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Pesquisa : C09.218.855 [Categoria DeCS]
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[PMID]:28613358
[Au] Autor:Spiess K; Martínez JRG
[Ti] Título:OCT Angiography: Assessment of Retinal Ischemia in Susac's Syndrome.
[So] Source:Ophthalmic Surg Lasers Imaging Retina;48(6):505-508, 2017 Jun 01.
[Is] ISSN:2325-8179
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Descriptive study of two patients affected by Susac's syndrome (SuS) focusing on the chronic retinal tissue damage assessed by optical coherence tomography (OCT) and OCT angiography (OCTA). To the authors' knowledge, this is the first case series reporting OCTA in SuS. OCTA imaging revealed secondary hypoxia due to branch retinal artery occlusion of the deep capillary plexus, with additional involvement of the superficial capillary network in one case. OCTA is a noninvasive and quick diagnostic tool for detecting hypoxic retinal damage in SuS. With further development of OCTA, as wide-field imaging, a better understanding of the retinal and choroidal blood supply will be gained. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:505-508.].
[Mh] Termos MeSH primário: Angiofluoresceinografia/métodos
Isquemia/diagnóstico
Retina/patologia
Doenças Retinianas/diagnóstico
Síndrome de Susac/complicações
Tomografia de Coerência Óptica/métodos
[Mh] Termos MeSH secundário: Adulto
Feminino
Fundo de Olho
Seres Humanos
Isquemia/etiologia
Masculino
Meia-Idade
Doenças Retinianas/etiologia
Síndrome de Susac/diagnóstico
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170913
[Lr] Data última revisão:
170913
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170615
[St] Status:MEDLINE
[do] DOI:10.3928/23258160-20170601-10


  2 / 128 MEDLINE  
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[PMID]:28094021
[Au] Autor:Roessler-Górecka M; Mendel T; Wisniowska J; Seniów J
[Ad] Endereço:2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland. Electronic address: roessler@ipin.edu.pl.
[Ti] Título:Neuropsychological characteristics of encephalopathy in Susac's Syndrome - Case report.
[So] Source:Neurol Neurochir Pol;51(2):174-179, 2017 Mar - Apr.
[Is] ISSN:0028-3843
[Cp] País de publicação:Poland
[La] Idioma:eng
[Ab] Resumo:Susac's Syndrome (SS) is a rare, autoimmune angiopathy characterized by hearing loss, retinal artery occlusions and encephalopathy, which is usually expressed in multifocal neurological signs and symptoms, confusion state and cognitive impairment. There have been few descriptions of neuropsychological assessment of SS. We present a case study of 29-year-old woman who developed full SS. During the post-acute stage of disease, she was admitted to neurorehabilitation ward to improve her cognitive-behavioral and motor functioning. The initial assessment revealed attention, memory and executive dysfunctions, as well as behavioral changes including impulsivity, affective dysregulation and reduced self-awareness of disease deficits. After five weeks recovery process supported by rehabilitation program, improvement was observed, although some cognitive-behavioral deficits were still present in the follow-up assessment.
[Mh] Termos MeSH primário: Encefalopatias/diagnóstico
Testes Neuropsicológicos/estatística & dados numéricos
Psicometria/estatística & dados numéricos
Síndrome de Susac/diagnóstico
[Mh] Termos MeSH secundário: Adulto
Corpo Caloso/patologia
Diagnóstico Diferencial
Feminino
Seguimentos
Seres Humanos
Cápsula Interna/patologia
Imagem por Ressonância Magnética
Exame Neurológico
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170118
[St] Status:MEDLINE


  3 / 128 MEDLINE  
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[PMID]:27989422
[Au] Autor:Hung J; Leuillette F; Stekelorom T; Devos P; Moritz F
[Ad] Endereço:Service d'ophtalmologie, centre hospitalier de Boulogne-sur-Mer, 33, rue Jacques-Monod, 62200 Boulogne-sur-Mer, France. Electronic address: hung.jacques@gmail.com.
[Ti] Título:[A typical case of Susac's syndrome].
[Ti] Título:Syndrome de Susac typique..
[So] Source:J Fr Ophtalmol;40(1):e15-e17, 2017 Jan.
[Is] ISSN:1773-0597
[Cp] País de publicação:France
[La] Idioma:fre
[Mh] Termos MeSH primário: Síndrome de Susac/diagnóstico
[Mh] Termos MeSH secundário: Feminino
Angiofluoresceinografia
Seres Humanos
Imagem por Ressonância Magnética
Oclusão da Artéria Retiniana/diagnóstico
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; LETTER
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170907
[Lr] Data última revisão:
170907
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161220
[St] Status:MEDLINE


  4 / 128 MEDLINE  
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[PMID]:27984352
[Au] Autor:Vodopivec I; Prasad S
[Ad] Endereço:Department of Neurology (IV, SP), Brigham and Women's Hospital, Boston, MA; and Department of Neurology (IV, SP), Harvard Medical School, Boston, MA.
[Ti] Título:Short Follow-up Bias Confounds Estimates of the "Typical" Clinical Course of Susac Syndrome.
[So] Source:J Neuroophthalmol;37(2):149-153, 2017 Jun.
[Is] ISSN:1536-5166
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: To evaluate the validity of the prevailing concept that Susac syndrome (SS), a rare microvasculopathy of the brain, retina, and inner ear, is a self-limiting disease. METHODS: We performed a literature search to identify all cases of SS reported between 1973 and October 2015. If available, we determined their demographics, duration of follow-up, and the clinical course that was labeled as monocyclic or polycyclic. We attempted to determine the number of relapses and the relapse rate in patients with polycyclic disease. RESULTS: Our literature search yielded 185 relevant publications reporting 405 cases of SS. The duration of follow-up could be determined in 247/405 cases, with a range 0.5-312 months. The mean was 41 months but the distribution was skewed, with a median of 24 months. Defining the clinical course as monocyclic or polycyclic was possible in 102 patients who were followed for greater than 24 months; 53 were identified as having a polycyclic course. Patients labeled polycyclic were followed longer than those labeled monocyclic (median 62 vs 42 months, P < 0.001). The number or frequency of attacks per patient could not be determined. CONCLUSIONS: The follow-up of published cases of SS is short, creating an inherent bias toward the impression that the disease is self-limiting. Our findings suggest that stratification of SS into monocyclic, polycyclic, and chronic continuous courses may oversimplify the phenotype of SS; instead, the possibility of a relapsing-remitting course must be considered in all patients with this disorder.
[Mh] Termos MeSH primário: Angiofluoresceinografia/métodos
Síndrome de Susac/diagnóstico
Tomografia de Coerência Óptica/métodos
[Mh] Termos MeSH secundário: Encéfalo/patologia
Seguimentos
Fundo de Olho
Seres Humanos
Recidiva
Retina/patologia
Fatores de Tempo
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170913
[Lr] Data última revisão:
170913
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161217
[St] Status:MEDLINE
[do] DOI:10.1097/WNO.0000000000000472


  5 / 128 MEDLINE  
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[PMID]:27127902
[Au] Autor:Kumar K; Jariwala B; Raj P; Agarwal A
[Ad] Endereço:a Dr. Agarwal's Retina Foundation and Dr. Agarwal's Eye Hospital , Chennai , India.
[Ti] Título:Retinal Neovascularisation in Susac Syndrome: A Rare Complication.
[So] Source:Semin Ophthalmol;32(4):492-495, 2017.
[Is] ISSN:1744-5205
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:PURPOSE: To report a case of Susac syndrome with ocular complications in the chronic stage of the disease. METHODS: Case report. RESULTS: A 37-year-old male patient of Indian origin presented with painless blurring of vision in the right eye. Ophthalmoscopic examination revealed vitreous hemorrhage in the right eye and NVE in both eyes well-documented angiographically. Old medical records revealed a previous attack of bilateral BRAO as a complete triad of Susac syndrome 10 years before, with an uncomplicated clinical course. Panretinal photocoagulation helped in stabilization of the condition. CONCLUSION: Ocular neovascularisation like NVE with vitreous hemorrhage can be a late manifestation of occlusive arteriolitis in Susac syndrome.
[Mh] Termos MeSH primário: Fotocoagulação a Laser/métodos
Neovascularização Retiniana/etiologia
Síndrome de Susac/complicações
[Mh] Termos MeSH secundário: Adulto
Angiofluoresceinografia
Fundo de Olho
Seres Humanos
Imagem por Ressonância Magnética
Masculino
Oftalmoscopia
Neovascularização Retiniana/diagnóstico
Neovascularização Retiniana/cirurgia
Síndrome de Susac/diagnóstico
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171003
[Lr] Data última revisão:
171003
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160430
[St] Status:MEDLINE
[do] DOI:10.3109/08820538.2015.1122068


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[PMID]:27089010
[Au] Autor:Agarwal A; Soliman M; Sarwar S; Sadiq MA; Do DV; Nguyen QD; Sepah YJ
[Ad] Endereço:Ocular Imaging Research and Reading Center, Stanley M. Truhlsen Eye Institute, University of Nebraska Medical Center, Omaha, Nebraska.
[Ti] Título:SPECTRAL-DOMAIN OPTICAL COHERENCE TOMOGRAPHY EVALUATION OF RETINAL STRUCTURE IN PATIENTS WITH SUSACS SYNDROME.
[So] Source:Retin Cases Brief Rep;11(2):123-125, 2017 Spring.
[Is] ISSN:1937-1578
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE: To report spectral-domain optical coherence tomography (SD-OCT) features in patients diagnosed with Susacs syndrome. METHODS: Clinical report of two cases. RESULTS: Spectral-domain optical coherence tomography was performed in two patients diagnosed with Susacs syndrome. Both the patients had normal macular perfusion on fluorescein angiography (FA). However, SD-OCT revealed bilateral, temporal macular atrophy with disorganization and thinning of the retinal layers. The outer plexiform layer showed nodularity and waviness suggestive of ischemic swelling of the bipolar cells. CONCLUSION: Retinal structural changes in Susacs syndrome have not been described earlier. Spectral-domain optical coherence tomography may be more sensitive than fluorescein angiography in detecting microstructural retinal alterations in various layers, especially in apparently perfused retina. These findings may provide an insight into the pathogenesis of Susacs syndrome.
[Mh] Termos MeSH primário: Doenças Retinianas/diagnóstico por imagem
Síndrome de Susac/diagnóstico por imagem
Tomografia de Coerência Óptica/métodos
[Mh] Termos MeSH secundário: Adulto
Feminino
Seres Humanos
Masculino
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170918
[Lr] Data última revisão:
170918
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160419
[St] Status:MEDLINE
[do] DOI:10.1097/ICB.0000000000000308


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[PMID]:28103199
[Au] Autor:Kleffner I; Dörr J; Ringelstein M; Gross CC; Böckenfeld Y; Schwindt W; Sundermann B; Lohmann H; Wersching H; Promesberger J; von Königsmarck N; Alex A; Guthoff R; Frijns CJ; Kappelle LJ; Jarius S; Wildemann B; Aktas O; Paul F; Wiendl H; Duning T; European Susac Consortium (EuSaC)
[Ad] Endereço:Department of Neurology, University Hospital of Münster, Münster, Germany.
[Ti] Título:Diagnostic criteria for Susac syndrome.
[So] Source:J Neurol Neurosurg Psychiatry;87(12):1287-1295, 2016 Dec.
[Is] ISSN:1468-330X
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Susac syndrome is characterised by the triad of encephalopathy with or without focal neurological signs, branch retinal artery occlusions and hearing loss. Establishment of the diagnosis is often delayed because the triad is complete only in a minority of patients at disease onset. This leads to a critical delay in the initiation of appropriate treatment. Our objective was to establish criteria for diagnosis of either definite or probable Susac syndrome. METHOD: The establishment of diagnostic criteria was based on the following three steps: (1) Definition of a reference group of 32 patients with an unambiguous diagnosis of Susac syndrome as assessed by all interdisciplinary experts of the European Susac Consortium (EuSaC) team (EuSaC cohort); (2) selection of diagnostic criteria, based on common clinical and paraclinical findings in the EuSaC cohort and on a review of the literature; and (3) validation of the proposed criteria in the previously published cohort of all Susac cases reported until 2012. RESULTS: Integrating the clinical presentation and paraclinical findings, we propose formal criteria and recommend a diagnostic workup to facilitate the diagnosis of Susac syndrome. More than 90% of the cases in the literature fulfilled the proposed criteria for probable or definite Susac syndrome. We surmise that more patients could have been diagnosed with the recommended diagnostic workup. CONCLUSIONS: We propose diagnostic criteria for Susac syndrome that may help both experts and physicians not familiar with Susac syndrome to make a correct diagnosis and to prevent delayed treatment initiation.
[Mh] Termos MeSH primário: Síndrome de Susac/diagnóstico
[Mh] Termos MeSH secundário: Adolescente
Adulto
Estudos de Coortes
Diagnóstico Tardio
Diagnóstico Diferencial
Intervenção Médica Precoce
Feminino
Fidelidade a Diretrizes
Seres Humanos
Comunicação Interdisciplinar
Colaboração Intersetorial
Masculino
Meia-Idade
Valores de Referência
Síndrome de Susac/terapia
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW; VALIDATION STUDIES
[Em] Mês de entrada:1705
[Cu] Atualização por classe:171114
[Lr] Data última revisão:
171114
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170120
[St] Status:MEDLINE
[do] DOI:10.1136/jnnp-2016-314295


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[PMID]:27853020
[Au] Autor:Entezari M; Karimi S; Feizi M
[Ad] Endereço:Department of Ophthalmology, Ophthalmic Research Center, Torfeh Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
[Ti] Título:Progressive Susac syndrome with bilateral visual loss and disability.
[So] Source:Indian J Ophthalmol;64(9):678-680, 2016 Sep.
[Is] ISSN:1998-3689
[Cp] País de publicação:India
[La] Idioma:eng
[Ab] Resumo:Susac syndrome (SS) is a rare retinal-cochlear-cerebral disease with an unclear etiology. A 35-year-old man presented with sudden painless vision loss in the right eye and 2 months later in the left eye with hemiparesis, behavioral changes, and hearing loss. Ophthalmic examinations revealed multiple branch retinal artery occlusions (BRAOs) in both eyes. Brain magnetic resonance imaging showed inflammatory changes with multiple "punched-out" lesions in the corpus callosum which confirmed the diagnosis of SS. Despite intravenous and oral corticosteroid therapy, the disease progressed with the development of new BRAOs, low vision in both eyes, and disability. Prompt diagnosis and early treatment may save the vision and even patient's life.
[Mh] Termos MeSH primário: Síndrome de Susac/diagnóstico
Baixa Visão/diagnóstico
Pessoas com Deficiência Visual
[Mh] Termos MeSH secundário: Administração Oral
Adulto
Avaliação da Deficiência
Progressão da Doença
Combinação de Medicamentos
Angiofluoresceinografia
Glucocorticoides/uso terapêutico
Seres Humanos
Injeções Intravenosas
Imagem por Ressonância Magnética
Masculino
Metilprednisolona/uso terapêutico
Prednisolona/uso terapêutico
Oclusão da Artéria Retiniana/diagnóstico
Oclusão da Artéria Retiniana/tratamento farmacológico
Síndrome de Susac/tratamento farmacológico
Baixa Visão/tratamento farmacológico
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Drug Combinations); 0 (Glucocorticoids); 9PHQ9Y1OLM (Prednisolone); X4W7ZR7023 (Methylprednisolone)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161118
[St] Status:MEDLINE
[do] DOI:10.4103/0301-4738.194334


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[PMID]:27787385
[Au] Autor:Vishnevskia-Dai V; Chapman J; Sheinfeld R; Sharon T; Huna-Baron R; Manor RS; Shoenfeld Y; Zloto O
[Ad] Endereço:aGoldschleger Eye Institute bDepartment of Neurology, Sagol Neuroscience Center, Tel Hashomer cDepartment of Ophthalmology, Sheba Medical Center, Tel Hshomer dZabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Hashomer, are affiliated to the Sackler faculty of medicine Tel Aviv University, Israel.
[Ti] Título:Susac syndrome: clinical characteristics, clinical classification, and long-term prognosis.
[So] Source:Medicine (Baltimore);95(43):e5223, 2016 Oct.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Susac syndrome is a rare condition characterized by the clinical triad of central nervous system (CNS) dysfunction, sensorineural hearing impairment, and branch retinal artery occlusion (BRAO). The purpose of this study is to examine the demographics, clinical characteristics, treatment, and long-term prognosis of Susac syndrome. The data recorded for all Susac syndrome patients treated at the Sheba Medical Center between 1998 and 2014 included demographics, clinical signs at presentation and during the disease course, imaging findings, treatment, and prognosis.Susac syndrome was diagnosed in 10 patients (age range 30-45 years). Only 2 patients presented with the full triad and 7 patients developed the full triad during mean follow-up period of 35 months. The average time to full triad was 7 months. Based on our observations at presentation, we divided the disease course into suspected, incomplete, and complete Susac syndrome. All 10 patients were treated at diagnosis with a pulse of high-dose intravenous methylprednisolone. There was improvement in visual acuity and visual field at the end of follow-up compared to baseline, but it was not statistically significant (P = 0.479 and P = 0.053, respectively). Five patients remained with neurological damage, and 5 patients had no improvement of their hearing loss at study closure. In conclusion, Susac syndrome is a rare condition that can mimic other disorders. The diagnosis is challenging because most patients do not initially present with the definitive triad. We suggest a clinical classification for the syndrome that may assist in early diagnosis.
[Mh] Termos MeSH primário: Encéfalo/diagnóstico por imagem
Previsões
Metilprednisolona/administração & dosagem
Síndrome de Susac/classificação
[Mh] Termos MeSH secundário: Adulto
Progressão da Doença
Relação Dose-Resposta a Droga
Olho/diagnóstico por imagem
Feminino
Seguimentos
Glucocorticoides/administração & dosagem
Seres Humanos
Injeções Intravenosas
Imagem por Ressonância Magnética
Masculino
Meia-Idade
Prognóstico
Estudos Retrospectivos
Síndrome de Susac/diagnóstico
Síndrome de Susac/tratamento farmacológico
[Pt] Tipo de publicação:JOURNAL ARTICLE; OBSERVATIONAL STUDY
[Nm] Nome de substância:
0 (Glucocorticoids); X4W7ZR7023 (Methylprednisolone)
[Em] Mês de entrada:1702
[Cu] Atualização por classe:170224
[Lr] Data última revisão:
170224
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:161028
[St] Status:MEDLINE


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[PMID]:27781269
[Au] Autor:Kowacs F; Ferreira Gomes M; Pigozzo T; Rodrigues Palmeira AL; Muratt Carpenedo C; Castro Ribeiro M
[Ad] Endereço:Depto. de Clínica Médica/Neurologia, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil.
[Ti] Título:Migraine-Like Headache as Presentation Symptom in Susac Syndrome.
[So] Source:Headache;56(10):1667-1669, 2016 Nov.
[Is] ISSN:1526-4610
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Cefaleia/complicações
Transtornos de Enxaqueca/complicações
Síndrome de Susac/fisiopatologia
[Mh] Termos MeSH secundário: Progressão da Doença
Feminino
Cefaleia/diagnóstico por imagem
Seres Humanos
Imagem por Ressonância Magnética
Transtornos de Enxaqueca/diagnóstico por imagem
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1704
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161027
[St] Status:MEDLINE
[do] DOI:10.1111/head.12991



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