Abstract:In subacute sclerosing panencephalitis (SSPE) the persistence of measles virus (MeV) may be related to the altered immune response. In this study, cytokine responses of lymphocytes and monocytes were evaluated in SSPE compared to controls with non-inflammatory (NICON) and inflammatory (ICON) diseases. Patients with SSPE (n = 120), 78 patients with ICON and 63 patients with NICON were included in this study. Phenotypes of peripheral blood mononuclear cells (PBMC) have been analyzed by flow cytometry. CD3 and CD… Show more
“…[15] Most patients with SSPE exhibit a decreased MV-specific T helper (Th) 1 cytokine and preserved Th2 cytokine synthesis. [16] As a result of suppression of Th1 cytokine production, it was determined that peripheral blood mononuclear cells of SSPE patients showed defective interferon-gamma (IFN-γ) response to MV. [17] Higher serum interleukin-2 (IL-2) concentrations and suppressed Th2 cytokines (IL-4, IL-6 and IL-10) were also shown in patients compared to the control group.…”
Background:
Subacute sclerosing panencephalitis (SSPE) is a rare devastating complication of measles virus (MV) involving the central nervous system (CNS). SSPE occurs 4–11/100,000 cases of the measles.
Main Body:
A poor cellular immune response seems to predispose individuals to the development of SSPE. The presence of mutations that may lead to MV persistence has also been demonstrated in samples obtained from SSPE patients. However, no study to date has definitively revealed the pathogenesis of SSPE caused by persistent infection in the CNS of MV.
Conclusion:
In this review, we provide a brief overview of SSPE from both an immunological and genetic perspective. We will try to focus on the mechanisms underlying the pathogenesis of SSPE that results in MV persistence. Clarifying the pathogenesis of SSPE will enable both the expansion of therapeutic options and the prediction of disease prognosis.
“…[15] Most patients with SSPE exhibit a decreased MV-specific T helper (Th) 1 cytokine and preserved Th2 cytokine synthesis. [16] As a result of suppression of Th1 cytokine production, it was determined that peripheral blood mononuclear cells of SSPE patients showed defective interferon-gamma (IFN-γ) response to MV. [17] Higher serum interleukin-2 (IL-2) concentrations and suppressed Th2 cytokines (IL-4, IL-6 and IL-10) were also shown in patients compared to the control group.…”
Background:
Subacute sclerosing panencephalitis (SSPE) is a rare devastating complication of measles virus (MV) involving the central nervous system (CNS). SSPE occurs 4–11/100,000 cases of the measles.
Main Body:
A poor cellular immune response seems to predispose individuals to the development of SSPE. The presence of mutations that may lead to MV persistence has also been demonstrated in samples obtained from SSPE patients. However, no study to date has definitively revealed the pathogenesis of SSPE caused by persistent infection in the CNS of MV.
Conclusion:
In this review, we provide a brief overview of SSPE from both an immunological and genetic perspective. We will try to focus on the mechanisms underlying the pathogenesis of SSPE that results in MV persistence. Clarifying the pathogenesis of SSPE will enable both the expansion of therapeutic options and the prediction of disease prognosis.
“…Além disso, a PEES está associada com resposta imune deficiente do próprio indivíduo e com alterações celulares causadas pelo patógeno. 3 Tipicamente, a doença afeta crianças e adolescentes, principalmente aqueles que foram infectados pelo vírus antes de terem completado 2 anos de idade. 3,4 As mulheres são menos afetadas que os homens, e o período para a aparição dos primeiros sintomas é mais tardio nelas.…”
Section: Introductionunclassified
“…3 Tipicamente, a doença afeta crianças e adolescentes, principalmente aqueles que foram infectados pelo vírus antes de terem completado 2 anos de idade. 3,4 As mulheres são menos afetadas que os homens, e o período para a aparição dos primeiros sintomas é mais tardio nelas. 5 A incidência da doença está inversamente relacionada com a cobertura vacinal e em países desenvolvidos a prevalência tem declinado constantemente desde a introdução da vacina contra o vírus do sarampo na década de 1960.…”
Introdução: A panencefalite esclerosante subaguda é uma doença rara, progressiva, degenerativa e fatal, que afeta o Sistema Nervoso Central. É causada por uma variante genética do vírus do sarampo, acometendo, normalmente, crianças e adolescentes. Objetivos:Analisar estudos sobre a panencefalite esclerosante subaguda, com objetivo de compreender a fisiopatologia da doença e avaliar as opções de tratamentos disponíveis, bem como suas efetividades e limitações. Método: Revisão da literatura composta por artigos disponíveis nas bases de dados PubMed, SciELO e Lilacs. Os descritores foram: “Subacute sclerosing panencephalitis" e " treatment”, totalizando 39 artigos publicados entre 1989 e 2022. Resultado: Com a redução da vacinação contra o sarampo desde o início da pandemia de COVID-19 há um risco futuro de que os casos de panencefalite esclerosante subaguda aumentem. Por isso, entender os mecanismos de ação viral é de extrema importância para a descoberta de medicações eficientes contra essa doença. Dentre os tratamentos utilizados até o momento, destacam-se os imunomoduladores e os antivirais. As terapias com Isoprinosina, Aprepitanto, Ribavirina, Favipiravir e Remdesivir apresentaram bons resultados contra a doença. Conclusão: Por se tratar de uma doença fatal, mas evitável, é importante o investimento em medidas preventivas, como a vacinação, e a realização de diagnóstico e tratamento precoce. Quanto às opções terapêuticas, os imunomoduladores e antivirais são promissores, mas ainda são necessários mais estudos para confirmar as reais vantagens dos medicamentos existentes, uma vez que os trabalhos não mostraram resultados totalmente conclusivos.
The direct impact and sequelae of infections in children and adults result in significant morbidity and mortality especially when they involve the central (CNS) or peripheral nervous system (PNS). The historical understanding of the pathophysiology has been mostly focused on the direct impact of the various pathogens through neural tissue invasion. However, with the better understanding of neuroimmunology, there is a rapidly growing realization of the contribution of the innate and adaptive host immune responses in the pathogenesis of many CNS and PNS diseases.The balance between the protective and pathologic sequelae of immunity is fragile and can easily be tipped towards harm for the host. The matter of immune privilege and surveillance of the CNS/PNS compartments and the role of the blood-brain barrier (BBB) and blood nerve barrier (BNB) makes this even more complex. Our understanding of the pathogenesis of many post-infectious manifestations of various microbial agents remains elusive, especially in the diverse African setting. Our exploration and better understanding of the neuroimmunology of some of the infectious diseases that we encounter in the continent will go a long way into helping us to improve their management and therefore lessen the burden.Africa is diverse and uniquely poised because of the mix of the classic, well described, autoimmune disease entities and the specifically “tropical” conditions. This review explores the current understanding of some of the para- and post-infectious autoimmune manifestations of CNS and PNS diseases in the African context. We highlight the clinical presentations, diagnosis and treatment of these neurological disorders and underscore the knowledge gaps and perspectives for future research using disease models of conditions that we see in the continent, some of which are not uniquely African and, where relevant, include discussion of the proposed mechanisms underlying pathogen-induced autoimmunity. This review covers the following conditions as models and highlight those in which a relationship with COVID-19 infection has been reported: a) Acute Necrotizing Encephalopathy; b) Measles-associated encephalopathies; c) Human Immunodeficiency Virus (HIV) neuroimmune disorders, and particularly the difficulties associated with classical post-infectious autoimmune disorders such as the Guillain-Barré syndrome in the context of HIV and other infections. Finally, we describe NMDA-R encephalitis, which can be post-HSV encephalitis, summarise other antibody-mediated CNS diseases and describe myasthenia gravis as the classic antibody-mediated disease but with special features in Africa.
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