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10.4 BSE and CJD, or Kuru?



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:

Category .............. CJD ........... HSE ......... Kuru
Amyloid plaques .. Rare ...........Common .. Common
EEC spike ............ Present ...... Absent ..... Absent
Age ....................... >50 ........... Presentation ....... Dementia .. Ataxia ...... Ataxia
Duration .............. 1yr ......... >1yr
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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.

Brian J Ford

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