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kuru
Epidemiology:
1) kuru was once endemic among members of the Fore linguistic tribal group of the Eastern highlands of Papua New Guinea
2) currently < 10 cases/year are reported
3) no new cases of Kuru have been reported since cessation of ritualistic cannibalism
Clinical manifestations:
1) cerebellar ataxia
2) associated involuntary movements
a) choreoathetosis
b) myoclonus
c) tremor
3) development of mental impairment & frontal release signs occur later
4) incubation period may be as long as 50 years
Management:
-> brain material from affected individuals transmits the disease to primates
General
transmissible spongiform encephalopathy (prion disease)
Properties
PATHOLOGY: kuru plaque
Database Correlations
OMIM 245300
References
- Harrison's Principles of Internal Medicine, 13th ed.
Isselbacher et al (eds), McGraw-Hill Inc. NY,
1994, pg 2319
- Collinge J et al, Kuru in the 21st century - an acquired human
prion disease with very long incubation periods.
Lancet 2006, 367:2068
PMID: 16798390
- National Institute of Neurological Disorders and Stroke (NINDS)
NINDS Kuru Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Kuru-Information-Page