The idea that the new human spongiform disease was a 'new variant of CJD' was rejected in BSE: The Facts and the controversy was widely reported in the press at the time. Symptomatic comparison seemed to associate the new human diseases more closely with kuru, of Papua New Guinea. At the time the proposal was discounted by the authorities, but it has been announced in April, 1997 that research by the MRC Prion Unit is now going to embrace studies in Papua New Guinea to see what correlations might, after all, exist. Here, a submission to the Lancet from mid-1996 is released for the first time:
The CJD surveillance programme reported in your columns(1) has revealed cases of an atypical spongiform encephalopathy in young adults. It is widely attributed as a new variant of Creutzfeldt-Jakob disease. However, many of the signs and symptoms of CJD are absent, or at least highly modified, in these new cases. The symptomatic basis for considering these new cases of human spongiform encephalopathy (HSE) to be a strain of CJD is weak.
The suggestion has been published(2) that the new syndrome is similar to kuru, the epidemic spongiform disease found in the Fore people of the Eastern Highlands in Papua New Guinea. The research workers who have correlated data from the first British patients(3) have even described the histological changes as featuring kuru-type plaques.
Tabular presentation of some of the diagnostic characteristics suggest that the new syndrome has more in common with kuru than with CJD:
|Age||> 50||< 50||< 50|
|Duration||< 1 year||> 1 year||> 1 year|
This may have practical implications. The clinical picture of kuru was first documented four decades ago(4) and more is known of its management than that of a truly novel condition. Since the symptomatology seems to reveal similarities between the new HSE and kuru, I believe it would be realistic to abandon the notion that the syndrome is a variant of CJD and consider it as a strain of kuru.
1 Collinge J, Rossor M, A new variant of prion disease, Lancet 1996; 347: 916-917.
2 Ford BJ, BSE: The Facts, London: Corgi, 208pp, 1996.
3 Will RG, et al, A new variant of Creutzfeldt-Jakob disease in the UK, Lancet 1996, 347: 921-925.
4 Gajdusek DC, Degenerative disease of the nervous system in New Guinea, the endemic occurrence of kuru in the native population, N Engl J Med 1957; 257: 974-78.
Book details: BSE - The Facts, 208pp, ISBN 0 552 14530 0, £4.99, London: Corgi Books, 1996.