 44 Binney St., Boston MA 02115
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