Characteristics and management of Susac syndrome in an emergent country: a multi-center case series from Brazil

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0
Tipo de produção
article
Data de publicação
2022
DOI
Scopus
Web of Science
PubMed
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Título do Volume
Editora
SPRINGER-VERLAG ITALIA SRL
Autores
SANTIAGO, Igor Bessa
ARAUJO, Abraao Lazaro Meneses
NOBREGA, Ivna Lacerda Pereira
SILVA, Walisson Grangeiro Bringel
MENDES, Lucas Silvestre
PONTE, Jose Israel Araujo
DIAS, Daniel Aguiar
CASTRO, Jose Daniel Vieira de
CUNHA, Francisco Marcos Bezerra
SOBREIRA-NETO, Manoel Alves
Citação
NEUROLOGICAL SCIENCES, v.43, n.11, p.6449-6460, 2022
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Resumo
Background Susac syndrome (SS) is a rare endotheliopathy with an estimated prevalence of 0.14-0.024 per 100,000. It is an important differential diagnosis in demyelinating disorders. There are few case series and no large randomized controlled trials, and most reports come from developed countries. We report six cases of SS in three centers in Brazil and discuss management challenges in emergent countries. Methods This is a retrospective case series of patients diagnosed with SS in three medical centers in Brazil between April 2018 and July 2021. The European Susac consortium (EuSaC) criteria were used for diagnosis of SS. Demographic data and clinical interventions were described and outcomes were assessed subjectively and by applying the modified Rankin Scale (mRS) on last follow-up. Results Six patients were diagnosed with SS (3 males, 3 females). Mean age at presentation was 36 years (range 17 to 54). The most common initial symptom was confusion, followed by visual impairment and hearing loss. Characteristic snowball lesions on magnetic resonance imaging (MRI) were present in four patients (66%). Retinal artery abnormalities were present in half (3/6) of patients, and sensorineural hearing loss was present in four patients (66%). Outcome was favorable (mRS <= 2) in five patients (86%). Patients treated early had a more favorable outcome. Conclusion Emergent countries face challenges in the diagnosis and management of patients with SS, such as access to advanced tests (fluorescein angiography, serial MRI) and treatment drugs (rituximab, mycophenolate). Further research should consider particularities of patients with SS in emergent countries.
Palavras-chave
Susac syndrome, Demyelinating disorders, Neuroimmunology, Vasculopathy
URI
https://observatorio.fm.usp.br/handle/OPI/50828
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Coleções
Artigos e Materiais de Revistas Científicas - HC/ICHC