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array:24 [ "pii" => "S2173579417302268" "issn" => "21735794" "doi" => "10.1016/j.oftale.2017.12.002" "estado" => "S300" "fechaPublicacion" => "2018-03-01" "aid" => "1254" "copyright" => "Sociedad Española de Oftalmología" "copyrightAnyo" => "2017" "documento" => "article" "crossmark" => 1 "subdocumento" => "ssu" "cita" => "Arch Soc Esp Oftalmol. 2018;93:126-35" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "Traduccion" => array:1 [ "es" => array:19 [ "pii" => "S0365669117302939" "issn" => "03656691" "doi" => "10.1016/j.oftal.2017.10.006" "estado" => "S300" "fechaPublicacion" => "2018-03-01" "aid" => "1254" "copyright" => "Sociedad Española de Oftalmología" "documento" => "article" "crossmark" => 1 "subdocumento" => "ssu" "cita" => "Arch Soc Esp Oftalmol. 2018;93:126-35" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 963 "formatos" => array:2 [ "HTML" => 48 "PDF" => 915 ] ] "es" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Revisión</span>" "titulo" => "La utilidad diagnóstica del electrorretinograma negativo" "tienePdf" => "es" "tieneTextoCompleto" => "es" "tieneResumen" => array:2 [ 0 => "es" 1 => "en" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "126" "paginaFinal" => "135" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "The diagnostic usefulness of the negative electroretinogram" ] ] "contieneResumen" => array:2 [ "es" => true "en" => true ] "contieneTextoCompleto" => array:1 [ "es" => true ] "contienePdf" => array:1 [ "es" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figura 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2607 "Ancho" => 2974 "Tamanyo" => 885079 ] ] "descripcion" => array:1 [ "es" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">A. Electrorretinograma de campo completo en condiciones escotópicas mostrando la respuesta combinada de conos y bastones en un individuo sano. Se observa una mayor amplitud de la onda b en comparación con la onda a. B. Electrorretinograma de campo completo en condiciones escotópicas mostrando la respuesta combinada de conos y bastones en una paciente con enfermedad de Oguchi. Se observa una menor amplitud de la onda b en comparación con la onda a (respuesta negativa o electrorretinograma negativo).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "C. Fuente García, J.J. González-López, F.J. Muñoz-Negrete, G. Rebolleda" "autores" => array:4 [ 0 => array:2 [ "nombre" => "C." "apellidos" => "Fuente García" ] 1 => array:2 [ "nombre" => "J.J." "apellidos" => "González-López" ] 2 => array:2 [ "nombre" => "F.J." "apellidos" => "Muñoz-Negrete" ] 3 => array:2 [ "nombre" => "G." "apellidos" => "Rebolleda" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2173579417302268" "doi" => "10.1016/j.oftale.2017.12.002" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173579417302268?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0365669117302939?idApp=UINPBA00004N" "url" => "/03656691/0000009300000003/v1_201802240423/S0365669117302939/v1_201802240423/es/main.assets" ] ] "itemSiguiente" => array:19 [ "pii" => "S2173579417302232" "issn" => "21735794" "doi" => "10.1016/j.oftale.2017.08.001" "estado" => "S300" "fechaPublicacion" => "2018-03-01" "aid" => "1236" "copyright" => "Sociedad Española de Oftalmología" "documento" => "article" "crossmark" => 1 "subdocumento" => "fla" "cita" => "Arch Soc Esp Oftalmol. 2018;93:136-8" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Short communication</span>" "titulo" => "Multifocal Best's disease: The importance of genetic tests" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "es" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "136" "paginaFinal" => "138" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Enfermedad de Best multifocal: la importancia de los test genéticos" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 681 "Ancho" => 900 "Tamanyo" => 54558 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Right eye retinography showing yellowish lesions in the macula.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "C. Alba Linero, M. Rodríguez Calvo de Mora, M. España Contreras, C. Hernando Ayala" "autores" => array:4 [ 0 => array:2 [ "nombre" => "C." "apellidos" => "Alba Linero" ] 1 => array:2 [ "nombre" => "M." "apellidos" => "Rodríguez Calvo de Mora" ] 2 => array:2 [ "nombre" => "M." "apellidos" => "España Contreras" ] 3 => array:2 [ "nombre" => "C." "apellidos" => "Hernando Ayala" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0365669117302757" "doi" => "10.1016/j.oftal.2017.08.003" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0365669117302757?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173579417302232?idApp=UINPBA00004N" "url" => "/21735794/0000009300000003/v1_201803010437/S2173579417302232/v1_201803010437/en/main.assets" ] "itemAnterior" => array:19 [ "pii" => "S2173579417301317" "issn" => "21735794" "doi" => "10.1016/j.oftale.2017.06.004" "estado" => "S300" "fechaPublicacion" => "2018-03-01" "aid" => "1192" "copyright" => "Sociedad Española de Oftalmología" "documento" => "article" "crossmark" => 1 "subdocumento" => "fla" "cita" => "Arch Soc Esp Oftalmol. 2018;93:119-25" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Original article</span>" "titulo" => "Next generation sequencing in the diagnosis of Stargardt's disease" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "es" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "119" "paginaFinal" => "125" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "La secuenciación masiva (NGS) como método diagnóstico en la enfermedad de Stargardt" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1218 "Ancho" => 1650 "Tamanyo" => 155349 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">(A and B) Case 1. (C and D) Case 2. Autofluorescense of both cases suggesting bull's-eye maculopathy and absence of flecks.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "B. Jimenez-Rolando, S. Noval, I. Rosa-Perez, E. Mata Diaz, A. del Pozo, C. Ibañez, J.C. Silla, V.E.F. Montaño, R. Martin-Arenas, E. Vallespin" "autores" => array:10 [ 0 => array:2 [ "nombre" => "B." "apellidos" => "Jimenez-Rolando" ] 1 => array:2 [ "nombre" => "S." "apellidos" => "Noval" ] 2 => array:2 [ "nombre" => "I." "apellidos" => "Rosa-Perez" ] 3 => array:2 [ "nombre" => "E." "apellidos" => "Mata Diaz" ] 4 => array:2 [ "nombre" => "A." "apellidos" => "del Pozo" ] 5 => array:2 [ "nombre" => "C." "apellidos" => "Ibañez" ] 6 => array:2 [ "nombre" => "J.C." "apellidos" => "Silla" ] 7 => array:2 [ "nombre" => "V.E.F." "apellidos" => "Montaño" ] 8 => array:2 [ "nombre" => "R." "apellidos" => "Martin-Arenas" ] 9 => array:2 [ "nombre" => "E." "apellidos" => "Vallespin" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0365669117301338" "doi" => "10.1016/j.oftal.2017.03.012" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0365669117301338?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173579417301317?idApp=UINPBA00004N" "url" => "/21735794/0000009300000003/v1_201803010437/S2173579417301317/v1_201803010437/en/main.assets" ] "en" => array:20 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Review</span>" "titulo" => "The diagnostic usefulness of the negative electroretinogram" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "126" "paginaFinal" => "135" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "C. Fuente García, J.J. González-López, F.J. Muñoz-Negrete, G. Rebolleda" "autores" => array:4 [ 0 => array:3 [ "nombre" => "C." "apellidos" => "Fuente García" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 1 => array:4 [ "nombre" => "J.J." "apellidos" => "González-López" "email" => array:1 [ 0 => "juliojose.gonzalez@live.com" ] "referencia" => array:3 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 2 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 2 => array:3 [ "nombre" => "F.J." "apellidos" => "Muñoz-Negrete" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "G." "apellidos" => "Rebolleda" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Oftalmología, Hospital Universitario Ramón y Cajal, Instituto Ramón y Cajal de Investigaciones Sanitarias (IRYCIS), Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Departamento de Cirugía, Ciencias Médicas y Sociales, Universidad de Alcalá de Henares, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "La utilidad diagnóstica del electrorretinograma negativo" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 3670 "Ancho" => 2668 "Tamanyo" => 804065 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">A 52-year-old woman diagnosed with Oguchi disease. (A) Negative response in the full-field electroretinogram showing a bilateral reduction of the b wave with preservation of the a wave in the joint response of cones and rods in scotopic conditions. (B) Wide-field retinography of both eyes (Optos California, Optos plc, Fife, Scotland) showing posterior pole tapetal reflex, characteristic of the disease. (C) Autofluorescence pattern with normal blue light in both eyes (Optos California, Optos plc, Fife, Scotland).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Electroretinography is a non-invasive technique used for the functional study of the retina, as well as for the diagnosis and monitoring of diseases that affect the different layers of it. With the advent of new diagnostic and multimodal imaging technologies, like optical coherence tomography and retinography based on laser scanning (scanning laser ophthalmoscopy), the use of electrodiagnostic techniques is increasingly being relegated to retinal consultations.</p><p id="par0010" class="elsevierStylePara elsevierViewall">Briefly, full-field electroretinogram (ERG) or Ganzfeld records the mass electrical response in photoreceptors (PR) and non-neuronal cells of the retina induced by diffuse light stimuli. This response manifests as a wave having, in order of appearance, the following main components: wave a (negative), that originates in the PR, and waves b1 and b2 (positive) originated in Müller glial cells and bipolar cells. The e waves, or oscillatory potentials, appear on the rising crest of the b wave and are formed in the middle layers of the retina (amacrine cells).<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">1</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">The International Society of Clinical Electrophysiology of Vision has established a clinical protocol consisting of 5 stages: rod response, combined response, oscillatory potentials, single flash cone response and flicker response. The first 3 are performed under scotopic conditions and the last 2 under photopic conditions, after a 10-min period of adaptation to light to saturate the rods.<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">2</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Alterations in the different ERG waves can indicate the point in the different layers of the retina where the alteration is found.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The objective of this review is to describe the negative response of ERG and discuss its diagnostic utility in the diseases in which this pattern may appear.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Negative electroretinogram</span><p id="par0030" class="elsevierStylePara elsevierViewall">Negative ERG occurs when a reduction of b wave and preservation of a wave appear in the combined response of cones and rods (ratio b/a <1; [<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>]). This type of pattern indicates an alteration in bipolar or Müller cells, or in the transmission of the photoreceptor stimulus-bipolar cells, while the function of cones and rods remains preserved.<a class="elsevierStyleCrossRef" href="#bib0265"><span class="elsevierStyleSup">3</span></a></p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">One way to classify negative ERG is according to the laterality of the compromise, which often helps discern whether the disease is hereditary or acquired (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>).</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">The negative bilateral and symmetric ERG is typical of hereditary diseases. Some of these diseases can present characteristic signs in the fundoscopy, but the ERG remains fundamental for its diagnosis and classification. This group includes different types of congenital stationary night blindness (CSNB), X-linked juvenile retinoschisis and Duchenne and Becker muscular dystrophies (DMD/BMD).</p><p id="par0045" class="elsevierStylePara elsevierViewall">Moreover, a negative unilateral or bilateral asymmetric ERG can be found in acquired diseases such as some types of immunomediated retinitis like Birdshot retinochoroidopathy, autoimmune retinopathy, cancer or melanoma-associated retinopathy or retinal toxicity induced by different drugs or siderosis.<a class="elsevierStyleCrossRef" href="#bib0270"><span class="elsevierStyleSup">4</span></a></p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Genetic disorders</span><p id="par0050" class="elsevierStylePara elsevierViewall">The genetic disorders that most frequently present a negative response in ERG are CSNB, X-linked juvenile retinoschisis and DMD/BMD.</p><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Congenital stationary night blindness</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Introduction</span><p id="par0055" class="elsevierStylePara elsevierViewall">The group of diseases classified as CSNB comprises a series of conditions, generally non-progressive, that present alterations in photoreceptor processing, the retinoid recycling of retinal pigment epithelium or in the transmission of signals through retina bipolar cells.<a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">12</span></a> The estimated prevalence is 5%.<a class="elsevierStyleCrossRef" href="#bib0315"><span class="elsevierStyleSup">13</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">The CSNB are clinically and genetically heterogeneous. More than 360 mutations have been described in 17 different genes.<a class="elsevierStyleCrossRef" href="#bib0320"><span class="elsevierStyleSup">14</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Often, patients complain of altered night vision or with dim light. In addition, although less frequently, they may present photophobia.</p><p id="par0070" class="elsevierStylePara elsevierViewall">Some forms may be associated with other ocular alterations such as poor visual acuity, myopia, nystagmus, strabismus and fundus anomalies. Night vision alterations can sometimes be overlooked because, being highly subjective, are not routinely evaluated in clinics. For this reason, CSNBs are believed to be underdiagnosed.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Classification</span><p id="par0075" class="elsevierStylePara elsevierViewall">One way to classify CSNBs is based on the ocular fundus appearance.</p><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Congenital stationary night blindness with normal fundoscopy</span><p id="par0080" class="elsevierStylePara elsevierViewall">This group is subdivided in those that present alteration at the photoreceptor level or at the bipolar cell level.</p><p id="par0085" class="elsevierStylePara elsevierViewall">CSNB with photoreceptor dysfunction (Riggs type). Riggs described a case of CSNB of autosomal dominant inheritance in which a reduction of scotopic responses with residual function of the cones was observed. The ERG showed the fall of wave a in scotopic conditions, which reflected the photoreceptor dysfunction and a decrease in the ratio b/a. Under photopic conditions, ERG remained normal.<a class="elsevierStyleCrossRef" href="#bib0325"><span class="elsevierStyleSup">15</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">CSNB with bipolar cell dysfunction (Schubert–Bornschein type). Schubert and Bornschein described cases of CSNB in which the observed combined response of ERG under scotopic conditions presented a preservation of wave a with decrease of wave b, giving the characteristic electronegative pattern. The inheritance patterns that have been described within this subgroup are autosomal recessive and linked to X.</p><p id="par0095" class="elsevierStylePara elsevierViewall">In addition, this group is subdivided into an incomplete form in which the ON pathway of the bipolar cells is affected, and a complete one in which both the ON and OFF response pathways are altered.<a class="elsevierStyleCrossRef" href="#bib0320"><span class="elsevierStyleSup">14</span></a></p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Congenital stationary night blindness with abnormal fundoscopy</span><p id="par0100" class="elsevierStylePara elsevierViewall">This group includes Oguchi disease and fundus albipunctatus.</p><p id="par0105" class="elsevierStylePara elsevierViewall">Oguchi disease is a rare autosomal recessive non-progressive disease. Oguchi disease type 1 is caused by mutations in the <span class="elsevierStyleItalic">SAG</span> gene, which codes for arrestin, located on chromosome 2q37; while type 2 has mutations in the <span class="elsevierStyleItalic">GRK1</span> gene, which codes for rhodopsin kinase, and is located in chromosome 13q34.<a class="elsevierStyleCrossRef" href="#bib0330"><span class="elsevierStyleSup">16</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall">Patients are clinically characterized by stable night blindness from early childhood with normal vision under photopic conditions, but they often claim that their night vision improves when they remain in a dark environment for a long time. They present normal visual fields. The tapetal reflex in the fundus is key to the diagnosis of this disease (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>), especially when it disappears after prolonged adaptation to scotopic conditions (Mizuo–Nakamura phenomenon).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0115" class="elsevierStylePara elsevierViewall">In photopic conditions the ERG is usually normal, whereas in scotopic conditions a decrease in the amplitude of the b wave and conservation of the a wave is observed, giving an electronegative pattern. Both the ON and OFF response of the bipolar cells are preserved in Oguchi disease.<a class="elsevierStyleCrossRef" href="#bib0335"><span class="elsevierStyleSup">17</span></a></p><p id="par0120" class="elsevierStylePara elsevierViewall">Fundus albipunctatus. Autosomal recessive inheritance disease. The patients are clinically characterized by presenting nictalopia, late adaptation to darkness and fundus anomalies associated with mutations in the RDH5 gene, located on the long arm of chromosome 12, which codes for the enzyme 11-cis-retinol dehydrogenase.</p><p id="par0125" class="elsevierStylePara elsevierViewall">Visual acuity, color vision and visual fields are usually preserved. Examination of the fundus reveals small white spots in the posterior pole and mid periphery with macular area conservation.</p><p id="par0130" class="elsevierStylePara elsevierViewall">Standard scotopic ERGs of the International Society of Clinical Electrophysiology of Vision performed after 20<span class="elsevierStyleHsp" style=""></span>min of adaptation to darkness reveal abnormalities that reflect alterations in the regeneration of rhodopsin.<a class="elsevierStyleCrossRef" href="#bib0340"><span class="elsevierStyleSup">18</span></a></p><p id="par0135" class="elsevierStylePara elsevierViewall">Under scotopic conditions under low flash, the response is null or moderately diminished, whereas with bright flash the b/a ratio decreases, giving the electronegative pattern. The photopic response is diminished in 50% of the patients, with delays in the Flicker response being observed.<a class="elsevierStyleCrossRef" href="#bib0345"><span class="elsevierStyleSup">19</span></a></p></span></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">X-linked juvenile retinoschisis</span><p id="par0140" class="elsevierStylePara elsevierViewall">Disease of recessive inheritance linked to X that primarily affects males and is characterized by a decrease in visual acuity that begins in the first decade of life.</p><p id="par0145" class="elsevierStylePara elsevierViewall">Patients present mutations in the RS1<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">20</span></a> gene. The RS1 gene, located on chromosome Xp22.1, codes for retinoschisin, an adhesive protein responsible for the interaction/adhesion between photoreceptors, bipolar cells and Müller cells, which contributes to the maintenance of the structural integrity of the retina.<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">21</span></a> When this protein fails, cystic spaces are formed between these 2 layers of the retina. The function of the photoreceptors is preserved, but the transmission of the stimulus to the bipolar cells would be altered.</p><p id="par0150" class="elsevierStylePara elsevierViewall">It is also common to observe a tapetal reflex in these patients (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>) in the peripheral retina similar to that observed in a predominantly post-equatorial fashion in Oguchi's disease,<a class="elsevierStyleCrossRefs" href="#bib0270"><span class="elsevierStyleSup">4,20</span></a> which has also been described in some carrier women.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0155" class="elsevierStylePara elsevierViewall">In more advanced stages of the disease, vitreous hemorrhages, retinal detachments and neovascular glaucoma that can induce severe loss of vision can be observed.<a class="elsevierStyleCrossRef" href="#bib0360"><span class="elsevierStyleSup">22</span></a></p><p id="par0160" class="elsevierStylePara elsevierViewall">The ERG shows a reduction in the b wave amplitude and a relative preservation of the a wave in the scotopic ERG (negative ERG of rods and mixed), the photopic ERG remaining normal (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). This pattern shows the correct function of the photoreceptors. In addition, an increase in latency of the b wave can be observed.<a class="elsevierStyleCrossRef" href="#bib0365"><span class="elsevierStyleSup">23</span></a></p><p id="par0165" class="elsevierStylePara elsevierViewall">The oscillatory potentials are affected since the transmission of the electrical signal to the innermost layers of the retina is not good. Finally, in advanced stages of the disease, both wave a and wave b are seen diminished.</p><p id="par0170" class="elsevierStylePara elsevierViewall">Optical coherence tomography shows areas of schisis in the macular region (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>).</p><p id="par0175" class="elsevierStylePara elsevierViewall">The confirmation diagnosis is arrived at through a molecular genetic analysis by direct sequencing of the <span class="elsevierStyleItalic">RS1</span> gene, which detects mutations in approximately 90% of patients.<a class="elsevierStyleCrossRef" href="#bib0370"><span class="elsevierStyleSup">24</span></a></p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Duchenne muscular dystrophy/Becker muscular dystrophy</span><p id="par0180" class="elsevierStylePara elsevierViewall">DMD and DMB are neuromuscular diseases characterized by atrophy and progressive muscle weakness as a consequence of skeletal, smooth and cardiac muscle degeneration. DMD is more frequent, early and severe than BMD.</p><p id="par0185" class="elsevierStylePara elsevierViewall">These diseases of recessive inheritance linked to the X are caused by a deficit of dystrophin, a structural protein of skeletal and cardiac muscle. This protein is encoded by the DMD gene, located on the X chromosome. Its deficit leads to progressive necrosis.<a class="elsevierStyleCrossRef" href="#bib0375"><span class="elsevierStyleSup">25</span></a></p><p id="par0190" class="elsevierStylePara elsevierViewall">In these patients it has been observed that, even when they remain asymptomatic from the ophthalmological point of view and with a normal fundus, they can present a negative ERG pattern. Dystrophin is found in synaptic complexes between PRs and bipolar cells. Deletions in this gene produce losses of these unions causing a drop in the b wave.<a class="elsevierStyleCrossRef" href="#bib0380"><span class="elsevierStyleSup">26</span></a></p><p id="par0195" class="elsevierStylePara elsevierViewall">The suspected diagnosis is through serum creatine kinase levels. Other levels, such as metallopeptidase of matrix 9, metallopeptidase of matrix 2, tissue inhibitor of metalloproteinase 1, myostatin and follistatin can also be useful, with genetic studies being necessary to confirm the disease.<a class="elsevierStyleCrossRef" href="#bib0385"><span class="elsevierStyleSup">27</span></a></p></span></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Acquired diseases</span><p id="par0200" class="elsevierStylePara elsevierViewall">Finding a reduced b wave in scotopic conditions unilaterally or bilaterally asymmetrical leads to a retinopathy of acquired origin. The most frequently associated disease is Birdshot retinochoroidopathy.<a class="elsevierStyleCrossRef" href="#bib0390"><span class="elsevierStyleSup">28</span></a> It can also appear in other autoimmune retinopathies, in retinopathies associated to cancer/melanoma, in retinal toxicity induced by different drugs or in siderosis.</p><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Inflammatory</span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Birdshot retinochoroidopathy</span><p id="par0205" class="elsevierStylePara elsevierViewall">Birdshot disease is characterized by chronic, usually bilateral inflammation exhibiting vitritis-associated areas of choroidal depigmentation, cystic macular edema and vasculitis. These signs may vary depending on the stage of the disease.</p><p id="par0210" class="elsevierStylePara elsevierViewall">A strong association between the disease and the human leukocyte antigen-A29 has been found. This suggests a role for T-lymphocytes in the pathogenesis and a relationship with interleukin 17.<a class="elsevierStyleCrossRef" href="#bib0395"><span class="elsevierStyleSup">29</span></a></p><p id="par0215" class="elsevierStylePara elsevierViewall">Patients usually complain of decreased visual acuity and myodesopsies. Other symptoms that may appear include nictalopia, photopsias, sensation of “white noise”, diminished visual field and dyschromatopsia.<a class="elsevierStyleCrossRef" href="#bib0400"><span class="elsevierStyleSup">30</span></a></p><p id="par0220" class="elsevierStylePara elsevierViewall">Electrophysiological findings suggest that the internal retina is affected in Birdshot's disease. The presence of antiretinal antibodies in the inner plexiform layer has been described and, although the mechanisms by which it is produced are not yet established, a delay in the flicker response of the cones is usually observed, which is related to the inflammation of the superficial layers. A propensity to the ON response pathway, responsible for the fall of the b wave, is also observed. Internal retina inflammation causes an alteration in phototransduction, giving the characteristic electronegative pattern.<a class="elsevierStyleCrossRef" href="#bib0405"><span class="elsevierStyleSup">31</span></a></p><p id="par0225" class="elsevierStylePara elsevierViewall">The diagnosis of the disease is mainly clinical, through diagnostic criteria comprising 4 requirements (bilaterality, characteristic lesions, cells in anterior chamber and vitritis), and 3 that support the diagnosis (human leukocyte antigen A29+, vasculitis and cystic macular edema). An additional 3 exclusion criteria are also established (retrokeratic precipitates, posterior synechiae or some other predisposing cause: infectious, neoplastic, etc.).<a class="elsevierStyleCrossRef" href="#bib0410"><span class="elsevierStyleSup">32</span></a></p></span><span id="sec0070" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Autoimmune retinitis</span><p id="par0230" class="elsevierStylePara elsevierViewall">X-linked hyper IgM syndrome may also present this characteristic ERG pattern, involving a defect or deficiency of a protein called “CD40 ligand”, located in the gene family of the tumor necrosis factor (TNF) of chromosome X. As a consequence of this deficiency, the patient's affected T lymphocytes cannot instruct the B lymphocytes to change the production of gammaglobulins from IgM to IgG and IgA, presenting reduced levels of IgG and IgA in serum and a normal or elevated level of IgM. In addition, the CD40 ligand is also relevant in other protective functions of T lymphocytes, the loss of which predisposes to a range of infections.<a class="elsevierStyleCrossRef" href="#bib0415"><span class="elsevierStyleSup">33</span></a></p><p id="par0235" class="elsevierStylePara elsevierViewall">Patients with X-linked hyper IgM have an increased risk of developing neoplasms, mainly lymphomas and tumors in the biliary tract and liver.</p><p id="par0240" class="elsevierStylePara elsevierViewall">A described case showed a patient that debuted with nictalopia and decreased bilateral visual field. Clinical data suggested autoimmune retinopathy, while indirect immunohistochemistry detected antiretinal antibodies against antigens of the nerve fiber layer, ganglion cell layer and other components of the internal retina such as bipolar, amacrine and horizontal cells. In addition, a hypersensitivity of the retinal pigment epithelium (RPE) was observed.</p><p id="par0245" class="elsevierStylePara elsevierViewall">Both the EOG and the VF were altered, and the ERG showed a negative pattern under scotopic conditions in the mixed cones-rod response.<a class="elsevierStyleCrossRef" href="#bib0420"><span class="elsevierStyleSup">34</span></a></p><p id="par0250" class="elsevierStylePara elsevierViewall">Several cases of patients with a history of recurring acute anterior uveitis, panuveitis, and internal punctate choroidopathy episodes with a negative response in the ERG have also been described. The ERG in this heterogeneous group of patients predominantly presented ON and OFF pathways compromised by post-phototransduction.<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">11</span></a></p></span><span id="sec0075" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Retinopathy associated to carcinoma/melanoma</span><p id="par0255" class="elsevierStylePara elsevierViewall">The incidence of paraneoplastic syndromes in patients with cancer is estimated at around 10%, involving the visual and nervous system in approximately 0.01% of patients.<a class="elsevierStyleCrossRef" href="#bib0425"><span class="elsevierStyleSup">35</span></a> This small percentage of patients develop specific antibodies that recognize proteins and subsets of neurons in the retina. When this happens, these cases could involve retinopathy associated with carcinoma or melanoma. Clinically, said patients may exhibit diminished visual acuity, nictalopia, photopsies, diminished visual field, vascular attenuation or chorioretinal atrophy.<a class="elsevierStyleCrossRef" href="#bib0430"><span class="elsevierStyleSup">36</span></a> In up to 50% of cases, visual symptoms precede the symptomatology of the underlying disease.<a class="elsevierStyleCrossRef" href="#bib0435"><span class="elsevierStyleSup">37</span></a></p><p id="par0260" class="elsevierStylePara elsevierViewall">Some cases have been described with patients diagnosed with small cell lung carcinoma, gastrointestinal carcinomas, endometrial, breast and ovarian cancer.<a class="elsevierStyleCrossRefs" href="#bib0425"><span class="elsevierStyleSup">35,38</span></a> These patients evidence cone and rod degeneration, decreasing both wave a and b, while the isolated decrease of the b wave is much less frequent.</p><p id="par0265" class="elsevierStylePara elsevierViewall">However, in melanoma-associated retinopathy, a reduction of the b wave is observed with greater frequency, possibly due to the preferential involvement of the bipolar cell pathway ON.<a class="elsevierStyleCrossRef" href="#bib0445"><span class="elsevierStyleSup">39</span></a> This autoimmune response occurs more frequently in males 4.5:1.<a class="elsevierStyleCrossRef" href="#bib0450"><span class="elsevierStyleSup">40</span></a> The central visual acuity is usually preserved or slightly diminished. Generally, the visual fields are also normal, although both paracentral scotoma and peripheral losses can appear. Fundoscopy can reveal pallor of the optic nerve, vascular attenuation, changes in the RPE, vitritis or periflebitis.<a class="elsevierStyleCrossRef" href="#bib0455"><span class="elsevierStyleSup">41</span></a></p></span></span><span id="sec0080" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Toxicity</span><p id="par0270" class="elsevierStylePara elsevierViewall">Some substances are capable of producing retinal toxicity, affecting the functions of the different layers of the retina.</p><p id="par0275" class="elsevierStylePara elsevierViewall">When the alteration occurs at a post-phototransduction level, a negative ERG can be observed.</p><span id="sec0085" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Methanol</span><p id="par0280" class="elsevierStylePara elsevierViewall">Methanol is a neurotoxic substance that can be found in different industrial products and in some alcoholic beverages (often household distillates).</p><p id="par0285" class="elsevierStylePara elsevierViewall">The inhibition of mitochondrial cytochrome c oxidase causes cell death, especially in the retina and in the optic nerve.<a class="elsevierStyleCrossRef" href="#bib0460"><span class="elsevierStyleSup">42</span></a></p><p id="par0290" class="elsevierStylePara elsevierViewall">In the acute phase of retinal toxicity, patients exhibit photophobia, decreased visual acuity and visual field alterations. Retinal or papillary edema may appear in the fundus. In a second phase, atrophy and the characteristic alterations of ERG would appear.<a class="elsevierStyleCrossRef" href="#bib0465"><span class="elsevierStyleSup">43</span></a></p></span><span id="sec0090" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Vigabatrin</span><p id="par0295" class="elsevierStylePara elsevierViewall">Vigabatrin is an antiepileptic used in refractory epilepsies and infantile spasms. It is a selective and irreversible inhibitor of GABA-transaminase, the enzyme responsible for the metabolic cleavage of the GABA neurotransmitter.</p><p id="par0300" class="elsevierStylePara elsevierViewall">From the ophthalmological point of view, visual field loss is the first adverse effect that we find as a consequence of the toxicity of this drug.<a class="elsevierStyleCrossRef" href="#bib0470"><span class="elsevierStyleSup">44</span></a> Moreover, patients may present decreased visual acuity, dyschromatopsia, decreased sensitivity to contrast and alterations in the ERG.</p><p id="par0305" class="elsevierStylePara elsevierViewall">Electrophysiological studies show alterations in the cones and rods system, exhibiting a preference for a drop of the b wave and oscillatory potentials. The Flicker response may also appear delayed. All these findings point to an alteration in the inner layers of the retina, although the responsible underlying mechanism remains unknown.<a class="elsevierStyleCrossRef" href="#bib0475"><span class="elsevierStyleSup">45</span></a> A partial recovery of the electroretinographic alterations has been observed after ceasing the antiepileptic medication.</p></span><span id="sec0095" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">Quinine</span><p id="par0310" class="elsevierStylePara elsevierViewall">Quinine is a natural, white and crystalline alkaloid with antipyretic, antimalarial and analgesic properties produced by species of the genus <span class="elsevierStyleItalic">Cinchona</span>. Intoxication by this substance produces cinconism.</p><p id="par0315" class="elsevierStylePara elsevierViewall">Visual symptoms may appear in the 24<span class="elsevierStyleHsp" style=""></span>h after a dose greater than 4<span class="elsevierStyleHsp" style=""></span>g. In the acute phase patients experience nausea, vomiting, headache and hypotension. At this stage, fundoscopy can show massive retinal edema, cherry red spot, disc pallor and narrowing of blood vessels. Visual acuity is diminished and the visual field altered.<a class="elsevierStyleCrossRef" href="#bib0480"><span class="elsevierStyleSup">46</span></a></p><p id="par0320" class="elsevierStylePara elsevierViewall">ERG findings depend on the phase of the disorder. In the acute phase, all responses are reduced. When visual acuity is recovered, the a wave is normalized, however the b wave remains altered, giving the characteristic electronegative pattern. Over time the b wave may show a slight recovery, but it is rarely normalized.<a class="elsevierStyleCrossRef" href="#bib0485"><span class="elsevierStyleSup">47</span></a></p><p id="par0325" class="elsevierStylePara elsevierViewall">These alterations are partly due to the vasoconstrictor effect of the drug that predominantly produces ischemia of the inner retina layers, and to direct retinal toxicity caused by the block of cholinergic transmission within the synaptic layers.</p></span><span id="sec0100" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0120">Siderosis</span><p id="par0330" class="elsevierStylePara elsevierViewall">An intraocular foreign body can produce secondarily a state of siderosis. The physiopathological mechanism involves the transformation of the non-ionized or ferrous state into the ferric form.</p><p id="par0335" class="elsevierStylePara elsevierViewall">Symptoms include blurred vision, pupillary mydriasis, iris heterochromia or corneal staining. Fundoscopy can be normal or show papillary edema.</p><p id="par0340" class="elsevierStylePara elsevierViewall">In early stages a supernormal response in the ERG is found. If the foreign body remains in the intraocular space, the b wave of the ERG begins to decrease, wave a remains stable until advanced stages in which the ERG signal is undetectable. The pathophysiological mechanism of this situation is due to direct toxicity on the inner retina layers and the secondary ischemia that occurs.<a class="elsevierStyleCrossRef" href="#bib0285"><span class="elsevierStyleSup">7</span></a></p></span><span id="sec0105" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0125">Others</span><p id="par0345" class="elsevierStylePara elsevierViewall">Internal retinal toxicity with alterations in ERG has also been described with other substances such as vincristine, cisplatin or canthaxanthin.<a class="elsevierStyleCrossRef" href="#bib0285"><span class="elsevierStyleSup">7</span></a></p></span></span><span id="sec0110" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0130">Vascular</span><p id="par0350" class="elsevierStylePara elsevierViewall">In ischemia situations such as obstruction of the central retinal artery or obstruction of the central retinal vein, the ERG shows the characteristic negative response. However, given that the diagnosis of these entities is fundamentally clinical and angiographic, electrophysiological studies are rarely performed in these patients.</p><p id="par0355" class="elsevierStylePara elsevierViewall">In the obstruction of the central artery, the internal retina is compromised by the lack of irrigation, while the RPE and the photoreceptors that receive vascularization through the choroidocapillary system remain intact.</p><p id="par0360" class="elsevierStylePara elsevierViewall">During the acute phase of this process, internal layers edema and the cherry red spot are characteristic in fundoscopy and, together with the sharp decrease in visual acuity, facilitate diagnosis. However, after this phase, the retinal edema is resolved and fundoscopic findings are limited to a slight disc pallor and attenuation of vascularization, which could go unnoticed. In this phase, a decrease in wave b with conservation of the wave a is found together with a decrease in the oscillatory potentials and the flicker response in the ERG, revealing the damage of the inner retina layers with preservation of the photoreceptor function.<a class="elsevierStyleCrossRef" href="#bib0490"><span class="elsevierStyleSup">48</span></a></p><p id="par0365" class="elsevierStylePara elsevierViewall">Frequently, in the obstruction of the central retinal vein, the intense hemorrhages hinder the interpretation of angiography and make it harder to differentiate the non-ischemic form from the ischemic one. In addition to the study of the pupillary reflex, electrophysiology is a good tool to predict the degree of ischemia and the risk of neovascularization.<a class="elsevierStyleCrossRef" href="#bib0495"><span class="elsevierStyleSup">49</span></a> The decrease in the ratio b/a is proportional to capillary loss, indicating the degree of ischemia. Likewise, it has been observed that the amplitude of the b wave and the delay of the flicker response are related to the risk of developing neovascularization.<a class="elsevierStyleCrossRef" href="#bib0500"><span class="elsevierStyleSup">50</span></a></p></span></span><span id="sec0115" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0135">Conclusion</span><p id="par0370" class="elsevierStylePara elsevierViewall">The combined response of cones and rods under scotopic conditions in negative ERG enables the observer to differentiate an alteration in the transmission of the stimulus with preservation of photoreceptor function. Its correct identification allows narrowing the diagnosis and is of great help for diagnosing this small group of rare diseases.</p><p id="par0375" class="elsevierStylePara elsevierViewall">The bilaterality and symmetry of the waves, the recovery of normal electrophysiological patterns and the clinical characteristics of the patient must be taken into account when establishing the differential diagnosis.</p></span><span id="sec0120" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0140">Conflicts of interest</span><p id="par0380" class="elsevierStylePara elsevierViewall">JJGL has received grants for study from Abbvie, Allergan and Angelini. The rest of authors declare not having any conflict of interests.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:11 [ 0 => array:3 [ "identificador" => "xres993661" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec957098" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres993660" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec957097" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Negative electroretinogram" ] 6 => array:3 [ "identificador" => "sec0015" "titulo" => "Genetic disorders" "secciones" => array:3 [ 0 => array:3 [ "identificador" => "sec0020" "titulo" => "Congenital stationary night blindness" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0025" "titulo" => "Introduction" ] 1 => array:3 [ "identificador" => "sec0030" "titulo" => "Classification" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0035" "titulo" => "Congenital stationary night blindness with normal fundoscopy" ] 1 => array:2 [ "identificador" => "sec0040" "titulo" => "Congenital stationary night blindness with abnormal fundoscopy" ] ] ] ] ] 1 => array:2 [ "identificador" => "sec0045" "titulo" => "X-linked juvenile retinoschisis" ] 2 => array:2 [ "identificador" => "sec0050" "titulo" => "Duchenne muscular dystrophy/Becker muscular dystrophy" ] ] ] 7 => array:3 [ "identificador" => "sec0055" "titulo" => "Acquired diseases" "secciones" => array:3 [ 0 => array:3 [ "identificador" => "sec0060" "titulo" => "Inflammatory" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0065" "titulo" => "Birdshot retinochoroidopathy" ] 1 => array:2 [ "identificador" => "sec0070" "titulo" => "Autoimmune retinitis" ] 2 => array:2 [ "identificador" => "sec0075" "titulo" => "Retinopathy associated to carcinoma/melanoma" ] ] ] 1 => array:3 [ "identificador" => "sec0080" "titulo" => "Toxicity" "secciones" => array:5 [ 0 => array:2 [ "identificador" => "sec0085" "titulo" => "Methanol" ] 1 => array:2 [ "identificador" => "sec0090" "titulo" => "Vigabatrin" ] 2 => array:2 [ "identificador" => "sec0095" "titulo" => "Quinine" ] 3 => array:2 [ "identificador" => "sec0100" "titulo" => "Siderosis" ] 4 => array:2 [ "identificador" => "sec0105" "titulo" => "Others" ] ] ] 2 => array:2 [ "identificador" => "sec0110" "titulo" => "Vascular" ] ] ] 8 => array:2 [ "identificador" => "sec0115" "titulo" => "Conclusion" ] 9 => array:2 [ "identificador" => "sec0120" "titulo" => "Conflicts of interest" ] 10 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2017-07-26" "fechaAceptado" => "2017-10-11" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec957098" "palabras" => array:5 [ 0 => "Birdshot chorioretinopathy" 1 => "Congenital stationary night blindness" 2 => "Electroretinography" 3 => "Ocular paraneoplastic syndromes" 4 => "Retinoschisis" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec957097" "palabras" => array:5 [ 0 => "Ceguera nocturna estacionaria congénita" 1 => "Electrorretinografía" 2 => "Retinocoroiditis en perdigonada" 3 => "Retinosquisis" 4 => "Síndromes paraneoplásicos oculares" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">The definition of the negative response of the full field electroretinogram is the presence of a b-wave with less amplitude than the a-wave (b/a ratio <1) in the combined response of cones and rods. The presence of this pattern reflects an alteration in the bipolar cells, the Müller cells, or in the transmission of the stimulus from the photoreceptors to the bipolar cells, with preserved photoreceptor function.</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">This finding can be seen bilaterally and symmetrically in different hereditary conditions, such as congenital stationary night blindness, juvenile X-linked retinoschisis, and Duchenne and Becker muscular dystrophies. On the other hand, it can also be found unilaterally (or asymmetrically) in acquired pathologies, such as some types of immuno-mediated retinitis (Birdshot retinochoroiditis), autoimmune retinopathies, cancer/melanoma associated retinopathy, or retinal toxicity.</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">The objective of this review is to summarize the characteristics of the pathologies in which this finding can be observed, in order to highlight its usefulness in the differential diagnosis of retinal conditions.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">La respuesta negativa del electrorretinograma de campo completo se define como una disminución de la onda b con conservación de la onda a (ratio b/a < 1) en la respuesta combinada de conos y bastones. La presencia de este patrón denota una alteración en las células bipolares, en las células de Müller o en la transmisión del estímulo fotorreceptor-células bipolares, con preservación de la función de conos y bastones.</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Este hallazgo puede verse de forma bilateral y simétrica en diferentes enfermedades hereditarias como la ceguera nocturna estacionaria congénita, la retinosquisis juvenil ligada a X y las distrofias musculares de Duchenne y Becker. Por otro lado, también se puede encontrar de forma unilateral en enfermedades adquiridas como algunos tipos de retinitis inmunomediadas (retinocoroiditis en perdigonada o Birdshot), retinopatías autoinmunes, retinopatía asociada a cáncer/melanoma o toxicidad retiniana.</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">El objetivo de esta revisión es resumir las características de las afecciones en las que puede observarse este hallazgo, de gran utilidad en el diagnóstico diferencial de enfermedades de la retina.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Fuente García C, González-López JJ, Muñoz-Negrete FJ, Rebolleda G. La utilidad diagnóstica del electrorretinograma negativo. Arch Soc Esp Oftalmol. 2018;93:126–135.</p>" ] ] "multimedia" => array:4 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2607 "Ancho" => 2974 "Tamanyo" => 885079 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">(A) Full-field electroretinogram in scotopic conditions showing the combined response of cones and rods in a healthy individual. A greater amplitude of the b wave is observed compared to the a wave. (B) Full-field electroretinogram in scotopic conditions showing the combined response of cones and rods in a patient with Oguchi disease. A lower amplitude of the b wave is observed compared to the a wave (negative response or negative electroretinogram).</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 3670 "Ancho" => 2668 "Tamanyo" => 804065 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">A 52-year-old woman diagnosed with Oguchi disease. (A) Negative response in the full-field electroretinogram showing a bilateral reduction of the b wave with preservation of the a wave in the joint response of cones and rods in scotopic conditions. (B) Wide-field retinography of both eyes (Optos California, Optos plc, Fife, Scotland) showing posterior pole tapetal reflex, characteristic of the disease. (C) Autofluorescence pattern with normal blue light in both eyes (Optos California, Optos plc, Fife, Scotland).</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 3004 "Ancho" => 2667 "Tamanyo" => 850842 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">A 25-year-old male diagnosed with X-linked juvenile retinoschisis. (A) Negative response in the full-field electroretinogram showing a bilateral reduction of the b wave with conservation of the a wave in the joint response of cones and rods in scotopic conditions. (B) Bilateral wide-field retinography showing that tapetal reflex in the periphery (Optos California, Optos plc, Fife, Scotland). (C) Optical coherence tomography of bilateral spectral domain (CIRRUS™ HD-OCT 5000, Carl Zeiss Meditec AG, Jena, Germany) in which areas of retinal schisis are observed in the macular region.</p>" ] ] 3 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Hereditary \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="center" valign="top" scope="col" style="border-bottom: 2px solid black">Acquired \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Bilateral symmetric</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top"><span class="elsevierStyleItalic">Most frequently unilateral or bilateral asymmetric</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Congenital stationary night blindness (CSNB)</span><br><span class="elsevierStyleHsp" style=""></span>Type 1: NYX<br><span class="elsevierStyleHsp" style=""></span>Type 2: CACNA1F<br><span class="elsevierStyleHsp" style=""></span>Oguchi 1: SAG<br><span class="elsevierStyleHsp" style=""></span>Oguchi 2: GRK1<br><span class="elsevierStyleHsp" style=""></span>Fundus albipunctatus: RDH5 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top"><span class="elsevierStyleItalic">Inflammatory:</span><br><span class="elsevierStyleHsp" style=""></span>Birdshot retinochoroiditis<br><span class="elsevierStyleHsp" style=""></span>Autoimmune retinitis<br><span class="elsevierStyleHsp" style=""></span>Retinopathy associated to carcinoma/melanoma \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">X-linked retinoschisis (XLRS)</span><br><span class="elsevierStyleHsp" style=""></span>RS1 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top"><span class="elsevierStyleItalic">Toxicity:</span><br><span class="elsevierStyleHsp" style=""></span>Methanol<a class="elsevierStyleCrossRef" href="#bib0270"><span class="elsevierStyleSup">4</span></a><br><span class="elsevierStyleHsp" style=""></span>Vigabatrin<a class="elsevierStyleCrossRef" href="#bib0275"><span class="elsevierStyleSup">5</span></a><br><span class="elsevierStyleHsp" style=""></span>Quinine<a class="elsevierStyleCrossRef" href="#bib0280"><span class="elsevierStyleSup">6</span></a><br><span class="elsevierStyleHsp" style=""></span>Siderosis<a class="elsevierStyleCrossRef" href="#bib0285"><span class="elsevierStyleSup">7</span></a><br><span class="elsevierStyleHsp" style=""></span>Vincristine<a class="elsevierStyleCrossRef" href="#bib0290"><span class="elsevierStyleSup">8</span></a><br><span class="elsevierStyleHsp" style=""></span>Cisplatin<a class="elsevierStyleCrossRef" href="#bib0295"><span class="elsevierStyleSup">9</span></a><br><span class="elsevierStyleHsp" style=""></span>Canthaxanthin<a class="elsevierStyleCrossRef" href="#bib0300"><span class="elsevierStyleSup">10</span></a> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Becker and Duchenne muscular dystrophies</span><br><span class="elsevierStyleHsp" style=""></span>DMD \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top"><span class="elsevierStyleItalic">Vascular alterations</span><a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">11</span></a>:<br><span class="elsevierStyleHsp" style=""></span>Central retinal artery occlusion<br><span class="elsevierStyleHsp" style=""></span>Central retinal vein occlusion \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1684828.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Summary of causes of decrease in ratio b:a (negative response) in the combined response of cones and rods in scotopic conditions of the full-field electroretinogram.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:50 [ 0 => array:3 [ "identificador" => "bib0255" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Effect of shorter dark adaptation on ISCEV standard DA 0.01 and DA 3 skin ERGs in healthy adults" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "R. 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