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Herrero Suárez, Jorge Peña Suárez, Luis Caminal-Montero" "autores" => array:4 [ 0 => array:4 [ "nombre" => "Lucía" "apellidos" => "Suárez-Pérez" "email" => array:1 [ 0 => "luciasuarezp27@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Sara E." "apellidos" => "Herrero Suárez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "Jorge" "apellidos" => "Peña Suárez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] 3 => array:3 [ "nombre" => "Luis" "apellidos" => "Caminal-Montero" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Unidad de Enfermedades Autoinmunes Sistémicas, Unidad de Gestión Clínica de Medicina Interna, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Servicio de Radiología, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome confusional y vasculitis retiniana" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 669 "Ancho" => 900 "Tamanyo" => 42884 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Fluorescein angiography Ischaemia in temporal area, arterial occlusion, and active vasculitis in the nasal pole.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 33-year-old male consulting due to behavioural disorders, aphasia, severe occipital headache, unstable gait, and hearing loss. He denied substance abuse.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The following were highlighted on examination: absence of meningeal signs, sparse language with bradylalia, bradypsychia, amnesia and temporary disorientation.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Complementary studies: haemogram, general biochemistry, C reactive protein and coagulation, normal. Lumbar puncture showed hyperproteinorrhoea, with Gram, panbacterial and myobacterial negative PCR. Lues serologies, HIV and neurotropic virus, as well as ANA, ANCA and HLA-B5 were negative. A video-EEG showed signs of mild diffuse encephalopathy and cranial CT was normal. Cranial MRI showed multiple supra-and intratentorial punctiform lesions in leptomeninges and “snowball” corpus callosum lesions (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Treatment with steroids was initiated (initial dose: methylprednisolone 1 g/24 h/iv/for 7 days, later continued with 60 mg/24 h oral prednisone in a decreasing regimen) despite which he developed loss of visual acuity in both eyes, and ischaemic vascular occlusions were found on fluorescein angiography (FAG) (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). In addition, audiometry showed bilateral sensory hearing loss (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Susac syndrome (SS) was diagnosed given the clinical triad of encephalopathy, sensorineural hearing loss and retinal vasculitis with arterial occlusions. SS is an immune-mediated, pauci-inflammatory occlusive microvascular endotheliopathy that affects the brain, the retina, and the inner ear, first described in 1979.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> Characteristic are snowball-shaped T2-FLAIR lesions on MRI at the level of the corpus callosum and occlusive retinal vasculitis on FAG.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The patient was treated with steroid boluses, rituximab, and acetylsalicylic acid and later, as there was no improvement, with immunoglobulins and mycophenolate,<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> resulting in stabilisation of symptoms and then progressive reduction of steroid dose to 7.5 mg/24 h of prednisone after 9 months.</p><span id="sec1040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect1100">Funding</span><p id="par1180" class="elsevierStylePara elsevierViewall">There was no source of funding.</p></span><span id="sec2040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect2100">Conflict of interests</span><p id="par2180" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflict of interests.</p></span><span id="sec3040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect3100">Acknowledgment</span><p id="par3180" class="elsevierStylePara elsevierViewall">In gratitude to Dra. Miriam García Fernández from the Service of Ophthalmology of the Hospital Universitario Central of Asturias for the AFG images.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec1040" "titulo" => "Funding" ] 1 => array:2 [ "identificador" => "sec2040" "titulo" => "Conflict of interests" ] 2 => array:2 [ "identificador" => "sec3040" "titulo" => "Acknowledgment" ] 3 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2019-02-28" "fechaAceptado" => "2019-05-24" "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Suárez-Pérez L, Herrero Suárez SE, Peña Suárez J, Caminal-Montero L. Síndrome confusional y vasculitis retiniana. Reumatol Clin. 2021;17:52–54.</p>" ] ] "multimedia" => array:3 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2131 "Ancho" => 1733 "Tamanyo" => 249865 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Brain MRI: 1: Axial. Diffusion with hyperintense punctiform lesions in relation to microinfarcts. 2: Sagittal, T2 Flair and T1 sagittal. Characteristic involvement of the corpus callosum. 3: Axial T2 Flair with contrast. hypersignal and leptomeningeal enhancement (additive signal T1 contrast + T2). 4: Axial T1 contrast with “military” uptake pattern.</p>" ] ] 1 => array:8 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 669 "Ancho" => 900 "Tamanyo" => 42884 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Fluorescein angiography Ischaemia in temporal area, arterial occlusion, and active vasculitis in the nasal pole.</p>" ] ] 2 => array:8 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1519 "Ancho" => 1508 "Tamanyo" => 172125 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0015" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Audiometry: Tonal audiometry showing a drop in both air and bone conduction, a sign of sensorineural hearing loss.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Susac’s syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "J.O. 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