A prion disease found exclusively among the Fore linguistic group natives of the highlands of new guinea. The illness is primarily restricted to adult females and children of both sexes. It is marked by the subacute onset of tremor and ataxia followed by motor weakness and incontinence. Death occurs within 3-6 months of disease onset. The condition is associated with ritual cannibalism, and has become rare since this practice has been discontinued. Pathologic features include a noninflammatory loss of neurons that is most prominent in the cerebellum, glial proliferation, and amyloid plaques. (From Adams et al., Principles of Neurology, 6th ed, p773) [ MESH:D007729 ]
Synonyms: kuru encephalopathy
Term information
- ICD9:046.0 (MONDO:i2s)
- DOID:648 (MONDO:equivalentTo)
- icd11.foundation:553889510 (Orphanet:454745)
- GARD:7617 (MONDO:GARD)
- ICD10CM:A81.81 (MONDO:equivalentTo)
- UMLS:C0022802 (MONDO:equivalentTo)
- SCTID:86188000 (MONDO:equivalentTo)
- EFO:1001008 (MONDO:equivalentTo)
- MedDRA:10023497 (EFO:1001008)
- MESH:D007729 (MONDO:equivalentTo)
- Orphanet:454745 (MONDO:equivalentTo)
- MEDGEN:9653 (MONDO:equivalentTo)
gard_rare, ordo_disorder, otar, rare, nord_rare, orphanet_rare
http://identifiers.org/medgen/9653
http://purl.bioontology.org/ontology/ICD10CM/A81.81
http://purl.obolibrary.org/obo/DOID_648
http://linkedlifedata.com/resource/umls/id/C0022802
http://identifiers.org/snomedct/86188000
http://www.orpha.net/ORDO/Orphanet_454745
http://identifiers.org/mesh/D007729
Term relations
- movement disorder
- human prion disease
- prion disease
- disease arises from feature some neurodegenerative disease