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I read the article by Murray concerning sporadic Creutzfeldt-Jakob disease (CJD) in adolescents with interest (1). Seven cases of sporadic CJD in adolescents in all are described (1) without any history of potential iatrogenic exposure.
Although an infectious protein or prion is considered to be the cause of the transmissible spongiform encephalopaties such as CJD, bovine spongiform encephal...
Although an infectious protein or prion is considered to be the cause of the transmissible spongiform encephalopaties such as CJD, bovine spongiform encephalopathy and Kuru, the occurrence of sporadic CJD in
adolescents without any preceding known exposure to potentially infectious material, would raise the question of a different etiology such as a slow virus.
Many slow viruses have the ability to survive adverse environments, evade host immunity and persist for years, which are characteristic of transmissible spongiform encephalopathy agents (2)
The negative immunocytochemistry prion protein examination of the brain biopsy specimen in UK case No. 2 (1) would make a prion protein etiology less likely as the cause of sporadic CJD in adolescents, at least in the examined case.
Virus like 25-nm diameter particles have been found repeatedly in highly infectious brain fractions with little prion protein (3).
No form of prion protein has fulfilled Koch’s postulates for infection, pathologic prion protein not being proportional to or necessary for infection and recombinant prions failing to produce significant infectivity (2).
Electron microscopic information has revealed spherical 25-35 nm particles in infectious material (2) which are large enough to contain a viral genome of 1-4 kb, which could encode a protective nucleocapsid and enzyme for replication (2).
Host prion protein may act as a cellular facilitator for infectious particles, and develop into pathological amyloid (2).
An infectious slow virus seems a more likely cause for sporadic CJD development in adolescents than infectious prion protein, the virus perhaps causing production of pathological amyloid or prion protein with consequent neuronal destruction and catastrophic brain deterioration.
Steven R Brenner
1. Murray K, Ritchie DL, Bruce M, et al. Sportadic Creutzfeldt-Jakob disease in two adolescents. J Neurol Neurosurg Psychiatry 2008; 79: 14-18.
2. Manuelidis, L. A 25 nm virion is the likely cause of transmissible spongiform
Encephalopaties. J Cell Biochem. 2007; 100: 897-915.
3. Manuelidis L, Yu ZX, Barquero N, Mullins B. Cells infected with scrapie and Creutzfeldt-Jakob disease agents produce intracellular 25-nm virus-like particles.
Proc Natl Acad Sci USA. 2007 104: 1965-1970 Epub 2007 2007 Jan 31.
No competing interests