There is one other human disease which closely resembles CJD:

Kuru was the first human spongiform encephalopathy to be investigated in detail and is one of the most fascinating and horrifying stories to have emerged from any epidemiological investigation. This disease occurred in 169 villages occupied by the Fore tribes in the highlands of New Guinea.

The first cases were recorded in the 1950s and involved symptoms very similar to those of CJD. The Fore people practised ritual cannibalism as a rite of mourning for their dead. Women and children participated in these ceremonies but adult men did not take part, explaining the age/sex distribution of kuru - it was never seen in very young children, rarely in adult men and was most common in adolescents and in adult women. This was the first clue that kuru was not caused by a conventional infectious agent, e.g. a virus. The incubation period for kuru can be longer than thirty years, but is usually shorter than this, e.g. fifteen years. Ritual cannibalism was stopped in the late 1950s and the occurrence of kuru has declined dramatically over the last few decades. Kuru is now very rare and only occurs in those who participated in cannibalistic feasts over thirty years ago.


© AJC 1997.