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Encefalite Borna virus
ORPHA:637051CID-10 · A85.8CID-11 · 1C80DOENÇA RARA

A vacina para a encefalite transmitida por carrapatos é uma vacina utilizada para prevenir a encefalite transmitida por carrapatos. Essa doença é mais comum na Europa Central e Oriental, e no norte da Ásia. mais de 87% das pessoas vacinadas desenvolvem imunidade. No entanto, ela não é eficaz se recebida logo após a picada de um carrapato infectado. A via de injeção é intramuscular.

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Introdução

O que você precisa saber de cara

📋

Doença infeciosa rara caracterizada por meningoencefalite grave não purulenta causada pelo vírus da doença Borna 1 (BoDV-1). Os doentes apresentam febre, dor de cabeça, mal-estar e fadiga, seguidos após alguns dias por náusea, confusão e desorientação, e lentidão psicomotora. Ataxia, afasia, dispneia, espasmos mioclónicos multifocais e paraplegia foram descritos. A condição é frequentemente fatal após progredir para letargia, deterioração dos reflexos do tronco cerebral e coma. O exame do líquido cefalorraquidiano revela pleocitose linfo-monocítica moderada e presença de RNA BoDV-1.

Publicações científicas
52 artigos
Último publicado: 2024

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Casos conhecidos
18
pacientes catalogados
Início
Adolescent
+ adult, elderly
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: A85.8
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa11
Total histórico52PubMed
Últimos 10 anos86publicações
Pico202214 papers
Linha do tempo
20202015Hoje · 2026📈 2022Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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Publicações mais relevantes

Timeline de publicações
0 papers (10 anos)
#1

Development of a strand-specific RT-qPCR assay for detecting and quantifying Borna disease virus RNAs in infected cells.

Journal of virological methods2026 May

Borna disease virus 1 (BoDV-1) is a nonsegmented negative-strand RNA virus (NSV) that persists in the central nervous system of a broad range of mammalian species. Considering the recent reports linking BoDV-1 to fatal encephalitis in humans, a deeper understanding of its viral life cycle is required. Similar to other NSVs, BoDV-1 produces three types of viral RNAs in infected cells: genome, antigenome, and mRNA. Although a conventional reverse transcription quantitative real-time PCR (RT-qPCR) assay is used to detect viral RNAs, this assay cannot distinguish between RNA species because of primer-independent cDNA synthesis during the reverse transcription (RT) reaction. In this study, we developed a strand-specific RT-qPCR (ssRT-qPCR) assay, in which an RT-primer fused with a non-viral tag sequence at the 5'-end is used in the RT reaction. This assay successfully detected and quantified three types of BoDV-1 RNAs distinctively. Furthermore, using this assay, we measured the intracellular kinetics of the BoDV-1 RNA species, revealing that BoDV-1 may control transcription, but not replication, to establish and maintain persistent infection. Overall, this novel ssRT-qPCR assay is a powerful tool for understanding the life cycle of BoDV-1.

#2

Is vaccination a feasible public health strategy against fatal Borna disease virus 1 (BoDV-1) encephalitis? An epidemiological perspective.

PLoS pathogens2025 Oct

Human Borna disease virus 1 (BoDV-1) encephalitis is characterized by rapid clinical progression, an absence of a causal therapy and an extremely high case fatality rate. Here, we discuss prevention options through a hypothetical vaccine focusing on epidemiological features.

#3

Structural insights into the dynamic mechanism of bornavirus polymerase.

Proceedings of the National Academy of Sciences of the United States of America2025 Sep 30

Borna disease virus 1 (BoDV-1), an emerging zoonotic pathogen from the Bornaviridae family, is neurotropic and can infect a variety of mammalian hosts, including humans. Linked to severe encephalitis and high mortality, BoDV-1 currently lacks licensed treatments or vaccines. The BoDV-1 polymerase complex, comprising the large (L) and phosphoprotein (P) subunits, is crucial for viral replication and transcription, making it a promising target for antiviral intervention. Here, we present the cryoelectron microscopy structure of the apo BoDV-1 L-P complex, revealing a unique "mitten-shaped" architecture. The structure characterizes key domains involved in RNA synthesis, including RNA-dependent RNA polymerase, polyribonucleotidyltransferase, and an inactive methyltransferase domain. While no RNA or NTPs were visible, we observed distinct conformational states, showing large-scale rearrangements of the P tetramer and L domains, as well as remodeling of the RNA template, nucleoside triphosphates, and nascent RNA entrances and/or exits, upon introducing RNA and NTPs. These findings highlight the dynamic structural changes probably associated with polymerase activity and advance the understanding of the BoDV-1 polymerase mechanisms, offering a basis for developing targeted antiviral strategies against this deadly pathogen.

#4

Mapping Bornavirus encephalitis-A comparative study of viral spread and immune response in human and animal dead-end hosts.

PLoS pathogens2025 Aug

Borna disease virus 1 (BoDV-1) has long been recognized as a cause of fatal encephalitis in animals and was only recently identified as a zoonotic pathogen causing a similar disease in humans. This study provides the first comprehensive comparative analysis of BoDV-1-induced neuropathology in human and animal end hosts, including horses, sheep, and alpacas. Using immunohistochemical analyses, we investigated the topographical distribution of BoDV-1 and inflammatory responses in the central nervous system across 19 cases. Key findings reveal distinct differences and overlaps between humans and animals. While humans exhibited heterogeneous patterns especially of the lymphocyte infiltration, animals displayed more species-specific inflammation and viral spread patterns. In horses, the hippocampus and basal ganglia were consistently affected, whereas sheep showed predominant involvement of the frontal cortex and stria olfactoria. Alpacas demonstrated a less uniform distribution but highlighted the brainstem and basal ganglia as critical sites. Intriguingly, across all species, a negative association was observed between lymphocyte infiltration and the number of BoDV-1-infected cells. These findings enhance our understanding of BoDV-1 pathogenesis and is a first step of cross-species comparison in unraveling disease mechanisms in BoDV-1 infection. Further research is warranted to elucidate the implications of these findings for therapeutic strategies and to explore the entry and dissemination routes of BoDV-1 in different hosts.

#5

Evaluating the potential of anti-dsRNA antibodies as an alternative viral sensing tool in encephalitides of different species.

Frontiers in veterinary science2025

Although laboratory methods have advanced, the cause of many encephalitides is still unknown. Molecular methods like multiplex PCR and microarrays are considered to be often less sensitive than Next Generation Sequencing, whereas the latter is time-consuming and costly. These analyses require appropriate tissue preparations and are more difficult to perform on formalin-fixed, paraffin-embedded (FFPE) tissues. Anti-double-stranded RNA (dsRNA) antibodies could potentially identify virus infections independently of the viral genome and can be applied to FFPE material. This study examined the applicability of monoclonal anti-dsRNA antibodies by immunohistochemistry to confirm encephalitides caused by different RNA viruses and comparing the findings with those obtained using monoclonal and polyclonal virus-specific antibodies. The viruses studied included negative-sense (Borna disease virus 1, BoDV-1; canine distemper virus, CDV; Rift Valley fever virus, RVFV) and positive-sense single stranded RNA viruses (severe acute respiratory disease syndrome coronavirus 2, SARS-CoV-2; tick-borne encephalitis virus, TBEV; Theiler's murine encephalomyelitis virus, TMEV). Interestingly, dsRNA was detected in both infected and non-infected animals and inconsistently co-localized to BoDV-1, TBEV, and TMEV antigen. Strict co-localization was lacking in CDV, SARS-CoV-2 and RVFV. Despite the co-localization of dsRNA with virus antigen for some RNA viruses, anti-dsRNA antibodies were unreliable as markers for unknown virus infections. Future studies should explore the upstream components of the immune response, including the interferon signaling cascade to assess their potential as effective virus-sensing tool.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC4 artigos no totalmostrando 86

2026

Development of a strand-specific RT-qPCR assay for detecting and quantifying Borna disease virus RNAs in infected cells.

Journal of virological methods
2025

Spillover infections by rustrela virus, borna disease virus 1 and tick-borne encephalitis virus revealed by retrospective screening of mammalian encephalitis of unknown origin.

BMC veterinary research
2025

Is vaccination a feasible public health strategy against fatal Borna disease virus 1 (BoDV-1) encephalitis? An epidemiological perspective.

PLoS pathogens
2025

First therapeutic drug monitoring of experimental favipiravir in Borna disease virus 1 (BoDV-1) encephalitis patients reveals significant gaps in antiviral treatment: a pilot investigation.

European journal of medical research
2025

Structural insights into the dynamic mechanism of bornavirus polymerase.

Proceedings of the National Academy of Sciences of the United States of America
2025

Mapping Bornavirus encephalitis-A comparative study of viral spread and immune response in human and animal dead-end hosts.

PLoS pathogens
2025

Screening for Borna disease virus 1 (BoDV-1) in Austria: Absence of human cases in a retrospective case-finding study.

Infection, genetics and evolution : journal of molecular epidemiology and evolutionary genetics in infectious diseases
2025

Highly specific serological diagnosis of Borna disease virus 1 (BoDV-1) and variegated squirrel bornavirus 1 (VSBV-1) encephalitis by novel antibody isotype assay with multiple viral antigens.

Journal of clinical virology : the official publication of the Pan American Society for Clinical Virology
2025

Experimental infection of shrews (Crocidura russula) with Borna disease virus 1: Insights into viral spread and shedding.

PNAS nexus
2025

Borna disease virus 2 maintains genomic polymorphisms by superinfection in persistently infected cells.

Npj viruses
2025

Evaluating the potential of anti-dsRNA antibodies as an alternative viral sensing tool in encephalitides of different species.

Frontiers in veterinary science
2025

Neuropathology, pathomechanism, and transmission in zoonotic Borna disease virus 1 infection: a systematic review.

The Lancet. Infectious diseases
2024

Developing a universal multi-epitope protein vaccine candidate for enhanced borna virus pandemic preparedness.

Frontiers in immunology
2024

Analysis of BoDV-1 status, EEG resting-state alpha activity and pro-inflammatory cytokines in adults with and without major depressive disorder.

Frontiers in psychology
2024

Borna disease virus 1 induces ferroptosis, contributing to lethal encephalitis.

Journal of medical virology
2024

A single amino acid substitution in the Borna disease virus glycoprotein enhances the infectivity titer of vesicular stomatitis virus pseudotyped virus by altering membrane fusion activity.

Microbiology and immunology
2024

Lethal Borna disease virus 1 infections of humans and animals - in-depth molecular epidemiology and phylogeography.

Nature communications
2025

Clinical analysis of Bornavirus Encephalitis cases demonstrates a small time window for Etiological Diagnostics and treatment attempts, a large case series from Germany 1996-2022.

Infection
2024

Comparative study of virus and lymphocyte distribution with clinical data suggests early high dose immunosuppression as potential key factor for the therapy of patients with BoDV-1 infection.

Emerging microbes &amp; infections
2024

Detection of virus-specific T cells via ELISpot corroborates early diagnosis in human Borna disease virus 1 (BoDV-1) encephalitis.

Infection
2024

Long-term Elevation of Complement Factors in Cerebrospinal Fluid of Patients With Borna Disease Virus 1 Encephalitis.

The Journal of infectious diseases
2024

Suppression of Borna Disease Virus Replication during Its Persistent Infection Using the CRISPR/Cas13b System.

International journal of molecular sciences
2024

One Health in action: Investigation of the first detected local cluster of fatal borna disease virus 1 (BoDV-1) encephalitis, Germany 2022.

Journal of clinical virology : the official publication of the Pan American Society for Clinical Virology
2024

18fluorodeoxyglucose PET/CT as possible early diagnostic tool preceding MRI changes in Borna disease virus 1 encephalitis.

Lancet (London, England)
2023

Case report: Fatal Borna virus encephalitis manifesting with basal brain and brainstem symptoms.

Frontiers in neurology
2024

HAND2 suppresses favipiravir efficacy in treatment of Borna disease virus infection.

Antiviral research
2023

Evaluation of viral infection as an etiology of ME/CFS: a systematic review and meta-analysis.

Journal of translational medicine
2023

Severe Neurologic Disease in a Horse Caused by Tick-Borne Encephalitis Virus, Austria, 2021.

Viruses
2024

No evidence of an association of multiple sclerosis (MS) with Borna disease virus 1 (BoDV-1) infections in patients within an endemic region: a retrospective pilot study.

Infection
2023

Alzheimer's disease as a viral disease: Revisiting the infectious hypothesis.

Ageing research reviews
2024

Detection of bornavirus-reactive antibodies and BoDV-1 RNA only in encephalitis patients from virus endemic areas: a comparative serological and molecular sensitivity, specificity, predictive value, and disease duration correlation study.

Infection
2023

The regulation of persistent Borna disease virus infection by RNA silencing factors in human cells.

Biochemical and biophysical research communications
2023

Ribavirin Treatment for Severe Schizophrenia with Anti-Borna Disease Virus 1 Antibodies 30 Years after Onset.

Case reports in psychiatry
2023

Structural and biophysical characterization of the Borna disease virus 1 phosphoprotein.

Acta crystallographica. Section F, Structural biology communications
2023

First detected geographical cluster of BoDV-1 encephalitis from same small village in two children: therapeutic considerations and epidemiological implications.

Infection
2023

Magnetic resonance imaging of human variegated squirrel bornavirus 1 (VSBV-1) encephalitis reveals diagnostic pattern indistinguishable from Borna disease virus 1 (BoDV-1) encephalitis but typical for bornaviruses.

Emerging microbes &amp; infections
2023

Risk factors for Borna disease virus 1 encephalitis in Germany - a case-control study.

Emerging microbes &amp; infections
2023

Cerebrospinal fluid in Borna disease virus 1 (BoDV-1) encephalitis.

Journal of the neurological sciences
2023

IFN-γ-Based ELISpot as a New Tool to Detect Human Infections with Borna Disease Virus 1 (BoDV-1): A Pilot Study.

Viruses
2023

Human Infections with Borna Disease Virus 1 (BoDV-1) Primarily Lead to Severe Encephalitis: Further Evidence from the Seroepidemiological BoSOT Study in an Endemic Region in Southern Germany.

Viruses
2022

Vaccination against Borna Disease: Overview, Vaccine Virus Characterization and Investigation of Live and Inactivated Vaccines.

Viruses
2022

Reverse Genetics and Artificial Replication Systems of Borna Disease Virus 1.

Viruses
2022

Development of a nonhuman primate model for mammalian bornavirus infection.

PNAS nexus
2022

Nonarboviral Equine Encephalitides.

The Veterinary clinics of North America. Equine practice
2022

Human Borna disease virus 1 encephalitis shows marked pro-inflammatory biomarker and tissue immunoactivation during the course of disease.

Emerging microbes &amp; infections
2022

Healthcare-associated exposure to Borna disease virus 1 (BoDV-1).

Journal of occupational medicine and toxicology (London, England)
2022

Pathogen Screening for Possible Causes of Meningitis/Encephalitis in Wild Carnivores From Saxony-Anhalt.

Frontiers in veterinary science
2022

[Erratum to: Bornavirus encephalitis as a differential diagnosis to seronegative autoimmune encephalitis].

Der Nervenarzt
2022

Molecular epidemiology of human Borna disease virus 1 infection revisited.

Emerging microbes &amp; infections
2022

Antibodies against viral nucleo-, phospho-, and X protein contribute to serological diagnosis of fatal Borna disease virus 1 infections.

Cell reports. Medicine
2022

miR-505 inhibits replication of Borna disease virus 1 via inhibition of HMGB1-mediated autophagy.

The Journal of general virology
2022

[Bornavirus encephalitis as a differential diagnosis to seronegative autoimmune encephalitis].

Der Nervenarzt
2022

Human Borna disease virus 1 (BoDV-1) encephalitis cases in the north and east of Germany.

Emerging microbes &amp; infections
2021

Tick-Borne Encephalitis Virus (TBEV) Infection in Two Horses.

Viruses
2021

Fatal encephalitis and Borna Disease Virus-1 seropositivity in two kidney-transplant patients living in the same nonendemic area.

Transplant infectious disease : an official journal of the Transplantation Society
2021

Investigation of fatal human Borna disease virus 1 encephalitis outside the previously known area for human cases, Brandenburg, Germany - a case report.

BMC infectious diseases
2021

ICTV Virus Taxonomy Profile: Bornaviridae.

The Journal of general virology
2021

Active Case Finding of Current Bornavirus Infections in Human Encephalitis Cases of Unknown Etiology, Germany, 2018-2020.

Emerging infectious diseases
2021

A theoretical and generalized approach for the assessment of the sample-specific limit of detection for clinical metagenomics.

Computational and structural biotechnology journal
2022

New World camelids are sentinels for the presence of Borna disease virus.

Transboundary and emerging diseases
2020

First isolation, in-vivo and genomic characterization of zoonotic variegated squirrel Bornavirus 1 (VSBV-1) isolates.

Emerging microbes &amp; infections
2020

The Challenge of Assessing Mild Neuroinflammation in Severe Mental Disorders.

Frontiers in psychiatry
2020

Bornavirus Encephalitis Shows a Characteristic Magnetic Resonance Phenotype in Humans.

Annals of neurology
2020

Meningoencephalomyelitis of Unknown Origin in Cats: A Case Series Describing Clinical and Pathological Findings.

Frontiers in veterinary science
2020

Are human Borna disease virus 1 infections zoonotic and fatal? - Authors' reply.

The Lancet. Infectious diseases
2020

Are human Borna disease virus 1 infections zoonotic and fatal?

The Lancet. Infectious diseases
2020

Obesity induced by Borna disease virus in rats: key roles of hypothalamic fast-acting neurotransmitters and inflammatory infiltrates.

Brain structure &amp; function
2020

Borna disease outbreak with high mortality in an alpaca herd in a previously unreported endemic area in Germany.

Transboundary and emerging diseases
2020

Antiviral treatment perspective against Borna disease virus 1 infection in major depression: a double-blind placebo-controlled randomized clinical trial.

BMC pharmacology &amp; toxicology
2020

No evidence for European bats serving as reservoir for Borna disease virus 1 or other known mammalian orthobornaviruses.

Virology journal
2020

Zoonotic spillover infections with Borna disease virus 1 leading to fatal human encephalitis, 1999-2019: an epidemiological investigation.

The Lancet. Infectious diseases
2020

Relaunching human bornavirus research from encephalitis cases with unclear cause.

The Lancet. Infectious diseases
2019

Low prevalence of Borna disease virus 1 (BoDV-1) IgG antibodies in humans from areas endemic for animal Borna disease of Southern Germany.

Scientific reports
2019

Viral Equine Encephalitis, a Growing Threat to the Horse Population in Europe?

Viruses
2019

A Small Interfering RNA Cocktail Targeting the Nucleoprotein and Large Protein Genes Suppresses Borna Disease Virus Infection.

Frontiers in microbiology
2019

The neuropathology of fatal encephalomyelitis in human Borna virus infection.

Acta neuropathologica
2019

Intranasal Borna Disease Virus (BoDV-1) Infection: Insights into Initial Steps and Potential Contagiosity.

International journal of molecular sciences
2018

Fatal Encephalitic Borna Disease Virus 1 in Solid-Organ Transplant Recipients.

The New England journal of medicine
2018

Fatal Encephalitis Associated with Borna Disease Virus 1.

The New England journal of medicine
2017

Assessing the Diversity of Rodent-Borne Viruses: Exploring of High-Throughput Sequencing and Classical Amplification/Sequencing Approaches.

Advances in virus research
2017

Infections of horses and shrews with Bornaviruses in Upper Austria: a novel endemic area of Borna disease.

Emerging microbes &amp; infections
2017

Pathological findings in the red fox (Vulpes vulpes), stone marten (Martes foina) and raccoon dog (Nyctereutes procyonoides), with special emphasis on infectious and zoonotic agents in Northern Germany.

PloS one
2016

Screening red foxes (Vulpes vulpes) for possible viral causes of encephalitis.

Virology journal
2016

The pathogenesis of bornaviral diseases in mammals.

Animal health research reviews
2015

Transolfactory neuroinvasion by viruses threatens the human brain.

Acta virologica
2015

Viral immune surveillance: Toward a TH17/TH9 gate to the central nervous system.

Bioinformation

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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.

  1. Development of a strand-specific RT-qPCR assay for detecting and quantifying Borna disease virus RNAs in infected cells.
    Journal of virological methods· 2026· PMID 41592625mais citado
  2. Is vaccination a feasible public health strategy against fatal Borna disease virus 1 (BoDV-1) encephalitis? An epidemiological perspective.
    PLoS pathogens· 2025· PMID 41082543mais citado
  3. Structural insights into the dynamic mechanism of bornavirus polymerase.
    Proceedings of the National Academy of Sciences of the United States of America· 2025· PMID 40996804mais citado
  4. Mapping Bornavirus encephalitis-A comparative study of viral spread and immune response in human and animal dead-end hosts.
    PLoS pathogens· 2025· PMID 40758738mais citado
  5. Evaluating the potential of anti-dsRNA antibodies as an alternative viral sensing tool in encephalitides of different species.
    Frontiers in veterinary science· 2025· PMID 40191085mais citado
  6. Developing a universal multi-epitope protein vaccine candidate for enhanced borna virus pandemic preparedness.
    Front Immunol· 2024· PMID 39703502recente
  7. Epigenetic Targeting for Controlling Persistent Neurotropic Infections Caused by Borna Virus and HIV.
    Rev Med Virol· 2025· PMID 39643925recente
  8. Development of an RNA virus-based episomal vector with artificial aptazyme for gene silencing.
    Appl Microbiol Biotechnol· 2024· PMID 39422780recente
  9. Research Note: Development of a reverse transcriptase recombinase-aided amplification method for detection of Parrot Borna Virus 4.
    Poult Sci· 2024· PMID 39374571recente
  10. Comparative study of virus and lymphocyte distribution with clinical data suggests early high dose immunosuppression as potential key factor for the therapy of patients with BoDV-1 infection.
    Emerg Microbes Infect· 2024· PMID 38687703recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:637051(Orphanet)
  2. MONDO:0957421(MONDO)
  3. Busca completa no PubMed(PubMed)
  4. Artigo Wikipedia(Wikipedia)

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

Encefalite Borna virus
Compêndio · Raras BR

Encefalite Borna virus

ORPHA:637051 · MONDO:0957421
Prevalência
<1 / 1 000 000
Casos
18 casos conhecidos
CID-10
A85.8 · Outras encefalites virais especificadas
CID-11
Início
Adolescent, Adult, Elderly
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C5816783
EuropePMC
Wikipedia
DiscussaoAtiva

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