Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSPSANTIAGO, Igor BessaARAUJO, Abraao Lazaro MenesesNOBREGA, Ivna Lacerda PereiraSILVA, Walisson Grangeiro BringelMENDES, Lucas SilvestrePONTE, Jose Israel AraujoDIAS, Daniel AguiarCASTRO, Jose Daniel Vieira deCUNHA, Francisco Marcos BezerraSOBREIRA-NETO, Manoel AlvesBRAGA-NETO, PedroMARTINS, Gabriela JocaARAGAO, Ricardo Evangelista Marrocos deSILVA, Guilherme DiogoNOBREGA, Paulo Ribeiro2022-12-212022-12-212022NEUROLOGICAL SCIENCES, v.43, n.11, p.6449-6460, 20221590-1874https://observatorio.fm.usp.br/handle/OPI/50828Background 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.engrestrictedAccessSusac syndromeDemyelinating disordersNeuroimmunologyVasculopathyprevalencearticleCopyright SPRINGER-VERLAG ITALIA SRL10.1007/s10072-022-06320-4Clinical NeurologyNeurosciences1590-3478