Susac’s Syndrome – a case report
Autor(a) principal: | |
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Data de Publicação: | 2024 |
Outros Autores: | , , , |
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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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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1817546025967026176 |