Letter to the Editor
Optical coherence tomography angiography in the early diagnosis of Susac syndrome
Angiografía por tomografía de coherencia óptica en el diagnóstico precoz del síndrome de Susac
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No pathological enhancement was observed after intravenous administration of contrast (E). Lesions do not cause a mass effect or show diffusion restriction on DWI sequences (F).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "R.N. Villar-Quiles, N. Gómez-Ruiz, M. Jorquera-Moya, J. Porta-Etessam" "autores" => array:4 [ 0 => array:2 [ "nombre" => "R.N." "apellidos" => "Villar-Quiles" ] 1 => array:2 [ "nombre" => "N." "apellidos" => "Gómez-Ruiz" ] 2 => array:2 [ "nombre" => "M." "apellidos" => "Jorquera-Moya" ] 3 => array:2 [ "nombre" => "J." "apellidos" => "Porta-Etessam" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S021348531730258X" "doi" => "10.1016/j.nrl.2017.07.012" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S021348531730258X?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173580819300690?idApp=UINPBA00004N" "url" => "/21735808/0000003500000001/v1_202004040635/S2173580819300690/v1_202004040635/en/main.assets" ] "en" => array:15 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Letter to the Editor</span>" "titulo" => "Optical coherence tomography angiography in the early diagnosis of Susac syndrome" "tieneTextoCompleto" => true "saludo" => "Dear Editor," "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "62" "paginaFinal" => "63" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "J.L. García-Serrano, J.E. Muñoz de Escalona-Rojas, J.L. Callejas-Rubio, F.J. Barrero-Hernández" "autores" => array:4 [ 0 => array:3 [ "nombre" => "J.L." "apellidos" => "García-Serrano" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 1 => array:4 [ "nombre" => "J.E." "apellidos" => "Muñoz de Escalona-Rojas" "email" => array:1 [ 0 => "jemder78@hotmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 2 => array:3 [ "nombre" => "J.L." "apellidos" => "Callejas-Rubio" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "F.J." "apellidos" => "Barrero-Hernández" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Oftalmología, Hospital Universitario San Cecilio, Granada, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Unidad de Enfermedades autoinmunes, Hospital Universitario San Cecilio, Granada, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Universitario San Cecilio, Granada, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Angiografía por tomografía de coherencia óptica en el diagnóstico precoz del síndrome de Susac" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1706 "Ancho" => 3333 "Tamanyo" => 1480574 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">OCT-A image of the right eye revealing loss of superficial and deep retinal capillaries in the inferior temporal region (ischaemic areas marked by a circle).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Susac syndrome (SS) continues to represent a diagnostic challenge since its form of presentation overlaps with those of other conditions. The syndrome is usually misdiagnosed as demyelinating diseases including multiple sclerosis or acute disseminated encephalomyelitis due to the similar clinical presentation and paraclinical findings.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Occlusion secondary to autoimmune endotheliopathy affects precapillary arterioles of the brain, retina, and inner ear (cochlea and semicircular canals),<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">2</span></a> which may cause neurological, auditory, and visual alterations; impaired visual acuity is caused by branch retinal artery occlusion. However, not all patients present this triad of symptoms.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Neurological alterations include impaired level of consciousness and cognitive and behavioural alterations, which may be accompanied by such focal neurological signs as ataxia, paresis, and dysarthria. During this stage of subacute encephalopathy, MRI studies may reveal lesions to the corpus callosum, which help diagnose the condition.</p><p id="par0025" class="elsevierStylePara elsevierViewall">MRI may be helpful for early diagnosis in some cases, revealing microinfarcts in the central region of the corpus callosum in sagittal FLAIR and T2-weighted sequences; lesions typically resemble “snowballs,” and subsequently progress to black holes and atrophy. Lesions measure 3-7<span class="elsevierStyleHsp" style=""></span>mm, suggesting occlusion of precapillaries of less than 0.1<span class="elsevierStyleHsp" style=""></span>mm (100<span class="elsevierStyleHsp" style=""></span>μm) in diameter. Microinfarcts may take the form of linear or “spoke” lesions. Diffusion sequences may also reveal hyperdense lesions to the internal capsule, resembling a “string of pearls.” The presence of these imaging findings in addition to lesions to the central region of the corpus callosum is pathognomonic of SS.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">2</span></a> However, most patients with SS show no MRI alterations.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">3</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The usefulness of MRI is therefore limited in small-vessel diseases such as SS (vessels with a diameter below 0.1<span class="elsevierStyleHsp" style=""></span>mm); alterations in small-calibre vessels of the white matter are frequently difficult to detect, even with conventional arteriography.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">4</span></a> MRI is useful for detecting occlusion, stenosis, or dilation of medium- or large-calibre vessels (>0.5<span class="elsevierStyleHsp" style=""></span>mm).<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">3</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">We present the case of a 34-year-old woman who was referred to our centre due to motor instability, tinnitus, hearing impairment, and impaired visual acuity in the right eye.</p><p id="par0045" class="elsevierStylePara elsevierViewall">A T2-weighted FLAIR sequence obtained at symptom onset revealed small, nonspecific hyperintensities in the subcortical white matter, corpus callosum, and cerebellar peduncles (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0050" class="elsevierStylePara elsevierViewall">The right eye fundus examination revealed Gass plaques and opacification, thickening, and partial occlusion of an artery in the inferior temporal region. Optical coherence tomography angiography (OCT-A) of the posterior pole revealed loss of the superficial and deep retinal capillary plexuses of the right eye, with a decrease in the number of arterial branches and areas of peripheral ischaemia in the inferior temporal region (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0055" class="elsevierStylePara elsevierViewall">OCT-A is a new imaging technique that employs motion contrast to differentiate retinal blood vessel cells from the surrounding tissue, which is static. Measuring the signal amplitude of moving structures is a safe, non-invasive method of generating eye fundus angiograms with no need for contrast agents (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>).<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">5</span></a> With lateral resolution of 20<span class="elsevierStyleHsp" style=""></span>μm and depth resolution of 2.6<span class="elsevierStyleHsp" style=""></span>μm,<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">6</span></a> OCT-A enables visualisation of macular capillaries, enabling early, non-invasive detection of retinal capillary loss. In our patient, the technique was extremely helpful in diagnosing SS before any clear ischaemic lesions to the brain parenchyma were visible on MR images.</p><p id="par0060" class="elsevierStylePara elsevierViewall">No treatment for SS has been evaluated in controlled clinical trials due to the small number of cases of the condition.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">7</span></a> Therefore, patients are usually treated with different immunomodulatory agents, with no standardised approach.</p><p id="par0070" class="elsevierStylePara elsevierViewall">High-dose corticosteroid therapy continues to be a pillar of treatment, although patients frequently require complementary treatment with intravenous immunoglobulins, mycophenolate mofetil, or cyclophosphamide.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">8</span></a> Anticoagulant, antiplatelet, and anti-vasospastic agents should also be considered.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">9</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Early diagnosis of SS is needed to provide intensive, long-term treatment, which may improve prognosis.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">2</span></a> The course of the disease is unpredictable<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">10</span></a> and no reliable biomarkers have been identified.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">7</span></a> OCT-A generates images with a mean resolution of 8-10<span class="elsevierStyleHsp" style=""></span>μm, showing ischaemic processes<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">11</span></a> in small-calibre vessels (<100<span class="elsevierStyleHsp" style=""></span>μm) of the retinal parenchyma and their progression, and complements such other imaging techniques as MRI, in which some planes may not display endothelial lesions during the early stages of the disease.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: García-Serrano JL, Muñoz de Escalona-Rojas JE, Callejas-Rubio JL, Barrero-Hernández FJ. Angiografía por tomografía de coherencia óptica en el diagnóstico precoz del síndrome de Susac. Neurología. 2020;35:62–63.</p>" ] ] "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1180 "Ancho" => 800 "Tamanyo" => 95109 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">T2-weighted FLAIR sequence (axial plane) taken at symptom onset, revealing small hyperintensities (arrows) in the subcortical and periventricular white matter.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1706 "Ancho" => 3333 "Tamanyo" => 1480574 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">OCT-A image of the right eye revealing loss of superficial and deep retinal capillaries in the inferior temporal region (ischaemic areas marked by a circle).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:11 [ 0 => array:3 [ "identificador" => "bib0060" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Update on Susac syndrome: new insights in brain and retinal imaging and treatment options" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "J. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 4 | 0 | 4 |
| 2024 October | 29 | 6 | 35 |
| 2024 September | 48 | 26 | 74 |
| 2024 August | 33 | 8 | 41 |
| 2024 July | 20 | 17 | 37 |
| 2024 June | 27 | 2 | 29 |
| 2024 May | 23 | 6 | 29 |
| 2024 April | 57 | 4 | 61 |
| 2024 March | 75 | 2 | 77 |
| 2024 February | 36 | 6 | 42 |
| 2024 January | 69 | 7 | 76 |
| 2023 December | 49 | 10 | 59 |
| 2023 November | 70 | 5 | 75 |
| 2023 October | 99 | 26 | 125 |
| 2023 September | 58 | 5 | 63 |
| 2023 August | 33 | 6 | 39 |
| 2023 July | 50 | 4 | 54 |
| 2023 June | 47 | 1 | 48 |
| 2023 May | 62 | 1 | 63 |
| 2023 April | 68 | 6 | 74 |
| 2023 March | 50 | 2 | 52 |
| 2023 February | 37 | 1 | 38 |
| 2023 January | 59 | 4 | 63 |
| 2022 December | 53 | 4 | 57 |
| 2022 November | 65 | 4 | 69 |
| 2022 October | 38 | 6 | 44 |
| 2022 September | 45 | 7 | 52 |
| 2022 August | 35 | 10 | 45 |
| 2022 July | 27 | 4 | 31 |
| 2022 June | 20 | 6 | 26 |
| 2022 May | 30 | 6 | 36 |
| 2022 April | 35 | 6 | 41 |
| 2022 March | 37 | 9 | 46 |
| 2022 February | 45 | 8 | 53 |
| 2022 January | 50 | 6 | 56 |
| 2021 December | 17 | 7 | 24 |
| 2021 November | 18 | 7 | 25 |
| 2021 October | 51 | 11 | 62 |
| 2021 September | 27 | 10 | 37 |
| 2021 August | 50 | 8 | 58 |
| 2021 July | 40 | 4 | 44 |
| 2021 June | 25 | 9 | 34 |
| 2021 May | 20 | 8 | 28 |
| 2021 April | 58 | 21 | 79 |
| 2021 March | 59 | 5 | 64 |
| 2021 February | 12 | 9 | 21 |
| 2021 January | 16 | 12 | 28 |
| 2020 December | 15 | 12 | 27 |
| 2020 November | 17 | 6 | 23 |
| 2020 October | 20 | 9 | 29 |
| 2020 September | 11 | 11 | 22 |
| 2020 August | 14 | 8 | 22 |
| 2020 July | 12 | 11 | 23 |
| 2020 June | 15 | 9 | 24 |
| 2020 May | 9 | 10 | 19 |
| 2020 April | 17 | 6 | 23 |
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