Kuru (disease)

Classification according to ICD-10
A81.8	Kuru
ICD-10 online (WHO version 2019)

In Kuru is a prion disease , which in the 20th century epidemic among the people of the Fore in Papua New Guinea occurred and to a lesser extent in some neighboring nations. The word Kuru comes from the language of the local people and means muscle tremors .

Signs of illness

The disease manifests itself primarily in movement disorders and typically leads to death within 6 to 12 months of the onset of symptoms . Specifically, it concerns with the symptoms for both leading and uncertainties in terms of cerebellar ataxia , a rhythmic tremor and unnatural later in laughter, so the disease also Lach disease is called.

discovery

After the highlands of Papua New Guinea did not have any contact with western civilization until the 1930s , the disease was first described and studied in the second half of the 1950s. DC Gajdusek made a particular contribution , who was able to demonstrate, among other things, the transmission of kuru to monkeys in experiments, for which he was awarded the Nobel Prize for Medicine in 1976. A genetic cause was initially assumed for the disease, which at that time claimed more than 200 victims annually among the 10,000 fore . After the genetic hypothesis of epidemiological reasons, had become increasingly unlikely remained an intensive search for environmental toxins or infection sources also unsuccessful. Only after William J. Hadlow (1921–2015) had recognized the (neuropathological) similarity to scrapie , which was already known at the time as transferable , was the transferability of the kuru to monkeys investigated under a long observation period and was successful in the 1960s. After further decades of medical, epidemiological and anthropological research, the hypothesis was established that Kuru was transmitted among the Fore through endocannibalism (consumption of meat from deceased tribal members) and the associated handling of highly infectious brains. Since cannibalism was banned in 1954 for other, non-medical reasons, the incidence of the disease also steadily decreased until the disease completely disappeared towards the end of the century.

In retrospect, the beginning of the epidemic at the turn of the 19th to the 20th century was localized and presumably assumed a single (sporadic) case. Women and children who became infected parenterally when dealing with the infectious brain probably fell ill after a short incubation period , while oral intake alone only led to disease after decades. Men were probably less affected in general as they ate mostly lean meat.

Medical importance

The Kuru is of great medical and historical interest, but it received broader public attention, especially after the emergence of BSE and CJD in the 1990s. In 2009, medical researchers discovered that the Fore quickly developed a genetic mutation that prevented the disease from developing. In addition, it later turned out that this mutation also protects against all other Transmissible Spongiform Encephalopathies (TSE) . The researchers hope to gain insights into the treatment of other degenerative brain diseases such as Parkinson's disease and Alzheimer's disease .

Reporting requirement

Transmissible spongiform encephalopathies are in Austria in accordance with § 1 para. 1, point 1 Epidemics Act 1950 on suspicion, illness and death notifiable . In Germany, human spongiform encephalopathy (except for familial hereditary forms) is subject to notification by name in the event of suspicion, illness and death by the doctor etc. in accordance with Section 6 of the Infection Protection Act (IfSG) .

literature

• N. Bons, N. Mestre-Frances, P. Belli, F. Cathala, DC Gajdusek, P. Brown: Natural and experimental oral infection of nonhuman primates by bovine spongiform encephalopathy agents. In: Proc Natl Acad Sci USA . 1999 Mar 30; 96 (7), pp. 4046-4051.
• CJ Gibbs Jr, DM Asher, A. Kobrine, HL Amyx, MP Sulima, DC Gajdusek: Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery. In: Neurol Neurosurg Psychiatry. 1994 Jun; 57 (6), pp. 757-758.
• J. Tateishi, P. Brown, T. Kitamoto, ZM Hoque, R. Roos, R. Wollman, L. Cervenakova, DC Gajdusek: First experimental transmission of fatal familial insomnia. In: Nature . 1995 Aug 3; 376 (6539), pp. 434-435.
• J. Hinkelbein among others: Creutzfeldt-Jakob disease. In: Frank Wappler, Gerd Bürkle, Peter Tonner: Anesthesia and concomitant diseases. Thieme-Verlag, Stuttgart / New York 2006, ISBN 3-13-129941-X .

Individual evidence

1. ↑ Walter Bruchhausen: From bacteriology to molecular virology and prion research. The development of the infection theory . In: Dominik Gross, HJ Winkelmann (ed.): Medicine in the 20th century. Progress and Limits . Medical practice, Munich 2008, p. 6-25 .
2. ↑ Simon Mead et al: A Novel Protective Prion Protein Variant that Colocalizes with Kuru Exposure. In: The New England Journal of Medicine . Volume 361 (2009), No. 21, pp. 2056-2065.
3. ↑ A naturally occurring variant of the human prion protein completely prevents prion disease
4. ^ Former brain-eating Papua tribe offers clues on deadly diseases

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