loading...| Preferred Name |
Kuru |
| ID |
http://www.orpha.net/ORDO/Orphanet_454745 |
| definition |
A rare acquired human prion disease characterized by rapidly progressive, fatal neurodegeneration, caused by the consumption of prion-containing tissue in endocannibalistic funeral rituals in Papua New Guinea until the late 1950s. After a decades-long asymptomatic period and a non-specific prodromal phase with headaches and arthralgia, the most prominent neurological feature is ataxia, in addition to other symptoms involving the cerebellum, brain stem, mid-brain, hypothalamus, and cerebral cortex, and emotional changes including inappropriate euphoria and compulsive laughter, or depression and apprehension. The last reported patient died in 2005 with an incubation period extending over four decades. |
| definition_citation |
Orphanet |
| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=454745 |
| has_age_of_onset |
All ages |
| has_inheritance |
Not applicable Multigenic/multifactorial |
| hasDbXref |
ICD-10:A81.8 UMLS:C0022802 ICD-11:8E01.1 OMIM:245300 |
| label |
Kuru |
| notation |
ORPHA:454745 |
| part_of | |
| prefixIRI |
ORDO:Orphanet_454745 |
| prefLabel |
Kuru |
| present_in |
Worldwide AND has_cases/families_value : 2700.0 (Case) Worldwide AND has_point_prevalence_range : <1 / 1 000 000 |
| treeView | |
| subClassOf |