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

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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorSANTIAGO, Igor Bessa
dc.contributor.authorARAUJO, Abraao Lazaro Meneses
dc.contributor.authorNOBREGA, Ivna Lacerda Pereira
dc.contributor.authorSILVA, Walisson Grangeiro Bringel
dc.contributor.authorMENDES, Lucas Silvestre
dc.contributor.authorPONTE, Jose Israel Araujo
dc.contributor.authorDIAS, Daniel Aguiar
dc.contributor.authorCASTRO, Jose Daniel Vieira de
dc.contributor.authorCUNHA, Francisco Marcos Bezerra
dc.contributor.authorSOBREIRA-NETO, Manoel Alves
dc.contributor.authorBRAGA-NETO, Pedro
dc.contributor.authorMARTINS, Gabriela Joca
dc.contributor.authorARAGAO, Ricardo Evangelista Marrocos de
dc.contributor.authorSILVA, Guilherme Diogo
dc.contributor.authorNOBREGA, Paulo Ribeiro
dc.date.accessioned2022-12-21T13:30:19Z
dc.date.available2022-12-21T13:30:19Z
dc.date.issued2022
dc.description.abstractBackground 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.eng
dc.description.indexMEDLINEeng
dc.identifier.citationNEUROLOGICAL SCIENCES, v.43, n.11, p.6449-6460, 2022
dc.identifier.doi10.1007/s10072-022-06320-4
dc.identifier.eissn1590-3478
dc.identifier.issn1590-1874
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/50828
dc.language.isoeng
dc.publisherSPRINGER-VERLAG ITALIA SRLeng
dc.relation.ispartofNeurological Sciences
dc.rightsrestrictedAccesseng
dc.rights.holderCopyright SPRINGER-VERLAG ITALIA SRLeng
dc.subjectSusac syndromeeng
dc.subjectDemyelinating disorderseng
dc.subjectNeuroimmunologyeng
dc.subjectVasculopathyeng
dc.subject.otherprevalenceeng
dc.subject.wosClinical Neurologyeng
dc.subject.wosNeuroscienceseng
dc.titleCharacteristics and management of Susac syndrome in an emergent country: a multi-center case series from Brazileng
dc.typearticleeng
dc.type.categoryoriginal articleeng
dc.type.versionpublishedVersioneng
dspace.entity.typePublication
hcfmusp.author.externalSANTIAGO, Igor Bessa:Fortaleza Gen Hosp, Neurol Serv, Fortaleza, Ceara, Brazil
hcfmusp.author.externalARAUJO, Abraao Lazaro Meneses:Univ Fed Ceara, Dept Clin Med, Div Neurol, Rua Prof Rodolfo Teofilo Costa Mendes 1608, Fortaleza, Ceara, Brazil
hcfmusp.author.externalNOBREGA, Ivna Lacerda Pereira:Fortaleza Gen Hosp, Neurol Serv, Fortaleza, Ceara, Brazil
hcfmusp.author.externalSILVA, Walisson Grangeiro Bringel:Univ Fed Ceara, Dept Clin Med, Div Neurol, Rua Prof Rodolfo Teofilo Costa Mendes 1608, Fortaleza, Ceara, Brazil
hcfmusp.author.externalMENDES, Lucas Silvestre:Fortaleza Gen Hosp, Neurol Serv, Fortaleza, Ceara, Brazil
hcfmusp.author.externalPONTE, Jose Israel Araujo:Univ Fed Ceara, Div Ophthalmol, Fortaleza, Ceara, Brazil
hcfmusp.author.externalDIAS, Daniel Aguiar:Univ Fed Ceara, Div Radiol, Fortaleza, Ceara, Brazil
hcfmusp.author.externalCASTRO, Jose Daniel Vieira de:Univ Fed Ceara, Div Radiol, Fortaleza, Ceara, Brazil
hcfmusp.author.externalCUNHA, Francisco Marcos Bezerra:Univ Fed Cariri UFCA, Juazeiro Do Norte, Brazil
hcfmusp.author.externalSOBREIRA-NETO, Manoel Alves:Univ Fed Ceara, Dept Clin Med, Div Neurol, Rua Prof Rodolfo Teofilo Costa Mendes 1608, Fortaleza, Ceara, Brazil
hcfmusp.author.externalBRAGA-NETO, Pedro:Fortaleza Gen Hosp, Neurol Serv, Fortaleza, Ceara, Brazil; Univ Fed Ceara, Dept Clin Med, Div Neurol, Rua Prof Rodolfo Teofilo Costa Mendes 1608, Fortaleza, Ceara, Brazil
hcfmusp.author.externalMARTINS, Gabriela Joca:Fortaleza Gen Hosp, Neurol Serv, Fortaleza, Ceara, Brazil
hcfmusp.author.externalARAGAO, Ricardo Evangelista Marrocos de:Univ Fed Ceara, Div Ophthalmol, Fortaleza, Ceara, Brazil
hcfmusp.author.externalNOBREGA, Paulo Ribeiro:Univ Fed Ceara, Dept Clin Med, Div Neurol, Rua Prof Rodolfo Teofilo Costa Mendes 1608, Fortaleza, Ceara, Brazil
hcfmusp.citation.scopus0
hcfmusp.contributor.author-fmusphcGUILHERME DIOGO SILVA
hcfmusp.description.beginpage6449
hcfmusp.description.endpage6460
hcfmusp.description.issue11
hcfmusp.description.volume43
hcfmusp.origemWOS
hcfmusp.origem.pubmed35945382
hcfmusp.origem.scopus2-s2.0-85135822697
hcfmusp.origem.wosWOS:000837921800003
hcfmusp.publisher.cityMILANeng
hcfmusp.publisher.countryITALYeng
hcfmusp.relation.referenceAubart-Cohen F, 2007, MEDICINE, V86, P93, DOI 10.1097/MD.0b013e3180404c99eng
hcfmusp.relation.referenceCabral NL, 2009, J NEUROL NEUROSUR PS, V80, P755, DOI 10.1136/jnnp.2009.172098eng
hcfmusp.relation.referenceCosacov R., 2016, NEUROL ARGENT, V8, P122, DOI [10.1016/j.neuarg.2016.01.002, DOI 10.1016/J.NEUARG.2016.01.002]eng
hcfmusp.relation.referenceDorr J, 2013, NAT REV NEUROL, V9, P307, DOI 10.1038/nrneurol.2013.82eng
hcfmusp.relation.referenceFeigin VL, 2003, LANCET NEUROL, V2, P43, DOI 10.1016/S1474-4422(03)00266-7eng
hcfmusp.relation.referenceFeresiadou A, 2014, CASE REP NEUROL, V6, P171, DOI 10.1159/000362868eng
hcfmusp.relation.referenceFreua F, 2014, ARQ NEURO-PSIQUIAT, V72, P812, DOI 10.1590/0004-282X20140128eng
hcfmusp.relation.referenceGarcia-Carrasco M, 2014, AUTOIMMUN REV, V13, P347, DOI 10.1016/j.autrev.2014.01.038eng
hcfmusp.relation.referenceGarcia-Carrasco M, 2011, AUTOIMMUN REV, V10, P548, DOI 10.1016/j.autrev.2011.04.006eng
hcfmusp.relation.referenceGreco A, 2014, AUTOIMMUN REV, V13, P814, DOI 10.1016/j.autrev.2014.04.004eng
hcfmusp.relation.referenceJaramillo Velasquez D, 2020, Arch Soc Esp Oftalmol (Engl Ed), V95, P396, DOI 10.1016/j.oftal.2020.05.031eng
hcfmusp.relation.referenceJarius S, 2014, J NEUROINFLAMM, V11, DOI 10.1186/1742-2094-11-46eng
hcfmusp.relation.referenceKleffner I, 2016, J NEUROL NEUROSUR PS, V87, P1287, DOI 10.1136/jnnp-2016-314295eng
hcfmusp.relation.referencePapasavvas I, 2020, J OPHTHALMIC INFLAMM, V10, DOI 10.1186/s12348-020-00217-zeng
hcfmusp.relation.referenceRennebohm RM, 2020, INT J STROKE, V15, P484, DOI 10.1177/1747493017751737eng
hcfmusp.relation.referenceRivadeneira-Sotelo C, 2017, MEDWAVE, V17, DOI 10.5867/medwave.2017.07.7033eng
hcfmusp.relation.referenceSarbu N, 2016, RADIOGRAPHICS, V36, P1426, DOI 10.1148/rg.2016160031eng
hcfmusp.relation.referenceSkacel M, 2000, ARQ NEURO-PSIQUIAT, V58, P1128, DOI 10.1590/S0004-282X2000000600024eng
hcfmusp.relation.referenceSusac JO, 2003, NEUROLOGY, V61, P1783, DOI 10.1212/01.WNL.0000103880.29693.48eng
hcfmusp.relation.referenceSUSAC JO, 1979, NEUROLOGY, V29, P313, DOI 10.1212/WNL.29.3.313eng
hcfmusp.relation.referenceTitulaer MJ, 2013, LANCET NEUROL, V12, P157, DOI 10.1016/S1474-4422(12)70310-1eng
hcfmusp.relation.referenceVishnevskia-Dai V, 2016, MEDICINE, V95, DOI 10.1097/MD.0000000000005223eng
hcfmusp.relation.referenceWilf-Yarkoni A, 2020, BMC NEUROL, V20, DOI 10.1186/s12883-020-01892-0eng
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