{"Name":"Kuru","DiseaseID__c":"GARD:0007617","id":7617,"encodedName":"kuru","IsDeleted":false,"Disease_Name_Full__c":"Kuru","Xref_IDs__c":"86188000; A81.81; C0022802; D007729; DOID:648; MEDGEN:9653; MONDO:0006825; ORPHA:454745","USA_Estimate__c":"1,000","No_of_Specialist_Tagsa__c":3,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":"1 to 8,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":3,"Disease_Characteristics_Score__c":3,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0006825","Disease_Description__c":"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)","GARD_Name__c":"Kuru","GARD_Synonym__c":"kuru encephalitis; kuru encephalopathy","Curated_Disease_Description_Source__c":"MONDO:0006825","Curated_Disease_Description__c":"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)","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"1,000","Age_at_Onset_Snippet_Text__c":"at any time in life","SourceID__c":"ORPHA:454745","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0006825","ORPHANET_ID__c":"ORPHA:454745","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Kuru","Spanish_Description_Source__c":null,"Spanish_Description__c":null,"Spanish_Disease_Name__c":"kuru","Spanish_GARD_Synonym__c":null,"Category_Linearization__c":"ORPHA:98006","icd10_id__c":null,"mesh_id__c":null,"omim_id__c":null,"snomed_id__c":null,"umls_id__c":null,"GARD_Disease__c":[{"Curated_Disease_Description__c":"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)","Curated_Disease_Description_Source__c":"MONDO:0006825","GARD_Synonym__c":"kuru encephalitis; kuru encephalopathy","Name":"Kuru","Curated_USA_Estimate__c":"1,000","estimateUsa":"1,000"}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Neurology","Tag_Category__c":"Disease Category;Specialist","category_description":"Neurological diseases affect the brain, spinal cord, cranial nerves, autonomic nerves, or other peripheral nerves.","curated_tag_name":"Neurological diseases"},{"Tag_Name__c":"Psychiatry","Tag_Category__c":"Specialist"},{"Tag_Name__c":"Pediatrics","Tag_Category__c":"Specialist"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"All ages","Provided_By__c":"ORPHA:454745"}],"External_Identifier_Disease__c":[{"URL__c":"https://raresource.nih.gov/diseases/filter/0007617","Source__c":"RareSource"},{"URL__c":"https://www.ncbi.nlm.nih.gov/books/NBK1229","Source__c":"Gene Review","Xref__c":"NBK1229"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0022802","Source__c":"C0022802","Xref__c":"C0022802"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=86188000","Source__c":"C0022802; MONDO:0006825","Xref__c":"86188000"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=9653","Source__c":"C0022802","Xref__c":"MEDGEN:9653"},{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C007729","Source__c":"C0022802; MONDO:0006825","Xref__c":"D007729"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A648","Source__c":"MONDO:0006825","Xref__c":"DOID:648"},{"URL__c":"https://www.orpha.net/en/disease/detail/454745","Source__c":"C0022802; MONDO:0006825; ORPHA:454745","Xref__c":"ORPHA:454745"},{"URL__c":"http://purl.bioontology.org/ontology/ICD10CM/A81.81","Source__c":"MONDO:0006825","Xref__c":"A81.81"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0006825","Source__c":"GARD:0007617","Xref__c":"MONDO:0006825"}],"Inheritance__c":["Non-Mendelian inheritance"],"tags":{"Disease Category":["Neurology"],"Specialist":["Neurology","Psychiatry","Pediatrics"]},"synonyms":["kuru encephalitis"," kuru encephalopathy"]}