A Very Rare Form of Dementia
Kuru dementia is an extremely rare form of dementia restricted to the highlands of New Guinea. Kuru that means trembling with fear, affected a tribe of people called the Fore, pronounced for-ay.
Kuru is a prion disease very like scrapie in sheep, BSE mad cow disease and its human form vCJD (variant Creutzfeldt-Jakob disease). Kuru was the first transmissible spongiform encephalopathy (prion disease) discovered in humans.
Kuru is a disease passed on during funeral rites of the Fore tribe when the body of the dead was ritually cooked and eaten. The brain, the most infected part of the body of someone with Kuru was eaten by the closest females relatives of the departed. The women and children scooped out the brain from the skull and would not wash their hands for many weeks after the funeral. The disease was probably also passed onto many others in the tribe because of this practice.
Kuru dementia is now almost non existent for two main reasons. The disease wiped out many tribes who practiced this ritual and Government in New Guinea discouraged cannibalism. It is believed the disease existed for only a short time as elders could not remember it when they were young.
Signs of Kuru, like variant CJD is believed to have a long incubation period of many years. Signs and symptoms include profound neurological deterioration that manifests itself with disordered movement, tremors, unsteadiness, mood disorder and in some dementia. Death followed a comatose state within 6 to 12 months after the first appearance of symptoms.
There is no treatment for Kuru.
Kuru: The Dynamics of a Prion Disease
Kuru Infoprmation Page National Institute of Neurological Disorders and Stroke.
Robert G Will, (2003) Acquired prion disease: iatrogenic CJD, variant CJD, kuru National CJD Surveillance Unit, Department of Clinical Neuroscience, Western General Hospital, Edinburgh, UK Oxford Journals Medicine British Medical Bulletin Volume 66, Number 1 Pp. 255-265.
More Information About Variant Creutzfeldt-Jakob Disease