Susac’s Syndrome – a case report

Detalhes bibliográficos
Autor(a) principal: Rodrigues, Ana Rita
Data de Publicação: 2024
Outros Autores: Gomes, Pedro Marques, Cunha-Cabral, Diogo, Duarte, Delfim, Oliveira, Nuno
Tipo de documento: Artigo
Idioma: eng
por
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: https://doi.org/10.34631/sporl.2096
Resumo: Susac’s Syndrome (SS) is an occlusive immune-mediated microangiopathy that preferentially affects cerebral, retinal and cochlear arterioles. Classically, it is associated with the triad of visual defects, encephalopathy and sensorineural hearing loss (SNH). Diagnosis is based on clinical features and findings from fluorescein angiography (FA), brain magnetic resonance imaging (CE-MRI) and audiometry. Treatment is based on the use of high-dose corticosteroid therapy and must be started as soon as possible to prevent permanent sequelae. The clinical case presented is that of a 34-year-old woman with multiple visits to the Emergency Department due to persistent headaches, psychomotor slowing and focal neurological deficits lasting 2 months. From the study carried out, multiple hyperintense lesions were found on T2 CE-MRI involving the corpus callosum, areas of retinal ischemia and hyperfluorescence on FA and bilateral moderate SNH at high frequencies on the audiogram. The diagnosis of SS was assumed and immunosuppressive therapy was started, maintaining clinical, imaging and audiometric surveillance.
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spelling Susac’s Syndrome – a case reportSíndrome de Susac - um caso clínicoSíndrome de SusacencefalopatiaSurdez Neurossensorialenfartes retinianosAudiometriaSusac’s SyndromeencephalopathySensorineural hearing lossretinal artery occlusionaudiometrySusac’s Syndrome (SS) is an occlusive immune-mediated microangiopathy that preferentially affects cerebral, retinal and cochlear arterioles. Classically, it is associated with the triad of visual defects, encephalopathy and sensorineural hearing loss (SNH). Diagnosis is based on clinical features and findings from fluorescein angiography (FA), brain magnetic resonance imaging (CE-MRI) and audiometry. Treatment is based on the use of high-dose corticosteroid therapy and must be started as soon as possible to prevent permanent sequelae. The clinical case presented is that of a 34-year-old woman with multiple visits to the Emergency Department due to persistent headaches, psychomotor slowing and focal neurological deficits lasting 2 months. From the study carried out, multiple hyperintense lesions were found on T2 CE-MRI involving the corpus callosum, areas of retinal ischemia and hyperfluorescence on FA and bilateral moderate SNH at high frequencies on the audiogram. The diagnosis of SS was assumed and immunosuppressive therapy was started, maintaining clinical, imaging and audiometric surveillance.A Síndrome de Susac (SS) é uma microangiopatia imunomediada oclusiva que atinge preferencialmente as arteríolas cerebrais, retinianas e cocleares. Classicamente, associa-se à tríade de defeitos visuais, encefalopatia e hipoacusia neurossensorial (HNS). O diagnóstico baseia-se na clínica e achados da angiografia fluoresceínica (AF), ressonância magnética cerebral (RMN-CE) e audiometria. O tratamento assenta na utilização de corticoterapia em altas doses e deve ser iniciado o mais precocemente possível para prevenir sequelas permanentes. O caso clínico apresentado é de uma mulher de 34 anos com múltiplas idas ao Serviço de Urgência por cefaleias persistentes, lentificação psicomotora e défices neurológicos focais com 2 meses de evolução. Do estudo efetuado, constatadas múltiplas lesões hiperintensas na RMN-CE em T2 a envolver o corpo caloso, áreas de isquemia retiniana e hiperfluorescência na AF e HNS moderada bilateral nas frequências elevadas no audiograma. Assumiu-se o diagnóstico de SS e iniciou-se terapêutica imunossupressora, mantendo-se vigilância clínica, imagiológica e audiométrica.Sociedade Portuguesa de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço2024-06-04info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfapplication/pdfhttps://doi.org/10.34631/sporl.2096https://doi.org/10.34631/sporl.2096Portuguese Journal of Otorhinolaryngology and Head and Neck Surgery; Vol. 62 No. 2 (2024): June; 159-164Revista Portuguesa de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço; Vol. 62 Núm. 2 (2024): Junho; 159-164Revista Portuguesa de Otorrinolaringologia-Cirurgia de Cabeça e Pescoço; Vol. 62 N.º 2 (2024): Junho; 159-1642184-6499reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPengporhttps://journalsporl.com/index.php/sporl/article/view/2096https://journalsporl.com/index.php/sporl/article/view/2096/1101https://journalsporl.com/index.php/sporl/article/view/2096/1102Direitos de Autor (c) 2024 Revista Portuguesa de Otorrinolaringologia-Cirurgia de Cabeça e Pescoçoinfo:eu-repo/semantics/openAccessRodrigues, Ana RitaGomes, Pedro MarquesCunha-Cabral, DiogoDuarte, DelfimOliveira, Nuno2024-06-06T12:57:10Zoai:journalsporl.com:article/2096Portal AgregadorONGhttps://www.rcaap.pt/oai/openairemluisa.alvim@gmail.comopendoar:71602024-06-06T12:57:10Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Susac’s Syndrome – a case report
Síndrome de Susac - um caso clínico
title Susac’s Syndrome – a case report
spellingShingle Susac’s Syndrome – a case report
Rodrigues, Ana Rita
Síndrome de Susac
encefalopatia
Surdez Neurossensorial
enfartes retinianos
Audiometria
Susac’s Syndrome
encephalopathy
Sensorineural hearing loss
retinal artery occlusion
audiometry
title_short Susac’s Syndrome – a case report
title_full Susac’s Syndrome – a case report
title_fullStr Susac’s Syndrome – a case report
title_full_unstemmed Susac’s Syndrome – a case report
title_sort Susac’s Syndrome – a case report
author Rodrigues, Ana Rita
author_facet Rodrigues, Ana Rita
Gomes, Pedro Marques
Cunha-Cabral, Diogo
Duarte, Delfim
Oliveira, Nuno
author_role author
author2 Gomes, Pedro Marques
Cunha-Cabral, Diogo
Duarte, Delfim
Oliveira, Nuno
author2_role author
author
author
author
dc.contributor.author.fl_str_mv Rodrigues, Ana Rita
Gomes, Pedro Marques
Cunha-Cabral, Diogo
Duarte, Delfim
Oliveira, Nuno
dc.subject.por.fl_str_mv Síndrome de Susac
encefalopatia
Surdez Neurossensorial
enfartes retinianos
Audiometria
Susac’s Syndrome
encephalopathy
Sensorineural hearing loss
retinal artery occlusion
audiometry
topic Síndrome de Susac
encefalopatia
Surdez Neurossensorial
enfartes retinianos
Audiometria
Susac’s Syndrome
encephalopathy
Sensorineural hearing loss
retinal artery occlusion
audiometry
description Susac’s Syndrome (SS) is an occlusive immune-mediated microangiopathy that preferentially affects cerebral, retinal and cochlear arterioles. Classically, it is associated with the triad of visual defects, encephalopathy and sensorineural hearing loss (SNH). Diagnosis is based on clinical features and findings from fluorescein angiography (FA), brain magnetic resonance imaging (CE-MRI) and audiometry. Treatment is based on the use of high-dose corticosteroid therapy and must be started as soon as possible to prevent permanent sequelae. The clinical case presented is that of a 34-year-old woman with multiple visits to the Emergency Department due to persistent headaches, psychomotor slowing and focal neurological deficits lasting 2 months. From the study carried out, multiple hyperintense lesions were found on T2 CE-MRI involving the corpus callosum, areas of retinal ischemia and hyperfluorescence on FA and bilateral moderate SNH at high frequencies on the audiogram. The diagnosis of SS was assumed and immunosuppressive therapy was started, maintaining clinical, imaging and audiometric surveillance.
publishDate 2024
dc.date.none.fl_str_mv 2024-06-04
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv https://doi.org/10.34631/sporl.2096
https://doi.org/10.34631/sporl.2096
url https://doi.org/10.34631/sporl.2096
dc.language.iso.fl_str_mv eng
por
language eng
por
dc.relation.none.fl_str_mv https://journalsporl.com/index.php/sporl/article/view/2096
https://journalsporl.com/index.php/sporl/article/view/2096/1101
https://journalsporl.com/index.php/sporl/article/view/2096/1102
dc.rights.driver.fl_str_mv Direitos de Autor (c) 2024 Revista Portuguesa de Otorrinolaringologia-Cirurgia de Cabeça e Pescoço
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Direitos de Autor (c) 2024 Revista Portuguesa de Otorrinolaringologia-Cirurgia de Cabeça e Pescoço
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv Sociedade Portuguesa de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço
publisher.none.fl_str_mv Sociedade Portuguesa de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço
dc.source.none.fl_str_mv Portuguese Journal of Otorhinolaryngology and Head and Neck Surgery; Vol. 62 No. 2 (2024): June; 159-164
Revista Portuguesa de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço; Vol. 62 Núm. 2 (2024): Junho; 159-164
Revista Portuguesa de Otorrinolaringologia-Cirurgia de Cabeça e Pescoço; Vol. 62 N.º 2 (2024): Junho; 159-164
2184-6499
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
repository.mail.fl_str_mv mluisa.alvim@gmail.com
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