Kuru

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)

Also Known As:

Networked: 133 relevant articles (1 outcomes, 7 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Creutzfeldt-Jakob Syndrome (Creutzfeldt-Jakob Disease)
2.	Scrapie
3.	Bovine Spongiform Encephalopathy (Mad Cow Disease)
4.	Amyloid Plaque
5.	Alzheimer Disease (Alzheimer's Disease)

Experts

1.	Collinge, John: 10 articles (06/2015 - 06/2006)
2.	Mead, Simon: 8 articles (06/2015 - 06/2006)
3.	Whitfield, Jerome: 7 articles (06/2015 - 06/2006)
4.	Alpers, Michael P: 7 articles (04/2012 - 06/2006)
5.	Brandner, Sebastian: 4 articles (06/2015 - 03/2008)
6.	Uphill, James: 4 articles (04/2012 - 11/2008)
7.	Campbell, Tracy: 4 articles (04/2012 - 11/2008)
8.	Poulter, Mark: 4 articles (04/2012 - 11/2008)
9.	Hummerich, Holger: 4 articles (04/2012 - 11/2008)
10.	Asante, Emmanuel A: 3 articles (06/2015 - 03/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Kuru:

1.	Amyloid (Amyloid Fibrils)IBA 01/01/1998 - "Some PrP deposits are also of the amyloid variety (kuru type, multicentric or florid plaques), but immunohistochemistry, far more sensitive than conventional studies, revealed a number of other lesions (perivacuolar, neuronal, "synaptic" deposits...). " 01/01/2012 - "Neuropathologically, kuru is characterized by the presence of amyloid "kuru" plaques." 01/01/2004 - "Unicentric "kuru" plaques consisted of stellate arrangements (stars or cores) of amyloid bundles emanating from a dense interwoven centre. " 09/01/2001 - "Uniform topographical central nervous system histopathology includes spongiform change and neuronal loss, with amyloid (kuru) plaques in approximately 75% of cases." 01/01/2001 - "The first type, unicentric "kuru" plaque, consisted of stellate arrangements (stars or cores) of amyloid bundles emanating from a densely interwoven centre. "
2.	Acquired CJDIBA 08/01/2015 - "Atypical sporadic CJD-MM phenotype with white matter kuru plaques associated with intranuclear inclusion body and argyrophilic grain disease." 01/01/2014 - "The other SNP of the gene that encodes SCG10 (STMN2), which is related to clinical onset of sporadic CJD, was also associated with variant CJD and kuru. " 05/01/2011 - "Murine adapted human agents, including sporadic CJD (sCJD), New Guinea kuru, and Japanese CJD agents, display particularly distinct incubation times and maximal infectious brain titers. " 12/01/2010 - "In 2 cases of sporadic CJD, the kuru stellate plaque predominated." 08/11/2009 - "Many TSE agents can infect monotypic cultured GT1 cells, and unlike sporadic CJD isolates, kuru rapidly and stably infected these cells. "
3.	AntibodiesIBA 01/01/2008 - "Distinct immunohistochemical localization in Kuru plaques using novel anti-prion protein antibodies." 12/01/1989 - "Antibodies on the Western blot membrane from murine spleen PrPCJD fractions stained the kuru plaques in the CJD-infected mouse brain. " 03/01/1978 - "A serological survey is reported for antibodies to Coxsackie, ECHO and polioviruses on sera collected in the kuru region of Papua New Guinea during earliest European contacts with kuru-affected and control populations through the period of highest kuru incidence. " 12/01/1999 - "Histological studies showed atrophy of the cerebellar molecular layer, which contained kuru and multicentric plaques labelled with anti-prion protein antibodies. " 01/01/1999 - "Kuru transmitted by cannibalism involved mainly the cerebellum with cerebellar tracts degeneration and presence of amyloid plaques called "kuru plaques" labeled by anti PrP antibodies. "
4.	Methionine (L-Methionine)FDA Link 06/01/2004 - "Recent studies indicate that individuals homozygous for Methionine at a polymorphic position 129 of the prion protein were preferentially affected during the kuru epidemic. " 01/15/2001 - "Increased susceptibility to Kuru of carriers of the PRNP 129 methionine/methionine genotype." 11/19/2009 - "We now report a novel PRNP variant--G127V--that was found exclusively in people who lived in the region in which kuru was prevalent and that was present in half of the otherwise susceptible women from the region of highest exposure who were homozygous for methionine at PRNP codon 129. " 01/15/2001 - "Analysis of the PRNP 129 methionine (M)/valine (V) polymorphism in 80 patients and 95 unaffected controls demonstrated that the kuru epidemic preferentially affected individuals with the M/M genotype. " 11/27/2008 - "Genotype analysis of the polymorphism on codon 129 reveals (in conjunction with updated information from more kuru cases) that all three genotypes (VV, MV and MM (where M is methionine and V is valine)) are detected in kuru with some preference for MM homozygosity. "
5.	Codon (Codons)IBA 08/06/2013 - "To elucidate the epidemiological mechanism of human resistance, we estimated the incubation period and transmission rate of kuru for codon 129 heterozygotes and homozygotes using kuru incidence data and human genotype frequency data from 1957 to 2004. " 11/19/2009 - "Persons who were exposed to kuru and survived the epidemic in Papua New Guinea are predominantly heterozygotes at the known resistance factor at codon 129 of the prion protein gene (PRNP). " 11/27/2008 - "Elderly survivors of the kuru epidemic, who had multiple exposures at mortuary feasts, are predominantly PRNP codon 129 heterozygotes and this group show marked Hardy-Weinberg disequilibrium. " 11/27/2008 - "In these patients, the mean clinical duration of 17 months was longer than the overall average in kuru but similar to that previously reported for the same age group, and this may relate to the effects of both patient age and PRNP codon 129 genotype. " 08/01/2006 - "Autopsy case of Creutzfeldt-Jakob disease with Met/Val heterozygosity at codon 129 and type 1 protease-resistant prion protein presenting some florid-type plaques and many Kuru plaques in the cerebellum."
6.	Valine (L-Valine)FDA Link 01/15/2001 - "Analysis of the PRNP 129 methionine (M)/valine (V) polymorphism in 80 patients and 95 unaffected controls demonstrated that the kuru epidemic preferentially affected individuals with the M/M genotype. " 12/01/1996 - "Also they did not have a valine polymorphism at PrP codon 129 which is frequently observed in the patients with iatrogenic CJD and kuru. " 11/27/2008 - "Genotype analysis of the polymorphism on codon 129 reveals (in conjunction with updated information from more kuru cases) that all three genotypes (VV, MV and MM (where M is methionine and V is valine)) are detected in kuru with some preference for MM homozygosity. " 08/01/1999 - "Seventy percent of subjects showed the classic CJD phenotype, PrP(Sc) type 1, and at least one methionine allele at codon 129; 25% of cases displayed the ataxic and kuru-plaque variants, associated to PrP(Sc) type 2, and valine homozygosity or heterozygosity at codon 129, respectively. " 01/01/2015 - "Autopsy examination revealed a neuropathological phenotype reminiscent of both sCJDMV2-K (a sporadic CJD subtype in subjects methionine/valine heterozygous at codon 129 with PrP(Sc) type 2 and the presence of kuru plaques) and variant CJD (vCJD). "
7.	ParaffinIBA 02/01/2009 - "Immunohistochemistry was performed on paraffin sections from one case of vCJD, two cases of GSS, one case of kuru and two cases of sCJD. " 03/01/2003 - "We examined paraffin-embedded brain sections of sporadic MV2 Creutzfeldt-Jakob disease (sCJD) with Kuru plaques, sporadic VV2 CJD with plaque-like PrP(sc) (the abnornal form of prion protein) deposits, variant CJD (vCJD) with florid plaques, Gerstmann-Straüssler-Scheinker (GSS) with multicentric plaques and of Alzheimer's disease (AD) with senile plaques. " 09/03/2003 - "The study aimed to evaluate the fluorescent molecular-imaging probe 2-(1-[6-[(2-fluoroethyl)(methyl)amino]-2-naphthyl]ethylidene)malononitrile (FDDNP) for its ability to selectively and reproducibly label prion plaques in fixed, paraffin-embedded cerebellar sections from patients of confirmed Gerstmann-Sträussler-Scheinker disease, sporadic Creutzfeldt-Jacob disease (CJD) with kuru plaques, and variant CJD (vCJD). " 10/01/1988 - "We investigated paraffin-embedded brain sections from 41 patients with Creutzfeldt-Jakob disease (CJD) and from 9 with Gerstmann-Sträussler syndrome (GSS) using anti-human prion protein (PrP) antisera (anti-GSS kuru plaque cores and anti-PrP synthetic peptide) and anti-beta protein antiserum. " 12/01/1991 - "Formic acid pretreatment on formalin fixed, paraffin-embedded thin sections enhanced immunostaining of PrP in both congophilic and non-congophilic kuru plaques which were absent in sporadic CJD patients with short clinical courses. "
8.	PeptidesIBA 02/01/1991 - "To demonstrate that prion protein (PrP) is a component of kuru plaque cores, we fractionated and sequenced kuru plaque core derived peptides, following digestion with Achromobacter lyticus protease I. We identified 3 PrP-derived peptides by reverse-phase high-performance liquid chromatography and found a fragment of digests derived from a missense variant of PrP. " 01/01/1992 - "Immunohistochemical study of kuru plaques using antibodies against synthetic prion protein peptides."
9.	Ferritins (Ferritin)IBA 01/01/1994 - "To study the relationship between microglia and amyloid plaques in kuru, CJD and GSS, cerebellar tissues were examined by the double-immunostaining technique using anti-ferritin antibodies as the microglial marker and anti-scrapie amyloid antibody as plaque marker. "
10.	dicyanmethane (malononitrile)IBA 09/03/2003 - "The study aimed to evaluate the fluorescent molecular-imaging probe 2-(1-[6-[(2-fluoroethyl)(methyl)amino]-2-naphthyl]ethylidene)malononitrile (FDDNP) for its ability to selectively and reproducibly label prion plaques in fixed, paraffin-embedded cerebellar sections from patients of confirmed Gerstmann-Sträussler-Scheinker disease, sporadic Creutzfeldt-Jacob disease (CJD) with kuru plaques, and variant CJD (vCJD). "

Therapies and Procedures

1.	Transplants (Transplant) 09/01/1997 - "[Creutzfeldt-Jakob disease with a widespread presence of kuru-type plaques after cadaveric dural graft replacement. " 01/01/2013 - "Kuru was the result of ritual funeral practices similar to cannibalism; variant CJD affects young people who have eaten meat from cows with mad cow disease (mostly in the UK); and iatrogenic CJD is secondary to graft of human tissues performed in the 1980s (dura mater, pituitary extracted growth hormone). " 08/01/2007 - "Usually, peripheral infection such as oral(Kuru) or parenteral (human pituitary hormones) transmission causes cerebellar degenerative form, and central nervous system infection such as neurosurgical treatment, dura mater grafts or corneal grafts transmission causes clinical features similar to sporadic form of Creutzfeldt-Jakob disease (CJD). " 08/01/2014 - "Here, we review and discuss how genetic factors interplay with strain properties and route of transmission to influence disease susceptibility, incubation period and phenotypic expression in the light of the kuru epidemics due to ritual endocannibalism, the various series iatrogenic diseases secondary to extractive growth hormone treatment or dura mater graft and the epidemics of variant Creutzfeldt-Jakob disease linked to dietary exposure to the agent of bovine spongiform encephalopathy. "
2.	Dilatation and Curettage (D&C) 05/01/1994 - "First, a Slow Virus was evoked, then Hadlow DVM (USA) suggested using brain filtrates from deceased patients of Kuru in order to inoculate primates or small rodents; this was carried out by the team of D.C. "