Doença de príon encontrada exclusivamente entre os nativos do grupo linguístico Fore das terras altas da Nova Guiné. A doença é restrita principalmente a mulheres adultas e crianças de ambos os sexos. É marcada pelo início subagudo de tremor e ataxia seguido de fraqueza motora e incontinência. A morte ocorre dentro de 3-6 meses após o início da doença. A condição está associada ao canibalismo ritual e tornou-se rara desde que esta prática foi descontinuada. As características patológicas incluem perda não inflamatória de neurônios que é mais proeminente no cerebelo, proliferação glial e placas amilóides. (De Adams et al., Princípios de Neurologia, 6ª ed, p773)
Introdução
O que você precisa saber de cara
Doença de príon encontrada exclusivamente entre os nativos do grupo linguístico Fore das terras altas da Nova Guiné. A doença é restrita principalmente a mulheres adultas e crianças de ambos os sexos. É marcada pelo início subagudo de tremor e ataxia seguido de fraqueza motora e incontinência. A morte ocorre dentro de 3-6 meses após o início da doença. A condição está associada ao canibalismo ritual e tornou-se rara desde que esta prática foi descontinuada. As características patológicas incluem perda não inflamatória de neurônios que é mais proeminente no cerebelo, proliferação glial e placas amilóides. (De Adams et al., Princípios de Neurologia, 6ª ed, p773)
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
1 gene identificado com associação a esta condição. Padrão de herança: Multigenic/multifactorial, Not applicable.
Its primary physiological function is unclear. May play a role in neuronal development and synaptic plasticity. May be required for neuronal myelin sheath maintenance. May promote myelin homeostasis through acting as an agonist for ADGRG6 receptor. May play a role in iron uptake and iron homeostasis. Soluble oligomers are toxic to cultured neuroblastoma cells and induce apoptosis (in vitro) (By similarity). Association with GPC1 (via its heparan sulfate chains) targets PRNP to lipid rafts. Also
Cell membraneGolgi apparatus
Variantes genéticas (ClinVar)
79 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 11 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
2 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Kuru
Selecione um estado ou use sua localização para ver resultados.
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
When I use a word . . . Medical anniversaries in 2026.
My list of medically related anniversaries for 2026 (events in years ending '26 and'76) includes:• Births: Abū al-Walīd Muḥammad ibn Aḥmad ibn Rushd, the physician better known as Averroes (1126); the Flemish botanist Charles de L'Ecluse (Carolus Clusius) (1576); Amadeo Avogadro, Italian physicist (1776); Johann Spurzheim, German physician (1776); George Birkbeck, English physician (1776).• Deaths: Francis Bacon (1626); Jean Anthelme Brillat-Savarin, French lawyer and gastronome (1826); Friedrich Wilhelm Weiß, German physician and composer (1826); René Laënnec, French physician (1826); Philippe Pinel, French physician (1826); Walter Channing, American physician (1876); Victor Babeș, Romanian physician and bacteriologist (1926); William Bateson, English biologist (1926); Emile Coué, French pharmacist and psychologist (1926); Camillo Golgi, Italian anatomist (1926); Emil Kraepelin, German psychiatrist (1926); Einar Aaser, Norwegian physician (1976); Andrew Arthur Abbie, Australian anatomist and anthropologist (1976); Jacques Monod, molecular biologist (1976); George Whipple, American physician (1976); Alexander S Wiener, American physician (1976).• Biomedical texts published: Observationes medicae circa morborum acutorum historiam et curationem by Thomas Sydenham (1676); A Comment on Forty two Histories Discribed [sic] by Hippocrates in the First and Third Books of his Epidemics by John Floyer (1726); The botanical arrangement of all the vegetables naturally growing in Great Britain by William Withering (1776); De generis humani varietate nativa by Johann Friedrich Blumenbach (1776); L'uomo delinquente (Criminal Man) by Cesare Lombroso (1876); Geographical distribution of animals by Alfred Wallace (1876); The theory of the gene by Thomas Hunt Morgan (1926).• Clinical therapies introduced: George Richards Minot and William Parry Murphy (treatment of pernicious anaemia with liver, 1926).• Biochemical and bacteriological observations: Karl Wilhelm Scheele (uric acid in kidney stones, 1776); Otto Unverborden (discovery of aniline, 1826); Robert Koch (the anthrax bacillus, 1876); James Batcheller Sumner (crystallisation of jackbean urease, 1926).• Establishment of the Edinburgh Medical School (1726) and of The Body Shop (Anita Roddick, 1976).• Epidemics of typhus in Spain (1576) and Ebola virus infection in Yambuku, Zaire (1976).• Nobel prizes for physiology or medicine awarded to Baruch Samuel Blumberg for identifying Australia antigen as an indicator in the blood of hepatitis B and to Daniel Carleton Gajdusek for his work on the origin and spread of infectious diseases, particularly slow virus infections and specifically kuru (both 1926), and to Johannes Andreas Grib Fibiger for discovering the Spiroptera carcinoma (1976).
A Longitudinal Study on Symptom Severity in Early Childhood of Bilingual and Monolingual Children With Autism Spectrum Disorder.
In increasingly globalized and multilingual societies, a key question for individuals with autism spectrum disorder (ASD) is how bilingual exposure influences their linguistic, cognitive, and social development. This study aimed to investigate the potential impact of a bilingual home environment on the severity and progression of ASD symptoms in children. The study was conducted with 3-year follow-up results of a total of 69 children, 34 with bilingual home situations (BHSs) and 35 with monolingual home situations (MHSs). The Childhood Autism Rating Scale (CARS) was applied to assess the severity of the participants' ASD symptoms. Neurodevelopmental evaluations were performed using the Denver Developmental Screening Test II (DDST-II). No significant differences were determined between the MHS and BHS groups in terms of developmental quotient total (DQt) baseline (p = 0.618), DQt follow-up (p = 0.288), or CARS baseline (p = 0.970) scores. However, CARS follow-up scores, indicative of greater symptom severity, were significantly higher in the BHS group (p = 0.030). While the MHS group showed a notable decrease in CARS scores, the BHS group's scores tended to persist, with the decrease in CARS scores being significantly lower than that observed in the MHS group (p < 0.001). These findings suggest that children with ASD raised in bilingual home environments may exhibit comparatively less improvement in symptom severity over time. The influence of bilingualism on ASD may involve a range of complex linguistic, cognitive, and social factors. Future research should explore these variables in larger, more detailed studies.
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Response to Letter on "Hemostasis Time as a Criterion in Vital Pulpotomy: Methodological and Biological Considerations".
[Prion diseases : Creutzfeldt-Jakob and differential diagnoses].
Prions are unprecedented infectious pathogens that cause a group of rare and inevitably fatal neurodegenerative diseases, affecting approximately 1 person per 1 million inhabitants worldwide each year. These diseases include Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), kuru, fatal insomnia (FI) and variably protease-sensitive prionopathy (VPSPr), all of which involve a conformational change of normal cellular prion protein (PrPC) into abnormal scrapie prion protein (PrPSc) through a posttranslational process. This structural change is associated with profound alterations in the physicochemical properties of PrPC, making the molecule resistant to proteolysis. The conformational alteration of PrPC can occur either due to spontaneous conversion, dominant mutations in the prion protein gene (PRNP) which codes for PrPC or due to an infection with the pathogenic isoform PrPSc from exogenous sources. There is general consensus that PrPC serves as the substrate for the conversion to abnormal PrPSc. The latter multiplies exponentially and accumulates in the brain, forming deposits which are associated with the neurodegenerative changes. Although the understanding of the main causes of prion-induced neurodegeneration is still limited, the spread of PrPSc and neurotoxic signal transfer in the pathogenic process of prions appear to show an interaction. Prion diseases sometimes have long incubation times but also short clinical trajectories. Sporadic and genetic forms occur worldwide of which genetic forms are associated with mutations in PRNP. Zoonotic forms of prion diseases are also associated with bovine diseases. Substantial progress has been made in the diagnosis of these disorders and the diagnostics include magnetic resonance imaging (MRI) and laboratory investigations, particularly of the cerebrospinal fluid. Prionen sind beispiellose infektiöse Krankheitserreger, die eine Gruppe seltener und unweigerlich tödlicher neurodegenerativer Erkrankungen verursachen, die weltweit jährlich etwa 1 Person pro 1 Mio. Einwohner betreffen. Zu diesen Erkrankungen gehören die Creutzfeld-Jacob-Krankheit (CJK), Gerstmann-Sträussler-Scheinker-Syndrom (GSS), Kuru, fatale Schlaflosigkeit (FI) und variable Protease-sensitive Prionopathie (VPSPr), die alle eine konformationelle Veränderung des normalen zellulären Prionproteins (PrPC) in das abnormale Scrapie-Prionprotein (PrPSc) durch einen posttranslationalen Prozess beinhalten. Diese strukturelle Veränderung geht mit tiefgreifenden Veränderungen in den physikochemischen Eigenschaften von PrPC einher, wodurch das Molekül widerstandsfähig gegen Proteolyse wird. Die konformationelle Veränderung von PrPC kann entweder durch spontane Umwandlung, dominante Mutationen im Prionprotein(PRNP)-Gen, das für PrPC codiert, oder durch eine Infektion mit der pathogenen Isoform PrPsc aus exogenen Quellen entstehen. Es besteht allgemeine Einigkeit darüber, dass PrPC als Substrat für die Umwandlung zu abnormalem PrPSc dient. Letzteres vermehrt sich exponentiell und sammelt sich im Gehirn, wodurch Ablagerungen entstehen, die mit den neurodegenerativen Veränderungen verbunden sind. Obwohl das Verständnis der Hauptursachen der prioninduzierten Neurodegeneration noch begrenzt ist, scheint zwischen der Ausbreitung von PrPSc und neurotoxischer Signalübertragung im pathogenen Prozess der Prionen eine Wechselwirkung zu bestehen. Prionenerkrankungen weisen z. T. lange Inkubationszeiten auf und andererseits kurze klinische Verläufe. Weltweit kommen sporadische und genetische Formen vor, von denen genetische Formen mit Mutationen in PRNP assoziiert sind. Zoonotische Formen von Prionenerkrankungen sind u. a. mit Rinderkrankheiten verbunden. Bei der Diagnose dieser Störungen wurden erhebliche Fortschritte erzielt, die Diagnose umfassen Magnetresonanztomographie (MRT) und laborchemische Untersuchungen, insbesondere des Liquor cerebrospinalis.
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Molecular biology and evolutionComparison of remineralization ability of tricalcium silicate and of glass ionomer cement on residual dentin: an in vitro study.
BMC oral healthLoss-of-function polymorphisms in NQO1 are not associated with the development of subacute myelo-optico-neuropathy.
Molecular genetics & genomic medicineDevelopment of nanobiosensor for therapeutic drug monitoring in personalized cancer treatment approach.
Journal of biomaterials science. Polymer editionIslands and Neurology: An Exploration into a Unique Association.
The Neuroscientist : a review journal bringing neurobiology, neurology and psychiatryBone mineral density of the incus body and long process in patients with chronic otitis media and its relationship with functional outcome in type II tympanoplasty.
Acta oto-laryngologicaFrequency of fibromyalgianess in patients with rheumatoid arthritis and ankylosing spondylitis: A multicenter study of Turkish League Against Rheumatism (TLAR) network.
Archives of rheumatologyDetermination of anti-Müllerian hormone levels in blood and urine in fertile cats.
TheriogenologyFrom cell lines to cancer patients: personalized drug synergy prediction.
Bioinformatics (Oxford, England)Treatment of multiple adjacent gingival recessions using leucocyte- and platelet-rich fibrin with coronally advanced flap: a 12-month split-mouth controlled randomized clinical trial.
Clinical oral investigationsPrion meeting 2023: implications of a growing field.
PrionThe Association Between Physical Activity/Heart Rate Variability Data Obtained Using a Wearable Device and Timed Motor Functional Tests in Patients with Duchenne Muscular Dystrophy: A Pilot Study.
Journal of neuromuscular diseasesPotential of photon counting computed tomography derived spectral reconstructions to reduce beam-hardening artifacts in chest CT.
European journal of radiologyFacioscapulohumeral muscular dystrophy Health Index: Japanese translation and validation study.
Disability and rehabilitationCharacteristics of patients with late recurrence endometrial cancer.
Journal of cancer research and therapeuticsInvestigation of the effects of different drugs on the prevention of intrapleural adhesion in a rat model.
Turk gogus kalp damar cerrahisi dergisiViroids, Satellite RNAs and Prions: Folding of Nucleic Acids and Misfolding of Proteins.
VirusesDiagnostic and prognostic efficacy of optic nerve sheath diameter in patients with dysnatremia.
Medical ultrasonographyPopulation structure and migration in the Eastern Highlands of Papua New Guinea, a region impacted by the kuru epidemic.
American journal of human geneticsHydrogen-rich water supplementation improves metabolic profile during peripartum period in Gurcu goats and enhances the health and survival of kids.
Research in veterinary sciencePrognostic value of lymph node features in patients diagnosed with stage IIIC endometrial adenocancer.
Journal of cancer research and therapeuticsPsychometrics of rating scales for externalizing disorders in Japanese outpatients: The ADHD-Rating Scale-5 and the Disruptive Behavior Disorders Rating Scale.
International journal of methods in psychiatric researchAn autopsy case of MV 2K + C subtype of Creutzfeldt-Jakob disease.
Neuropathology : official journal of the Japanese Society of NeuropathologyKuru Disease: Bridging the Gap Between Prion Biology and Human Health.
CureusTreatment of trigger finger with metacarpophalangeal joint blocking orthosis vs relative motion extension orthosis: A randomized clinical trial.
Journal of hand therapy : official journal of the American Society of Hand TherapistsInserting "OFF-to-ON" BODIPY Tags into Cytokines: A Fluorogenic Interleukin IL-33 for Real-Time Imaging of Immune Cells.
ACS central scienceMultiple Penile and Scrotal Schwannomas in an Adult Patient: A Case Report.
Urologia internationalisNationwide survey of patients with multisystem proteinopathy in Japan.
Annals of clinical and translational neurologyThe Relationship Between Health-Related Quality of Life and Overall Survival in Patients With Advanced Renal Cell Carcinoma in CheckMate 214.
The oncologistThe effect of occupational exposure to noise and chemical agents on hearing abilities.
Archives of environmental & occupational healthAffinity-Based Magnetic Nanoparticle Development for Cancer Stem Cell Isolation.
PolymersEffect of Pre-exercise Sodium Citrate Ingestion on Repeated Sprint Performance in Soccer Players.
Journal of strength and conditioning researchRadical Prostatectomy on YouTube: Education or Disinformation?
Archivos espanoles de urologia[Evaluation of the GeSRU-Steps educational video concept (German Society of Residents in Urology e. V.)].
Urologie (Heidelberg, Germany)Anti-inflammatory, antioxidant, antiangiogenic, and therapeutic efficacy of neroli oil in rats with endometriotic lesions.
The journal of obstetrics and gynaecology researchAttitudes of parents of children with ADHD towards genetic testing: Data from a Turkish sample.
Journal of genetic counselingTreatment of Nocturnal Enuresis Using Miniaturised Smart Mechatronics With Artificial Intelligence.
IEEE journal of translational engineering in health and medicineIdentifying Behavior Change Techniques in an Artificial Intelligence-Based Fitness App: A Content Analysis.
Health education & behavior : the official publication of the Society for Public Health EducationAssessment of wellbore integrity failure risk and hazardous zones in depleted reservoirs underground gas storage during the operation processes.
Environmental science and pollution research internationalThe impact of the first wave of the COVID-19 pandemic on hospital admissions and treatment management of ectopic pregnancy.
Turkish journal of emergency medicineA combination of non-invasive tests for the detection of significant fibrosis in patients with metabolic dysfunction-associated steatotic liver disease is not superior to magnetic resonance elastography alone.
European radiologyThe concern about the educational quality of online videos on laparoscopic myomectomy.
Revista da Associacao Medica Brasileira (1992)Mapping Choice of Healthcare Institutes for Cancer Care: A Study in Northeast India.
Asian Pacific journal of cancer prevention : APJCPNatural history of Becker muscular dystrophy: a multicenter study of 225 patients.
Annals of clinical and translational neurologyEvaluation of the relationship between Ki67 expression level and neoadjuvant treatment response and prognosis in breast cancer based on the Neo-Bioscore staging system.
Discover oncologyHuman prion diseases and the prion protein - what is the current state of knowledge?
Translational neuroscienceInvestigation of Cognitive Distortions in Panic Disorder, Generalized Anxiety Disorder and Social Anxiety Disorder.
Journal of clinical medicineSeasonal variation in the expression pattern of heat shock protein 70 and 90 in Common carp (Cyprinus carpio) from Karataş Lake, Burdur, Türkiye.
Veterinary research forum : an international quarterly journalThe effect of hydrogen-rich water on letrozole-induced polycystic ovary syndrome in rats.
Reproductive biomedicine onlinePentraxin-3, endothelin-1, some biochemical parameters and hematology in bovine respiratory disease complex.
Iranian journal of veterinary researchPhase information is conserved in sparse, synchronous population-rate-codes via phase-to-rate recoding.
Nature communicationsThe prognostic significance of the heterologous component in uterine carcinosarcomas.
Revista da Associacao Medica Brasileira (1992)First Record, Distribution and Occurrence of A Protistan Entomopathogen, Adelina mesnili Perez (Coccidia: Adeleidae) in the Indian Meal Moth, Plodia interpunctella (Hübner) (Lepidoptera: Pyralidae) Populations in Türkiye.
Turkiye parazitolojii dergisiComparison of the effects of alpha lipoic acid and dexpanthenol in an experimental tracheal reconstruction animal model.
Saudi medical journalA Theoretical Framework on the Biology of Prion Diseases.
Acta informatica medica : AIM : journal of the Society for Medical Informatics of Bosnia & Herzegovina : casopis Drustva za medicinsku informatiku BiH[Polypharmacy and medication review in the context of prehabilitation].
Urologie (Heidelberg, Germany)Novel histotypes of sporadic Creutzfeldt-Jakob disease linked to 129MV genotype.
Acta neuropathologica communicationsTwo-Point Discrimination in Feet with Ankle Sprains.
Journal of the American Podiatric Medical AssociationEvaluation of YouTube Information Quality About Pes Planus.
Journal of the American Podiatric Medical AssociationBiomechanical Effect of Ankle Ligament Injury in AO Type 44B2.1 Lateral Malleolus Fractures After Lateral Plate Fixation: A Finite Element Analysis.
Journal of the American Podiatric Medical AssociationComputational Mechanical Analysis of AO 44A1, 44B1, and 44C1 Fractures with Finite Element Modeling: Evaluation of Screw, Plate, and Kirschner Wire Fixation.
Journal of the American Podiatric Medical AssociationChildhood Non-verbal Communication Scale (CNCS): Turkish Adaptation and Psychometric Properties.
Noro psikiyatri arsiviCase-based report of graded motor imagery experience in traumatic brachial plexus injury: The art of moving without moving.
Journal of hand therapy : official journal of the American Society of Hand TherapistsThe prognostic value of P53 index in predicting the recurrence of early low-risk endometrial cancer.
The journal of obstetrics and gynaecology researchCharacteristics and predictors of family accommodation in Turkish individuals with obsessive-compulsive disorder.
Turkish journal of medical sciencesMetal-Chelated Polymeric Nanomaterials for the Removal of Penicillin G Contamination.
PolymersAssessment of Reliability of Three Indices Measuring Gingival Overgrowth.
Acta stomatologica CroaticaShort-term clinical and hormonal effects of a deslorelin implant on late-prepubertal bitches - Based on flare-up signs.
TheriogenologyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Kuru.
É de uma associação que acompanha esta doença? Fale com a gente →
Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Kuru
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- When I use a word . . . Medical anniversaries in 2026.
- A Longitudinal Study on Symptom Severity in Early Childhood of Bilingual and Monolingual Children With Autism Spectrum Disorder.International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience· 2026· PMID 41472332mais citado
- Cross-education after forearm fracture: a randomised controlled trial.
- Response to Letter on "Hemostasis Time as a Criterion in Vital Pulpotomy: Methodological and Biological Considerations".Medical principles and practice : international journal of the Kuwait University, Health Science Centre· 2026· PMID 41719215mais citado
- [Prion diseases : Creutzfeldt-Jakob and differential diagnoses].
- Lithic Miniaturization Provides a Signature of an MIS4-3 Southern Dispersal of Homo sapiens.
- Leveraging the dominant-negative effect of the kuru-protective G127V prion protein variant as a novel therapeutic strategy.
- Prion-induced ferroptosis is facilitated by RAC3.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:454745(Orphanet)
- MONDO:0006825(MONDO)
- GARD:7617(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q274615(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
