metricas
covid
Buscar en
Neurología (English Edition)
Toda la web
Inicio Neurología (English Edition) Analysis of Sturge–Weber syndrome: A retrospective study of multiple associate...
Journal Information

Statistics

Follow this link to access the full text of the article

Original article
Analysis of Sturge–Weber syndrome: A retrospective study of multiple associated variables
Análisis del síndrome de Sturge-Weber: estudio retrospectivo de múltiples variables asociadas
A.I. Maraña Pérez
Corresponding author
maranaai@hotmail.com

Corresponding author.
, M.L. Ruiz-Falcó Rojas, V. Puertas Martín, J. Domínguez Carral, I. Carreras Sáez, A. Duat Rodríguez, V. Sánchez González
Servicio de Neuropediatría, Hospital Infantil Universitario Niño Jesús, Madrid, Spain
Read
8820
Times
was read the article
1581
Total PDF
7239
Total HTML
Share statistics
 array:25 [
  "pii" => "S2173580817300913"
  "issn" => "21735808"
  "doi" => "10.1016/j.nrleng.2015.12.006"
  "estado" => "S300"
  "fechaPublicacion" => "2017-07-01"
  "aid" => "845"
  "copyright" => "Sociedad Española de Neurología"
  "copyrightAnyo" => "2014"
  "documento" => "article"
  "crossmark" => 1
  "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/"
  "subdocumento" => "fla"
  "cita" => "Neurologia. 2017;32:363-70"
  "abierto" => array:3 [
    "ES" => true
    "ES2" => true
    "LATM" => true
  ]
  "gratuito" => true
  "lecturas" => array:2 [
    "total" => 2092
    "formatos" => array:3 [
      "EPUB" => 97
      "HTML" => 1544
      "PDF" => 451
    ]
  ]
  "Traduccion" => array:1 [
    "es" => array:20 [
      "pii" => "S0213485316000244"
      "issn" => "02134853"
      "doi" => "10.1016/j.nrl.2015.12.012"
      "estado" => "S300"
      "fechaPublicacion" => "2017-07-01"
      "aid" => "845"
      "copyright" => "Sociedad Española de Neurología"
      "documento" => "article"
      "crossmark" => 1
      "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/"
      "subdocumento" => "fla"
      "cita" => "Neurologia. 2017;32:363-70"
      "abierto" => array:3 [
        "ES" => true
        "ES2" => true
        "LATM" => true
      ]
      "gratuito" => true
      "lecturas" => array:2 [
        "total" => 6136
        "formatos" => array:3 [
          "EPUB" => 105
          "HTML" => 4117
          "PDF" => 1914
        ]
      ]
      "es" => array:13 [
        "idiomaDefecto" => true
        "cabecera" => "<span class="elsevierStyleTextfn">Original</span>"
        "titulo" => "An&#225;lisis del s&#237;ndrome de Sturge-Weber&#58; estudio retrospectivo de m&#250;ltiples variables asociadas"
        "tienePdf" => "es"
        "tieneTextoCompleto" => "es"
        "tieneResumen" => array:2 [
          0 => "es"
          1 => "en"
        ]
        "paginas" => array:1 [
          0 => array:2 [
            "paginaInicial" => "363"
            "paginaFinal" => "370"
          ]
        ]
        "titulosAlternativos" => array:1 [
          "en" => array:1 [
            "titulo" => "Analysis of Sturge-Weber syndrome&#58; A retrospective study of multiple associated variables"
          ]
        ]
        "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" => "fig0015"
            "etiqueta" => "Figura 3"
            "tipo" => "MULTIMEDIAFIGURA"
            "mostrarFloat" => true
            "mostrarDisplay" => false
            "figura" => array:1 [
              0 => array:4 [
                "imagen" => "gr3.jpeg"
                "Alto" => 1162
                "Ancho" => 960
                "Tamanyo" => 80477
              ]
            ]
            "descripcion" => array:1 [
              "es" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Resonancia magn&#233;tica craneal T1 con contraste que muestra angiomatosis leptomen&#237;ngea bilateral&#46;</p>"
            ]
          ]
        ]
        "autores" => array:1 [
          0 => array:2 [
            "autoresLista" => "A&#46;I&#46; Mara&#241;a P&#233;rez, M&#46;L&#46; Ruiz-Falc&#243; Rojas, V&#46; Puertas Mart&#237;n, J&#46; Dom&#237;nguez Carral, I&#46; Carreras S&#225;ez, A&#46; Duat Rodr&#237;guez, V&#46; S&#225;nchez Gonz&#225;lez"
            "autores" => array:7 [
              0 => array:2 [
                "nombre" => "A&#46;I&#46;"
                "apellidos" => "Mara&#241;a P&#233;rez"
              ]
              1 => array:2 [
                "nombre" => "M&#46;L&#46;"
                "apellidos" => "Ruiz-Falc&#243; Rojas"
              ]
              2 => array:2 [
                "nombre" => "V&#46;"
                "apellidos" => "Puertas Mart&#237;n"
              ]
              3 => array:2 [
                "nombre" => "J&#46;"
                "apellidos" => "Dom&#237;nguez Carral"
              ]
              4 => array:2 [
                "nombre" => "I&#46;"
                "apellidos" => "Carreras S&#225;ez"
              ]
              5 => array:2 [
                "nombre" => "A&#46;"
                "apellidos" => "Duat Rodr&#237;guez"
              ]
              6 => array:2 [
                "nombre" => "V&#46;"
                "apellidos" => "S&#225;nchez Gonz&#225;lez"
              ]
            ]
          ]
        ]
      ]
      "idiomaDefecto" => "es"
      "Traduccion" => array:1 [
        "en" => array:9 [
          "pii" => "S2173580817300913"
          "doi" => "10.1016/j.nrleng.2015.12.006"
          "estado" => "S300"
          "subdocumento" => ""
          "abierto" => array:3 [
            "ES" => true
            "ES2" => true
            "LATM" => true
          ]
          "gratuito" => true
          "lecturas" => array:1 [
            "total" => 0
          ]
          "idiomaDefecto" => "en"
          "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173580817300913?idApp=UINPBA00004N"
        ]
      ]
      "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0213485316000244?idApp=UINPBA00004N"
      "url" => "/02134853/0000003200000006/v1_201707060041/S0213485316000244/v1_201707060041/es/main.assets"
    ]
  ]
  "itemSiguiente" => array:20 [
    "pii" => "S2173580817300901"
    "issn" => "21735808"
    "doi" => "10.1016/j.nrleng.2015.12.005"
    "estado" => "S300"
    "fechaPublicacion" => "2017-07-01"
    "aid" => "844"
    "copyright" => "Sociedad Espa&#241;ola de Neurolog&#237;a"
    "documento" => "article"
    "crossmark" => 1
    "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/"
    "subdocumento" => "fla"
    "cita" => "Neurologia. 2017;32:371-6"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:2 [
      "total" => 790
      "formatos" => array:3 [
        "EPUB" => 57
        "HTML" => 525
        "PDF" => 208
      ]
    ]
    "en" => array:13 [
      "idiomaDefecto" => true
      "cabecera" => "<span class="elsevierStyleTextfn">Original article</span>"
      "titulo" => "Tendencies in cerebral aneurism treatment&#58; Analysis of a hospital series"
      "tienePdf" => "en"
      "tieneTextoCompleto" => "en"
      "tieneResumen" => array:2 [
        0 => "en"
        1 => "es"
      ]
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "371"
          "paginaFinal" => "376"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "es" => array:1 [
          "titulo" => "Tendencias en el tratamiento de los aneurismas cerebrales&#58; an&#225;lisis de una serie hospitalaria"
        ]
      ]
      "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" => "Figure 1"
          "tipo" => "MULTIMEDIAFIGURA"
          "mostrarFloat" => true
          "mostrarDisplay" => false
          "figura" => array:1 [
            0 => array:4 [
              "imagen" => "gr1.jpeg"
              "Alto" => 1800
              "Ancho" => 1569
              "Tamanyo" => 154581
            ]
          ]
          "descripcion" => array:1 [
            "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Hunt and Hess scale&#46;</p>"
          ]
        ]
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "A&#46; Lago, R&#46; L&#243;pez-Cuevas, J&#46;I&#46; Tembl, G&#46; Fortea, D&#46; G&#243;rriz, F&#46; Aparici, V&#46; Parkhutik"
          "autores" => array:7 [
            0 => array:2 [
              "nombre" => "A&#46;"
              "apellidos" => "Lago"
            ]
            1 => array:2 [
              "nombre" => "R&#46;"
              "apellidos" => "L&#243;pez-Cuevas"
            ]
            2 => array:2 [
              "nombre" => "J&#46;I&#46;"
              "apellidos" => "Tembl"
            ]
            3 => array:2 [
              "nombre" => "G&#46;"
              "apellidos" => "Fortea"
            ]
            4 => array:2 [
              "nombre" => "D&#46;"
              "apellidos" => "G&#243;rriz"
            ]
            5 => array:2 [
              "nombre" => "F&#46;"
              "apellidos" => "Aparici"
            ]
            6 => array:2 [
              "nombre" => "V&#46;"
              "apellidos" => "Parkhutik"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "en"
    "Traduccion" => array:1 [
      "es" => array:9 [
        "pii" => "S0213485316000232"
        "doi" => "10.1016/j.nrl.2015.12.011"
        "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/S0213485316000232?idApp=UINPBA00004N"
      ]
    ]
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173580817300901?idApp=UINPBA00004N"
    "url" => "/21735808/0000003200000006/v1_201707180027/S2173580817300901/v1_201707180027/en/main.assets"
  ]
  "itemAnterior" => array:20 [
    "pii" => "S2173580817300925"
    "issn" => "21735808"
    "doi" => "10.1016/j.nrleng.2015.12.007"
    "estado" => "S300"
    "fechaPublicacion" => "2017-07-01"
    "aid" => "851"
    "copyright" => "Sociedad Espa&#241;ola de Neurolog&#237;a"
    "documento" => "article"
    "crossmark" => 1
    "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/"
    "subdocumento" => "fla"
    "cita" => "Neurologia. 2017;32:355-62"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:2 [
      "total" => 1169
      "formatos" => array:3 [
        "EPUB" => 48
        "HTML" => 814
        "PDF" => 307
      ]
    ]
    "en" => array:12 [
      "idiomaDefecto" => true
      "cabecera" => "<span class="elsevierStyleTextfn">Original article</span>"
      "titulo" => "Executive functions and language in children with different subtypes of specific language impairment"
      "tienePdf" => "en"
      "tieneTextoCompleto" => "en"
      "tieneResumen" => array:2 [
        0 => "en"
        1 => "es"
      ]
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "355"
          "paginaFinal" => "362"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "es" => array:1 [
          "titulo" => "Funciones ejecutivas y lenguaje en subtipos de ni&#241;os con trastorno espec&#237;fico del lenguaje"
        ]
      ]
      "contieneResumen" => array:2 [
        "en" => true
        "es" => true
      ]
      "contieneTextoCompleto" => array:1 [
        "en" => true
      ]
      "contienePdf" => array:1 [
        "en" => true
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "V&#46; Acosta Rodr&#237;guez, G&#46;M&#46; Ram&#237;rez Santana, S&#46; Hern&#225;ndez Exp&#243;sito"
          "autores" => array:3 [
            0 => array:2 [
              "nombre" => "V&#46;"
              "apellidos" => "Acosta Rodr&#237;guez"
            ]
            1 => array:2 [
              "nombre" => "G&#46;M&#46;"
              "apellidos" => "Ram&#237;rez Santana"
            ]
            2 => array:2 [
              "nombre" => "S&#46;"
              "apellidos" => "Hern&#225;ndez Exp&#243;sito"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "en"
    "Traduccion" => array:1 [
      "es" => array:9 [
        "pii" => "S021348531600030X"
        "doi" => "10.1016/j.nrl.2015.12.018"
        "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/S021348531600030X?idApp=UINPBA00004N"
      ]
    ]
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173580817300925?idApp=UINPBA00004N"
    "url" => "/21735808/0000003200000006/v1_201707180027/S2173580817300925/v1_201707180027/en/main.assets"
  ]
  "en" => array:20 [
    "idiomaDefecto" => true
    "cabecera" => "<span class="elsevierStyleTextfn">Original article</span>"
    "titulo" => "Analysis of Sturge&#8211;Weber syndrome&#58; A retrospective study of multiple associated variables"
    "tieneTextoCompleto" => true
    "paginas" => array:1 [
      0 => array:2 [
        "paginaInicial" => "363"
        "paginaFinal" => "370"
      ]
    ]
    "autores" => array:1 [
      0 => array:4 [
        "autoresLista" => "A&#46;I&#46; Mara&#241;a P&#233;rez, M&#46;L&#46; Ruiz-Falc&#243; Rojas, V&#46; Puertas Mart&#237;n, J&#46; Dom&#237;nguez Carral, I&#46; Carreras S&#225;ez, A&#46; Duat Rodr&#237;guez, V&#46; S&#225;nchez Gonz&#225;lez"
        "autores" => array:7 [
          0 => array:4 [
            "nombre" => "A&#46;I&#46;"
            "apellidos" => "Mara&#241;a P&#233;rez"
            "email" => array:1 [
              0 => "maranaai&#64;hotmail&#46;com"
            ]
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">&#42;</span>"
                "identificador" => "cor0005"
              ]
            ]
          ]
          1 => array:2 [
            "nombre" => "M&#46;L&#46;"
            "apellidos" => "Ruiz-Falc&#243; Rojas"
          ]
          2 => array:2 [
            "nombre" => "V&#46;"
            "apellidos" => "Puertas Mart&#237;n"
          ]
          3 => array:2 [
            "nombre" => "J&#46;"
            "apellidos" => "Dom&#237;nguez Carral"
          ]
          4 => array:2 [
            "nombre" => "I&#46;"
            "apellidos" => "Carreras S&#225;ez"
          ]
          5 => array:2 [
            "nombre" => "A&#46;"
            "apellidos" => "Duat Rodr&#237;guez"
          ]
          6 => array:2 [
            "nombre" => "V&#46;"
            "apellidos" => "S&#225;nchez Gonz&#225;lez"
          ]
        ]
        "afiliaciones" => array:1 [
          0 => array:2 [
            "entidad" => "Servicio de Neuropediatr&#237;a&#44; Hospital Infantil Universitario Ni&#241;o Jes&#250;s&#44; Madrid&#44; Spain"
            "identificador" => "aff0005"
          ]
        ]
        "correspondencia" => array:1 [
          0 => array:3 [
            "identificador" => "cor0005"
            "etiqueta" => "&#8270;"
            "correspondencia" => "Corresponding author&#46;"
          ]
        ]
      ]
    ]
    "titulosAlternativos" => array:1 [
      "es" => array:1 [
        "titulo" => "An&#225;lisis del s&#237;ndrome de Sturge-Weber&#58; estudio retrospectivo de m&#250;ltiples variables asociadas"
      ]
    ]
    "resumenGrafico" => array:2 [
      "original" => 0
      "multimedia" => array:7 [
        "identificador" => "fig0015"
        "etiqueta" => "Figure 3"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
          0 => array:4 [
            "imagen" => "gr3.jpeg"
            "Alto" => 1162
            "Ancho" => 960
            "Tamanyo" => 80477
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">T1-weighted brain MRI with contrast showing bilateral leptomeningeal angiomatosis&#46;</p>"
        ]
      ]
    ]
    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Sturge&#8211;Weber syndrome &#40;SWS&#41; is a congenital vascular disease characterised by a facial capillary malformation &#40;port-wine stain&#41; associated with venous and capillary malformations in the brain and eyes&#46; Alterations at other locations&#44; such as the buccal cavity or the respiratory tract&#44; can also be observed&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">This congenital disease is sporadic rather than hereditary&#44; although some familial cases have been described&#46;<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">2</span></a> Recently&#44; this syndrome has been associated with a mutation in the gene <span class="elsevierStyleItalic">GNAQ</span>&#46;<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">3</span></a> Its incidence is between 1&#47;50<span class="elsevierStyleHsp" style=""></span>000 and 1&#47;230<span class="elsevierStyleHsp" style=""></span>000 live births&#46;<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">4</span></a> Aetiology of the disease remains unknown&#44; although it has been hypothesised that capillary malformations are the result of somatic mutations in the ectoderm&#46;<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Clinical manifestations of this syndrome are numerous&#44; and a thorough knowledge of all of them is essential to determine the correct diagnosis&#44; follow-up&#44; and treatment to improve patients&#8217; quality of life&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">The aim of this study is to describe the clinical and epidemiological characteristics&#44; as well as the different treatments applied to a cohort of patients diagnosed with SWS at a tertiary hospital&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Material and methods</span><p id="par0025" class="elsevierStylePara elsevierViewall">We conducted a cross-sectional comparative retrospective study by reviewing the data of patients diagnosed with SWS between 1998 and 2013 in the paediatric neurology department at a tertiary hospital&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Data were collected by reviewing the clinical histories of patients diagnosed with SWS over the last 15 years and matching them to the International Classification of Diseases &#40;ICD-10&#41; criteria&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">We excluded patients with facial angioma but no confirmed intracranial lesion&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Data obtained from each patient&#39;s clinical history were as follows&#58;<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">-</span><p id="par0045" class="elsevierStylePara elsevierViewall">Epidemiological variables&#58; sex and age at symptom onset&#46;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">-</span><p id="par0050" class="elsevierStylePara elsevierViewall">Variables related to skin lesions&#58; presence or absence of facial angioma&#44; side affected by the lesion&#44; and skin lesions in other locations&#46;</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">-</span><p id="par0055" class="elsevierStylePara elsevierViewall">Variables related to intracranial lesions&#58; presence of leptomeningeal angiomatosis&#44; extension and location of the lesion&#44; affected side&#46;</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">-</span><p id="par0060" class="elsevierStylePara elsevierViewall">Variables related to ophthalmological symptoms&#58; presence or absence of eye involvement&#44; type of eye disorder&#44; and treatment used&#46;</p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">-</span><p id="par0065" class="elsevierStylePara elsevierViewall">Variables related to neurological symptoms&#58; presence or absence of epilepsy&#44; age at onset&#44; symptoms of epileptic seizures&#44; presence or absence of hemiparesis&#44; presence of psychomotor retardation&#44; associated mental retardation&#44; recurrent headache attacks&#44; stroke-like episodes&#46;</p></li><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">-</span><p id="par0070" class="elsevierStylePara elsevierViewall">Variables related to complementary tests and the results obtained&#58; neurophysiological studies&#44; neuroimaging studies &#40;computed tomography and&#47;or brain magnetic resonance imaging&#41;&#46;</p></li><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">-</span><p id="par0075" class="elsevierStylePara elsevierViewall">Variables related to antiepileptic treatment&#58; antiepileptic drugs&#44; epilepsy surgery&#46;</p></li><li class="elsevierStyleListItem" id="lsti0040"><span class="elsevierStyleLabel">-</span><p id="par0080" class="elsevierStylePara elsevierViewall">Variables related to other treatments&#58; antiplatelet drugs&#44; epilepsy surgery&#46;</p></li></ul></p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Results</span><p id="par0085" class="elsevierStylePara elsevierViewall">We included 13 patients &#40;54&#37; males and 46&#37; females&#41; diagnosed with SWS at our hospital between 1998 and 2013&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">Age at diagnosis of SWS ranged from one month to 27 months&#44; with a mean age of 15 months&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Leptomeningeal angiomatosis was present in all cases&#44; and it was most frequently located in the right hemisphere &#40;46&#37;&#41; than in the left hemisphere &#40;38&#37;&#41;&#46; Two patients displayed bilateral involvement &#40;15&#37;&#41;&#46;</p><p id="par0100" class="elsevierStylePara elsevierViewall">In our series&#44; the lesion most frequently identified was leptomeningeal angiomatosis with involvement of more than 3 lobes ipsilaterally or even contralaterally &#40;38&#46;5&#37;&#41;&#44; followed by involvement of 3 lobes &#40;31&#37;&#41;&#46; Lesions predominantly affected the parietal&#44; temporal&#44; and occipital lobes&#46; We found involvement of only 2 lobes in 23&#37; of the cases &#40;predominantly in the occipitotemporal lobes&#41; and only one patient with involvement of the occipital lobe exclusively&#46;</p><p id="par0105" class="elsevierStylePara elsevierViewall">Facial angioma was not present in all patients&#59; 4 of them &#40;30&#37;&#41; showed no angioma at any location&#46; Of the patients with facial angioma &#40;61&#37;&#41;&#44; 3 displayed right facial angioma &#40;23&#37;&#41;&#44; 5 on the left side &#40;38&#37;&#41;&#44; and only one showed bilateral facial angioma&#46; According to the literature&#44; facial angiomatosis most frequently affects the first branch of the trigeminal nerve&#59; however&#44; it was difficult to delimit the angiomatosis lesion in this group&#44; and this variable was therefore not included in our study&#46;</p><p id="par0110" class="elsevierStylePara elsevierViewall">In this series&#44; skin lesions at locations other than the face were present in 3 of the 13 patients &#40;23&#37;&#41;&#46; Two of them were ipsilateral to facial and leptomeningeal angiomatosis&#59; in the third case&#44; skin lesion appeared as generalised cutis marmorata&#46;</p><p id="par0115" class="elsevierStylePara elsevierViewall">Ocular involvement was identified in 77&#37; of the patients&#44; with glaucoma being the most frequent &#40;46&#37;&#41;&#44; followed by strabismus &#40;23&#37;&#41;&#44; and choroid angiomatosis &#40;23&#37;&#41;&#46; Other ocular signs found&#44; although less frequently&#44; were visual field alterations&#44; ophthalmoparesis&#44; and refractive errors&#46; Four patients &#40;30&#37;&#41; presented different eye defects throughout the course of the disease&#46;</p><p id="par0120" class="elsevierStylePara elsevierViewall">Regarding ophthalmological treatment&#44; 61&#46;5&#37; of the patients needed surgery &#40;to treat glaucoma in most cases&#41;&#59; the remaining 38&#46;5&#37; of the patients received conservative treatment&#46;</p><p id="par0125" class="elsevierStylePara elsevierViewall">Of the other neurological symptoms typical of this disease&#44; the most frequent was epilepsy&#44; present in 100&#37; of the cases&#46; Partial seizures &#40;simple or complex&#41; were the most frequent &#40;62&#37; of the cases&#41;&#44; followed by secondarily generalised partial seizures &#40;31&#37;&#41;&#44; and generalised seizures &#40;8&#37;&#41;&#46; Age of onset of the first seizure ranged from 2 months to 11 years&#44; with a mean of 26 months&#46; Eight children &#40;62&#37;&#41; presented their first seizure before the age of 12 months&#44; and 75&#37; of them experienced the first seizure at 6 months of age or younger&#59; the most repeated value was 4 months&#46;</p><p id="par0130" class="elsevierStylePara elsevierViewall">Control of epileptic seizures has been very heterogeneous in this group of patients&#44; since 31&#37; of the patients needed more than 3 different drugs to control epilepsy&#44; 15&#37; tried 3 different drugs&#44; 31&#37; tried 2 different drugs&#44; and only 23&#37; of the patients achieved good seizure control with monotherapy&#46;</p><p id="par0135" class="elsevierStylePara elsevierViewall">The most widely used antiepileptic drugs were oxcarbazepine&#44; valproic acid&#44; levetiracetam&#44; and topiramate&#44; with valproic acid used in 92&#37; of the patients&#44; followed by equal percentages of patients treated with oxcarbazepine and levetiracetam &#40;46&#37;&#41;&#46; The most widely used drug combinations were valproic acid and levetiracetam&#44; and valproic acid with oxcarbazepine&#46;</p><p id="par0140" class="elsevierStylePara elsevierViewall">In light of one patient&#39;s refractory epileptic seizures and symptoms of incipient cognitive impairment&#44; we decided to treat epilepsy with a left hemispherectomy&#46; The patient remains seizure-free to date&#46; The resected section was analysed by the anatomical pathology service&#46; Architecture seemed preserved at the cortical level&#44; with no clearly diminished neuronal component&#46; We observed a mild gliosis and scattered microcalcifications at both the cortical and white matter levels&#44; in addition to occasional perivascular distribution&#46;</p><p id="par0145" class="elsevierStylePara elsevierViewall">The most frequent findings from conventional electroencephalography &#40;EEG&#41; and video-EEG were spikes&#44; spike-waves&#44; or polyspike-wave discharges in the lobes affected by leptomeningeal angiomatosis&#59; these features were present in 46&#37; of the cases&#46; Other electrical manifestations included low-voltage areas &#40;31&#37;&#41;&#44; low-voltage-associated spike-waves &#40;15&#46;5&#37;&#41;&#44; and areas of slow wave activity &#40;8&#37;&#41;&#46;</p><p id="par0150" class="elsevierStylePara elsevierViewall">Other neurological symptoms of SWS detected in our series included hemiparesis&#44; manifesting in 39&#37; of these patients&#59; recurrent headache attacks&#44; in 39&#37; of the patients&#59; and stroke-like episodes in 23&#37;&#46;</p><p id="par0155" class="elsevierStylePara elsevierViewall">We should not overlook associated psychomotor and mental retardation as an additional neurological finding in this syndrome&#59; it is not clear whether they are caused by the disease itself or secondary to epileptic seizures&#46; In our series&#44; 54&#37; of the patients did not develop symptoms of psychomotor or mental retardation&#46; Among the 46&#37; with symptoms&#44; we observed that those presenting signs of psychomotor retardation in their first months would later have intelligence quotients of less than 70 according to psychometric tests&#46;</p><p id="par0160" class="elsevierStylePara elsevierViewall">All patients had at least one follow-up magnetic resonance imaging scan performed at some point in time&#59; scans revealed leptomeningeal calcifications in 85&#37; of the patients&#44; with all calcifications located in the angiomatosis area&#46; Subsequent imaging studies performed on these same patients showed increases in the number of calcifications in 70&#37; of them&#46; Other imaging findings in this group of patients included a case of hemimegalencephaly and polymicrogyria&#44; another with secondary atrophy of the right hemipons and hemi-midbrain with opercular syndrome&#44; and 3 patients with atrophy &#40;generalised cortical-subcortical&#44; perirolandic frontotemporal&#44; and left temporal&#41; &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figs&#46; 1 and 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0165" class="elsevierStylePara elsevierViewall">Regarding use of coadjuvant treatments&#44; several studies have shown that an antiplatelet drug&#44; acetylsalicylic acid&#44; is effective for decreasing thrombotic and stroke-like episodes in patients with SWS&#46; Fifty-four per cent of the patients in our series were treated with acetylsalicylic acid&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Discussion</span><p id="par0170" class="elsevierStylePara elsevierViewall">SWS was first mentioned in 1860 by Schirmer&#44; a German ophthalmologist&#44; but it was not fully described until 1879 by Sturge&#46; Forty-three years later&#44; in 1922&#44; Weber completed the list of signs by describing the radiological findings&#46;<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">5</span></a></p><p id="par0175" class="elsevierStylePara elsevierViewall">The manifestations of this syndrome are multiple&#59; one of its salient features is the port-wine stain or facial angiomatosis&#44; which is the most frequent vascular malformation&#46; It manifests in 0&#46;3&#37; of newborns&#44; but only a small percentage of children with haemangiomas also have SWS&#46; Facial angiomatosis is typically located on the eyelid and the forehead&#44; especially in the distribution of the first and second branch of the trigeminal nerve&#46;</p><p id="par0180" class="elsevierStylePara elsevierViewall">In newborns&#44; the lesion is flat and pink-coloured&#46; As the child ages&#44; the colour may deepen to dark red&#44; and vascular ectasia lesions within the lesion may cause superficial nodularity and blebs&#46; Facial angioma is not present in all patients with SWS<a class="elsevierStyleCrossRefs" href="#bib0195"><span class="elsevierStyleSup">6&#44;7</span></a>&#59; 30&#37; of the patients in our series had no angiomas at any location&#46; In contrast&#44; rather than affecting only one side of the face&#44; skin lesions may occasionally present bilaterally&#44; or even extend to the chest and the limbs&#46;<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">8</span></a> Our study includes 2 patients with angiomatosis on one side of the body &#40;ipsilateral to the facial and leptomeningeal lesions&#41; and one patient with a skin lesion manifesting as generalised cutis marmorata&#46;</p><p id="par0185" class="elsevierStylePara elsevierViewall">Several studies have demonstrated that pulsed laser light is effective for treating this type of lesions&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">9</span></a> However&#44; due to the high recurrence rate caused by vessel reperfusion&#44; researchers are investigating new techniques&#44; such as the combination of topical rapamycin and pulsed laser light which is providing good results&#46;<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">10</span></a> Cases of intraoral angiomatosis have also been reported&#46; It is present in up to 40&#37; of the cases and occasionally causes significant periodontal alterations that increase the risk of bleeding during dental procedures&#44;<a class="elsevierStyleCrossRef" href="#bib0220"><span class="elsevierStyleSup">11</span></a> and even during difficult intubations&#46;<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">12</span></a></p><p id="par0190" class="elsevierStylePara elsevierViewall">Leptomeningeal angiomatosis manifests in 10&#37; to 20&#37; of the cases with typical facial angioma&#44; generally on the same side&#46; All patients diagnosed with SWS at our hospital presented leptomeningeal angiomatosis&#44; with or without facial involvement&#46; Parietal and occipital regions are the most frequently affected&#44; although any area of the brain may be involved as our case series illustrates&#46; The occipital lobe was the most frequently affected lobe&#44; whether alone or associated with angiomatosis in other lobes &#40;involvement of more than 3 lobes&#58; 38&#46;5&#37;&#59; 3 lobes&#58; 31&#37;&#59; 2 lobes&#58; 23&#37;&#59; and one lobe&#58; 7&#46;7&#37;&#41;&#46; Regarding the side affected by the leptomeningeal angioma&#44; no significant differences have been observed between the 2 hemispheres&#46; Bilateral involvement was detected in 15&#37; of the patients &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; Intracranial venous malformations may present in the absence of facial angioma &#40;atypical SWS or type-III SWS&#41;&#44; as occurs in 30&#37; of our patients&#46;<a class="elsevierStyleCrossRefs" href="#bib0195"><span class="elsevierStyleSup">6&#44;13</span></a> Several hypotheses postulate that the angioma present in classic SWS is the result of insufficient retraction and limited maturation of the cephalic venous plexus during the first 3 months of foetal development&#46;<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">13</span></a> Other evidence suggests that angiomas are not static&#59; rather&#44; these lesions undergo angiogenic remodelling&#46; These findings may lead to the development of new therapeutic strategies for SWS&#46;<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">14</span></a></p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0195" class="elsevierStylePara elsevierViewall">Ocular signs are present in a large number of patients&#46; They usually manifest as visual field defects&#44; generally as homonymous hemianopsia&#44; due to the presence of angiomas affecting both occipital lobes or optic pathways&#46; The risk of glaucoma is higher in patients younger than 10&#44; and it occurs in 30&#37; to 70&#37; of those diagnosed with SWS&#46;<a class="elsevierStyleCrossRefs" href="#bib0240"><span class="elsevierStyleSup">15&#44;16</span></a> Continuous follow-up was necessary&#44; even for patients whose intraocular pressure was initially normal&#46;</p><p id="par0200" class="elsevierStylePara elsevierViewall">Other ophthalmological complications include choroidal haemangiomas&#44; vascular malformations in the episcleral lamina or conjunctiva&#44; and heterochromia&#46;</p><p id="par0205" class="elsevierStylePara elsevierViewall">In our series&#44; ocular involvement was identified in 77&#37; of the patients&#59; glaucoma &#40;46&#37;&#41; was the most frequent as reported in the literature&#44; followed by strabismus &#40;23&#37;&#41;&#44; and choroid angiomatosis &#40;23&#37;&#41;&#46; Only one patient displayed hemianopsia with no other eye disorder&#46; Four patients &#40;30&#37;&#41; presented different eye disorders as the condition progressed&#46; Several articles address the different treatments for eye disorders in SWS&#58; trabeculectomy for glaucoma&#44; photodynamic therapy&#44; etc&#46;<a class="elsevierStyleCrossRefs" href="#bib0240"><span class="elsevierStyleSup">15&#44;17</span></a> Only 61&#46;5&#37; of the patients in our series needed surgery &#40;mostly to treat glaucoma&#41;&#59; the other 38&#46;5&#37; of the patients received conservative treatment&#46;</p><p id="par0210" class="elsevierStylePara elsevierViewall">Epilepsy is the most important of the neurological alterations potentially associated with this syndrome&#46; Seizures are frequently the first symptom of SWS&#44; and they manifest in 75&#37; to 80&#37; of these patients&#46; While seizures may occur at any age&#44; onset usually occurs in early childhood&#46;<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">18</span></a> The initial seizures are typically focal&#44; but they often evolve to become generalised tonic-clonic seizures&#46; Infantile spasms&#44; myoclonic seizures&#44; or atonic seizures may also be present&#46; In our study&#44; epilepsy manifested in 100&#37; of the cases&#46; Partial seizures &#40;simple or complex&#41; were the most frequent &#40;62&#37; of the cases&#41;&#44; followed by secondarily generalised partial seizures &#40;31&#37;&#41; and generalised seizures &#40;8&#37;&#41;&#46; Age at seizure onset ranged from 2 months to 11 years&#44; with a mean age of 26 months&#46; Eight children &#40;62&#37;&#41; presented their first seizure before the age of 12 months&#44; and 75&#37; of them suffered the first seizure at 6 months of age or younger&#46;</p><p id="par0215" class="elsevierStylePara elsevierViewall">The degree of seizure control achieved with antiepileptics was variable and unpredictable&#46; Some patients experience long seizure-free periods&#44; even without medication&#44; whereas others suffer frequent or prolonged attacks despite taking high doses of various antiepileptics&#46; Seizure control is very heterogeneous in this group of patients&#44; considering that 31&#37; of the patients needed more than 3 different drugs to control epilepsy&#44; 15&#37; tried 3 drugs&#44; 31&#37; tried 2 drugs&#44; and only 23&#37; of the patients achieved good control with monotherapy&#46;</p><p id="par0220" class="elsevierStylePara elsevierViewall">Seizure onset&#44; age at symptom onset&#44; and response to treatment all affect prognosis&#46; Seizure onset in children younger than one year and poor response to antiepileptics are associated with a higher probability of cognitive impairment&#46;<a class="elsevierStyleCrossRef" href="#bib0245"><span class="elsevierStyleSup">16</span></a></p><p id="par0225" class="elsevierStylePara elsevierViewall">One theory to explain epileptogenesis in patients with SWS-associated epilepsy is chronic ischaemia in cortical areas affected by leptomeningeal angiomatosis&#46; Nevertheless&#44; we have found cases of patients with intractable epilepsy &#40;caused by SWS&#41; whose epileptogenic focus was an area of type-IIa focal cortical dysplasia near the area displaying leptomeningeal angiomatosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0260"><span class="elsevierStyleSup">19&#44;20</span></a></p><p id="par0230" class="elsevierStylePara elsevierViewall">Several articles have analysed surgical treatment of refractory epilepsy in these patients&#44; and particularly the use of hemispherectomy to achieve better seizure control&#46;<a class="elsevierStyleCrossRefs" href="#bib0270"><span class="elsevierStyleSup">21&#44;22</span></a> One patient in our study underwent left hemispherectomy to treat refractory epilepsy and symptoms of cognitive impairment&#59; results were excellent since no seizures occurred in the 7 months between the surgery and the date of data collection&#46;</p><p id="par0235" class="elsevierStylePara elsevierViewall">Hemiparesis&#44; present in 39&#37; of the cases analysed&#44; frequently develops in its acute form at the time of seizure onset&#46;<a class="elsevierStyleCrossRef" href="#bib0270"><span class="elsevierStyleSup">21</span></a> The paretic limb does not develop normally and becomes atrophied&#46; Affected children sometimes present progressive loss of motor function caused by an unknown mechanism&#46; One theory points to the cumulative effect of repeated thrombotic episodes inside the leptomeningeal malformation&#44; or chronic alteration of blood flow&#46; Numerous studies seem to have demonstrated the efficacy of low doses of acetylsalicylic acid for reducing thrombotic episodes in these patients&#46;<a class="elsevierStyleCrossRefs" href="#bib0280"><span class="elsevierStyleSup">23&#8211;25</span></a></p><p id="par0240" class="elsevierStylePara elsevierViewall">Patients with SWS may also develop stroke-like episodes that cause transient neurological deficits often lasting several days or weeks&#46;<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">18</span></a> Of the cases we studied&#44; 23&#37; had presented one or more stroke-like episodes at some point&#46; These episodes may occur spontaneously&#44; accompany a cluster of seizures&#44; or precede a migraine or a seizure&#46; Treatment with low doses of acetylsalicylic acid has also proved effective for reducing these episodes&#46;<a class="elsevierStyleCrossRefs" href="#bib0280"><span class="elsevierStyleSup">23&#8211;25</span></a> Fifty-four percent of children in our study needed treatment with antiplatelet drugs due to thrombotic or stroke-like episodes&#46; Subjective improvement was reported in the form of decreased frequency and severity&#46;</p><p id="par0245" class="elsevierStylePara elsevierViewall">Headaches&#44; predominantly migraine&#44; are also more frequent in the context of this syndrome and they manifest at earlier ages than in the general population&#46;<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">18</span></a> Our series reports recurrent headache attacks in 39&#37; of the patients&#46;</p><p id="par0250" class="elsevierStylePara elsevierViewall">Children with SWS usually develop normally in the first few months after birth&#44; but developmental delays will manifest at a later stage&#46;<a class="elsevierStyleCrossRef" href="#bib0295"><span class="elsevierStyleSup">26</span></a> Cognitive function is more limited in patients displaying bilateral intracerebral lesions&#46;<a class="elsevierStyleCrossRef" href="#bib0300"><span class="elsevierStyleSup">27</span></a> We found that 46&#37; of the children studied in our series presented signs of psychomotor retardation&#46; Subsequent psychometric testing showed total intelligence quotients of less than 70 in these children&#46;</p><p id="par0255" class="elsevierStylePara elsevierViewall">Poorer cognitive function and presence of epilepsy in patients with SWS is also associated with a higher risk of behavioural problems&#46; However&#44; behavioural problems may also occur in patients with normal intelligence&#46;<a class="elsevierStyleCrossRefs" href="#bib0305"><span class="elsevierStyleSup">28&#44;29</span></a></p><p id="par0260" class="elsevierStylePara elsevierViewall">Other symptoms that may be associated with SWS have been analysed in recent years&#46; For example&#44; researchers have observed that growth hormone deficiency is up to 18 times more frequent in these patients than in the general population&#46; This deficiency occurs with no neuroimaging evidence of pituitary or hypothalamic anomalies&#46;<a class="elsevierStyleCrossRef" href="#bib0315"><span class="elsevierStyleSup">30</span></a> Cases of partial hypopituitarism<a class="elsevierStyleCrossRef" href="#bib0320"><span class="elsevierStyleSup">31</span></a> and central hypothyroidism that may be related to the use of antiepileptics have also been reported&#46;<a class="elsevierStyleCrossRef" href="#bib0325"><span class="elsevierStyleSup">32</span></a> Since effective treatment for these symptoms exists&#44; we recommend testing to facilitate early diagnosis of these patients&#46;</p><p id="par0265" class="elsevierStylePara elsevierViewall">Diagnosis of SWS is based on the presence of facial angioma and leptomeningeal angiomatosis&#46; As mentioned earlier&#44; some patients with intracranial malformations and no facial lesions may also develop neurological problems&#46;</p><p id="par0270" class="elsevierStylePara elsevierViewall">The neuroimaging technique of choice for diagnosis is gadolinium contrast magnetic resonance imaging&#44; since it can reveal leptomeningeal angiomatosis and the extent to which it affects brain structures&#46;<a class="elsevierStyleCrossRef" href="#bib0330"><span class="elsevierStyleSup">33</span></a> In some cases&#44; leptomeningeal involvement cannot be detected by neuroimaging techniques during childhood and only becomes apparent later in life&#46;<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">2</span></a> All patients underwent at least one magnetic resonance imaging scan at some point&#59; we found leptomeningeal calcifications in 85&#37; of the patients&#44; all of them located in the area affected by angiomatosis&#46; Subsequent imaging studies of the same patients showed increases in the number of calcifications in 70&#37; of them&#46;</p><p id="par0275" class="elsevierStylePara elsevierViewall">When magnetic resonance imaging is not available&#44; a cranial computed tomography may be able to show brain calcifications and provide some anatomical information&#46; Single-photon emission computed tomography has been able to show decreased blood flow in the part of the brain affected by leptomeningeal angiomatosis&#46;<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">18</span></a></p><p id="par0280" class="elsevierStylePara elsevierViewall">Patients with SWS may display multiple radiological findings other than those previously mentioned&#46; These include lobe atrophy or atrophy at other locations &#40;in 4 of our patients&#41;&#44; choroid plexus hypertrophy&#44; venous flow anomalies&#44; prominent deep collateral venous system&#44; etc&#46;</p><p id="par0285" class="elsevierStylePara elsevierViewall">EEG is a non-invasive method of assessing brain function&#44; which can be repeated as frequently as necessary&#46;<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">18</span></a> The most common EEG abnormality is decreased voltage in the affected hemisphere&#44; but this was not the case in our study&#46; Among our patients&#44; the most frequent abnormalities in EEG studies were spikes&#44; spike-waves&#44; or polyspike-waves &#40;46&#37;&#41;&#44; followed by low-voltage areas &#40;31&#37;&#41;&#44; low-voltage-associated spike-waves &#40;15&#46;5&#37;&#41;&#44; and areas of slow wave activity &#40;8&#37;&#41;&#46;</p><p id="par0290" class="elsevierStylePara elsevierViewall">Ongoing studies with EEG examine whether this tool may be helpful in achieving better outcomes and selecting treatments&#46;<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">18</span></a></p><p id="par0295" class="elsevierStylePara elsevierViewall">Some studies of children with SWS have revealed that Doppler ultrasonography may be a useful non-invasive tool for assessing the severity of anomalies in blood flow and for monitoring changes over time&#46;<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">18</span></a></p><p id="par0300" class="elsevierStylePara elsevierViewall">Marler et al&#46; have developed vascular biomarkers &#40;increased metalloproteinases in urine&#41; and described non-invasive vascular profiles of patients with different vascular disorders&#44; including patients with SWS&#46; Continuous identification and validation of biomarkers of this syndrome will be a significant component of future therapeutic approaches&#46;<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">18</span></a></p><p id="par0305" class="elsevierStylePara elsevierViewall">There is no specific treatment for SWS&#46; Treatment of cutaneous&#44; ocular&#44; neurological&#44; and endocrine manifestations yields uneven results&#46; Understanding and managing antiepileptic treatment in these children is of vital importance&#44; since some anticonvulsant agents&#44; such as topiramate&#44; may cause acute angle-closure glaucoma and worsen the patient&#39;s prognosis&#46;<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">18</span></a></p><p id="par0310" class="elsevierStylePara elsevierViewall">There is increasing consensus that low doses of acetylsalicylic acid may be beneficial&#44; based on the idea that antithrombotic treatment may prevent the exacerbation of anomalies in cerebral blood flow and of the hypoxic-ischaemic brain lesion&#46;<a class="elsevierStyleCrossRefs" href="#bib0280"><span class="elsevierStyleSup">23&#8211;25</span></a> Furthermore&#44; there are other strategies for preventing stroke-like episodes&#44; including annual influenza vaccination&#44; maintaining good hydration during disease&#44; and treating fevers&#44; but these have not been thoroughly studied&#46;<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">18</span></a></p><p id="par0315" class="elsevierStylePara elsevierViewall">Prognosis in SWS depends on the extension of leptomeningeal angiomatosis and its effect on perfusion in the cerebral cortex&#44; as well as severity of eye impairment&#46; Prognosis also depends on age at seizure onset and on the effectiveness of seizure control&#46; Neurological functions may deteriorate with age&#46; As a result&#44; approximately half of the adults with SWS present impairment&#44; including those who were initially normal&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Conflicts of interest</span><p id="par0320" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
    "textoCompletoSecciones" => array:1 [
      "secciones" => array:10 [
        0 => array:3 [
          "identificador" => "xres868504"
          "titulo" => "Abstract"
          "secciones" => array:5 [
            0 => array:2 [
              "identificador" => "abst0005"
              "titulo" => "Introduction"
            ]
            1 => array:2 [
              "identificador" => "abst0010"
              "titulo" => "Objectives"
            ]
            2 => array:2 [
              "identificador" => "abst0015"
              "titulo" => "Material and methods"
            ]
            3 => array:2 [
              "identificador" => "abst0020"
              "titulo" => "Results"
            ]
            4 => array:2 [
              "identificador" => "abst0025"
              "titulo" => "Conclusions"
            ]
          ]
        ]
        1 => array:2 [
          "identificador" => "xpalclavsec857434"
          "titulo" => "Keywords"
        ]
        2 => array:3 [
          "identificador" => "xres868505"
          "titulo" => "Resumen"
          "secciones" => array:5 [
            0 => array:2 [
              "identificador" => "abst0030"
              "titulo" => "Introducci&#243;n"
            ]
            1 => array:2 [
              "identificador" => "abst0035"
              "titulo" => "Objetivos"
            ]
            2 => array:2 [
              "identificador" => "abst0040"
              "titulo" => "Material y m&#233;todos"
            ]
            3 => array:2 [
              "identificador" => "abst0045"
              "titulo" => "Resultados"
            ]
            4 => array:2 [
              "identificador" => "abst0050"
              "titulo" => "Conclusiones"
            ]
          ]
        ]
        3 => array:2 [
          "identificador" => "xpalclavsec857433"
          "titulo" => "Palabras clave"
        ]
        4 => array:2 [
          "identificador" => "sec0005"
          "titulo" => "Introduction"
        ]
        5 => array:2 [
          "identificador" => "sec0010"
          "titulo" => "Material and methods"
        ]
        6 => array:2 [
          "identificador" => "sec0015"
          "titulo" => "Results"
        ]
        7 => array:2 [
          "identificador" => "sec0020"
          "titulo" => "Discussion"
        ]
        8 => array:2 [
          "identificador" => "sec0025"
          "titulo" => "Conflicts of interest"
        ]
        9 => array:1 [
          "titulo" => "References"
        ]
      ]
    ]
    "pdfFichero" => "main.pdf"
    "tienePdf" => true
    "fechaRecibido" => "2014-12-22"
    "fechaAceptado" => "2015-12-21"
    "PalabrasClave" => array:2 [
      "en" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Keywords"
          "identificador" => "xpalclavsec857434"
          "palabras" => array:6 [
            0 => "Angiomatosis"
            1 => "Epilepsy"
            2 => "Leptomeningeal"
            3 => "Neurocutaneous"
            4 => "Stroke-like episode"
            5 => "Sturge&#8211;Weber"
          ]
        ]
      ]
      "es" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Palabras clave"
          "identificador" => "xpalclavsec857433"
          "palabras" => array:6 [
            0 => "Angiomatosis"
            1 => "Epilepsia"
            2 => "Leptomen&#237;ngea"
            3 => "Neurocut&#225;neo"
            4 => "Stroke-like"
            5 => "Sturge-Weber"
          ]
        ]
      ]
    ]
    "tieneResumen" => true
    "resumen" => array:2 [
      "en" => array:3 [
        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Introduction</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Sturge&#8211;Weber syndrome is a congenital vascular disorder characterised by facial capillary malformation &#40;port-wine stain&#41; associated with venous and capillary malformations in the brain and eye&#46; Neurological symptoms and alterations in other locations may also be observed&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Objectives</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">This study describes the clinical and epidemiological characteristics and different treatments in a cohort of patients diagnosed with Sturge&#8211;Weber syndrome in a tertiary hospital&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Material and methods</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">This comparative&#44; retrospective and cross-sectional study was conducted by reviewing the medical records of patients diagnosed with Sturge&#8211;Weber syndrome between 1998 and 2013&#46;</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Results</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">The study included 13 patients &#40;54&#37; male&#44; 46&#37; female&#41; diagnosed with Sturge&#8211;Weber syndrome&#46; The mean age at diagnosis was 15 months&#46; Leptomeningeal angiomatosis was present in 100&#37; of cases&#58; right hemisphere &#40;46&#37;&#41;&#44; left hemisphere &#40;38&#37;&#41;&#44; and bilateral &#40;15&#37;&#41;&#46; Facial angioma was present in 61&#37; of the cases&#58; right &#40;23&#37;&#41;&#44; left &#40;38&#37;&#41; and bilateral &#40;7&#37;&#41;&#46; Other skin disorders were found in 23&#37; of the cases&#44; including 2 with hemilateral involvement on the side where facial and leptomeningeal angiomatosis was present and one case of generalised cutis marmorata&#46; Ocular disease was found in 77&#37; of patients&#59; the most common conditions were glaucoma &#40;46&#37;&#41;&#44; strabismus &#40;23&#37;&#41; and choroidal angioma &#40;23&#37;&#41;&#46; Epilepsy was present in 100&#37; of the cases&#44; with partial seizures &#40;simple or complex&#41; being the most frequent &#40;62&#37;&#41;&#46; Seizure control was highly variable&#59; 31&#37; of the patients had needed to try more than 3 drugs&#44; 15&#37; 3 drugs&#44; and 31&#37; 2 drugs&#44; while 23&#37; experienced good seizure control with monotherapy&#46; One patient required surgery for epilepsy &#40;left hemispherectomy&#41; and has been seizure-free since then&#46; The most frequent observations in electroencephalograms were spikes&#44; polyspikes&#44; and wave spikes in the lobes affected by leptomeningeal angiomatosis &#40;46&#37;&#41;&#46; Other neurological symptoms were hemiparesis &#40;39&#37;&#41;&#44; recurrent headaches &#40;39&#37;&#41;&#44; stroke-like episodes &#40;23&#37;&#41;&#44; psychomotor retardation &#40;46&#37;&#41;&#44; and mental retardation &#40;46&#37;&#41;&#46; Leptomeningeal calcifications could be seen in 85&#37; of patient MRIs&#44; as well as increased calcification in 70&#37;&#59; 54&#37; of the patients had been treated with aspirin&#46;</p></span> <span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Conclusions</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">There are multiple clinical manifestations of Sturge&#8211;Weber syndrome&#46; Being familiar with all of them is vitally important for diagnosing and for monitoring and treating the condition correctly&#44; which will improve the quality of life of these patients&#46;</p></span>"
        "secciones" => array:5 [
          0 => array:2 [
            "identificador" => "abst0005"
            "titulo" => "Introduction"
          ]
          1 => array:2 [
            "identificador" => "abst0010"
            "titulo" => "Objectives"
          ]
          2 => array:2 [
            "identificador" => "abst0015"
            "titulo" => "Material and methods"
          ]
          3 => array:2 [
            "identificador" => "abst0020"
            "titulo" => "Results"
          ]
          4 => array:2 [
            "identificador" => "abst0025"
            "titulo" => "Conclusions"
          ]
        ]
      ]
      "es" => array:3 [
        "titulo" => "Resumen"
        "resumen" => "<span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Introducci&#243;n</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">El s&#237;ndrome de Sturge-Weber es un trastorno vascular cong&#233;nito caracterizado por una malformaci&#243;n facial capilar &#40;mancha en vino de Oporto&#41; asociada a malformaciones venosas y capilares en el cerebro y en el ojo&#46; Tambi&#233;n pueden observarse alteraciones en otras localizaciones y s&#237;ntomas neurol&#243;gicos&#46;</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Objetivos</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Describir las caracter&#237;sticas cl&#237;nicas y epidemiol&#243;gicas&#44; as&#237; como los diferentes tratamientos realizados en una cohorte de pacientes diagnosticados de s&#237;ndrome de Sturge-Weber en un hospital terciario&#46;</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Material y m&#233;todos</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Estudio comparativo&#44; retrospectivo y transversal&#44; mediante la revisi&#243;n de historias cl&#237;nicas de pacientes diagnosticados de s&#237;ndrome de Sturge-Weber entre los a&#241;os 1998 y 2013&#46;</p></span> <span id="abst0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Resultados</span><p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Se incluyeron 13 pacientes &#40;54&#37; varones&#44; 46&#37; mujeres&#41; diagnosticados de s&#237;ndrome de Sturge-Weber&#46; La edad media al diagn&#243;stico fue de 15 meses&#46; Presencia de angiomatosis leptomen&#237;ngea en el 100&#37; de los casos&#58; hemisferio derecho &#40;46&#37;&#41;&#44; hemisferio izquierdo &#40;38&#37;&#41;&#44; afectaci&#243;n bilateral &#40;15&#37;&#41;&#46; Presencia de angioma facial &#40;61&#37;&#41;&#58; derecho &#40;23&#37;&#41;&#44; izquierdo &#40;38&#37;&#41; y bilateral &#40;7&#37;&#41;&#46; Otras alteraciones cut&#225;neas&#58; 23&#37; de los casos &#40;2 de ellos la afectaci&#243;n en el hemicuerpo del lado en el que se encontraba tambi&#233;n la angiomatosis facial y leptomen&#237;ngea y en el otro caso la afectaci&#243;n cut&#225;nea fue en forma de cutis marmorata generalizada&#41;&#46; Encontramos afectaci&#243;n ocular en el 77&#37; de los pacientes&#44; siendo las m&#225;s frecuentes&#58; glaucoma &#40;46&#37;&#41;&#44; estrabismo &#40;23&#37;&#41; y angiomatosis coroidea &#40;23&#37;&#41;&#46; Presencia de epilepsia 100&#37; de los casos&#44; siendo las crisis parciales &#40;simples o complejas&#41; las m&#225;s frecuentes &#40;62&#37;&#41;&#46; El control de las crisis epil&#233;pticas fue muy variable&#44; ya que el 31&#37; han necesitado probar m&#225;s de 3 f&#225;rmacos&#44; 15&#37; 3 f&#225;rmacos&#44; 31&#37; 2 f&#225;rmacos y 23&#37; tuvieron buen control con monoterapia&#46; Uno de los pacientes requiri&#243; cirug&#237;a de la epilepsia &#40;hemisferectom&#237;a izquierda&#41;&#44; quedando libre de crisis hasta la fecha&#46; En electroencefalogramas lo m&#225;s frecuente fue&#58; puntas&#44; puntas ondas o polipuntas-ondas en los l&#243;bulos afectados por angiomatosis leptomen&#237;ngea &#40;46&#37;&#41;&#46; Otros s&#237;ntomas neurol&#243;gicos&#58; hemiparesia &#40;39&#37;&#41;&#44; cefaleas recurrentes &#40;39&#37;&#41;&#44; episodios <span class="elsevierStyleItalic">stroke-like</span> &#40;23&#37;&#41;&#44; retraso psicomotor &#40;46&#37;&#41;&#44; retraso mental &#40;46&#37;&#41;&#46; Presencia calcificaciones leptomen&#237;ngeas en la resonancia magn&#233;tica &#40;85&#37;&#41;&#46; Aumento de las calcificaciones en el 70&#37;&#46; Pacientes tratados con &#225;cido acetilsalic&#237;lico&#58; 54&#37;&#46;</p></span> <span id="abst0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conclusiones</span><p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Son m&#250;ltiples las manifestaciones cl&#237;nicas del s&#237;ndrome de Sturge-Weber&#44; siendo de vital importancia conocerlas todas para poder realizar un correcto diagn&#243;stico&#44; seguimiento y tratamiento de las mismas&#44; mejorando as&#237; la calidad de vida de estos pacientes&#46;</p></span>"
        "secciones" => array:5 [
          0 => array:2 [
            "identificador" => "abst0030"
            "titulo" => "Introducci&#243;n"
          ]
          1 => array:2 [
            "identificador" => "abst0035"
            "titulo" => "Objetivos"
          ]
          2 => array:2 [
            "identificador" => "abst0040"
            "titulo" => "Material y m&#233;todos"
          ]
          3 => array:2 [
            "identificador" => "abst0045"
            "titulo" => "Resultados"
          ]
          4 => array:2 [
            "identificador" => "abst0050"
            "titulo" => "Conclusiones"
          ]
        ]
      ]
    ]
    "NotaPie" => array:1 [
      0 => array:2 [
        "etiqueta" => "&#9734;"
        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Mara&#241;a P&#233;rez AI&#44; Ruiz-Falc&#243; Rojas ML&#44; Puertas Mart&#237;n V&#44; Dom&#237;nguez Carral J&#44; Carreras S&#225;ez I&#44; Duat Rodr&#237;guez A&#44; et al&#46; An&#225;lisis del s&#237;ndrome de Sturge-Weber&#58; estudio retrospectivo de m&#250;ltiples variables asociadas&#46; Neurolog&#237;a&#46; 2017&#59;32&#58;363&#8211;370&#46;</p>"
      ]
    ]
    "multimedia" => array:3 [
      0 => array:7 [
        "identificador" => "fig0005"
        "etiqueta" => "Figure 1"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
          0 => array:4 [
            "imagen" => "gr1.jpeg"
            "Alto" => 1132
            "Ancho" => 900
            "Tamanyo" => 71314
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">T1-weighted brain MRI with contrast showing leptomeningeal angiomatosis with secondary atrophy in the right occipitotemporal area&#46;</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" => 1036
            "Ancho" => 850
            "Tamanyo" => 63222
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">T1-weighted brain MRI with contrast showing leptomeningeal angiomatosis with atrophy in the left hemisphere&#46;</p>"
        ]
      ]
      2 => array:7 [
        "identificador" => "fig0015"
        "etiqueta" => "Figure 3"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
          0 => array:4 [
            "imagen" => "gr3.jpeg"
            "Alto" => 1162
            "Ancho" => 960
            "Tamanyo" => 80477
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">T1-weighted brain MRI with contrast showing bilateral leptomeningeal angiomatosis&#46;</p>"
        ]
      ]
    ]
    "bibliografia" => array:2 [
      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0005"
          "bibliografiaReferencia" => array:33 [
            0 => array:3 [
              "identificador" => "bib0170"
              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Sturge&#8211;Weber syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
                            0 => "N&#46; Manivannan"
                            1 => "S&#46; Gokulanathan"
                            2 => "R&#46;S&#46; Ahathya"
                            3 => "Gubernath"
                            4 => "R&#46; Daniel"
                            5 => "Shanmugasundaram"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.4103/0975-7406.100304"
                      "Revista" => array:7 [
                        "tituloSerie" => "J Pharm Bioallied Sci"
                        "fecha" => "2012"
                        "volumen" => "4"
                        "numero" => "Suppl&#46; 2"
                        "paginaInicial" => "S349"
                        "paginaFinal" => "S352"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23066288"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0175"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Presentation&#44; diagnosis&#44; pathophysiology&#44; and treatment of the neurological features of Sturge&#8211;Weber syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "A&#46;M&#46; Comi"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1097/NRL.0b013e318220c5b6"
                      "Revista" => array:5 [
                        "tituloSerie" => "Neurologist"
                        "fecha" => "2011"
                        "volumen" => "17"
                        "paginaInicial" => "179"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21712663"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib0180"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Sturge&#8211;Weber syndrome and port-wine stains caused by somatic mutation in GNAQ"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "M&#46;D&#46; Shirley"
                            1 => "H&#46; Tang"
                            2 => "C&#46;J&#46; Gallione"
                            3 => "J&#46;D&#46; Baugher"
                            4 => "L&#46;P&#46; Frelin"
                            5 => "B&#46; Cohen"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1056/NEJMoa1213507"
                      "Revista" => array:6 [
                        "tituloSerie" => "N Engl J Med"
                        "fecha" => "2013"
                        "volumen" => "368"
                        "paginaInicial" => "1971"
                        "paginaFinal" => "1979"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23656586"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            3 => array:3 [
              "identificador" => "bib0185"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "S&#237;ndrome de Sturge Weber"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "I&#46; Pascual Castroviejo"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "LibroEditado" => array:4 [
                        "titulo" => "Diagn&#243;stico cl&#237;nico-radiol&#243;gico en neurolog&#237;a infantil"
                        "paginaInicial" => "81"
                        "paginaFinal" => "87"
                        "serieFecha" => "1971"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            4 => array:3 [
              "identificador" => "bib0190"
              "etiqueta" => "5"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Atypical imaging evolution of Sturge&#8211;Weber syndrome without facial nevus"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "R&#46;R&#46; Luke"
                            1 => "S&#46;I&#46; Malik"
                            2 => "A&#46;W&#46; Hernandez"
                            3 => "D&#46;J&#46; Donahue"
                            4 => "M&#46;S&#46; Perry"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.pediatrneurol.2012.10.002"
                      "Revista" => array:6 [
                        "tituloSerie" => "Pediatr Neurol"
                        "fecha" => "2013"
                        "volumen" => "48"
                        "paginaInicial" => "143"
                        "paginaFinal" => "145"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23337009"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            5 => array:3 [
              "identificador" => "bib0195"
              "etiqueta" => "6"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Clinical features of Sturge&#8211;Weber syndrome without facial nevus&#58; five novel cases"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "L&#46; Siri"
                            1 => "L&#46; Giordano"
                            2 => "P&#46; Accorsi"
                            3 => "M&#46; Cossu"
                            4 => "L&#46; Pinelli"
                            5 => "L&#46; Tassi"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.ejpn.2012.06.011"
                      "Revista" => array:6 [
                        "tituloSerie" => "Eur J Paediatr Neurol"
                        "fecha" => "2013"
                        "volumen" => "17"
                        "paginaInicial" => "91"
                        "paginaFinal" => "96"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22819211"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            6 => array:3 [
              "identificador" => "bib0200"
              "etiqueta" => "7"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Sturge&#8211;Weber syndrome with bilateral facial nevus and early cerebral calcification"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:2 [
                            0 => "S&#46;A&#46; Zaki"
                            1 => "V&#46; Lad"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.4103/1817-1745.92825"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Pediatr Neurosci"
                        "fecha" => "2011"
                        "volumen" => "6"
                        "paginaInicial" => "114"
                        "paginaFinal" => "115"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22408657"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            7 => array:3 [
              "identificador" => "bib0205"
              "etiqueta" => "8"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Periodontal manifestations and ambulatorial management in a patient with sturge Weber syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "O&#46; Pagin"
                            1 => "N&#46;B&#46; del Neri"
                            2 => "P&#46; Battisti Mde"
                            3 => "A&#46;L&#46; Capelozza"
                            4 => "P&#46;S&#46; Santos"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1097/SCS.0b013e318271016c"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Craniofac Surg"
                        "fecha" => "2012"
                        "volumen" => "23"
                        "paginaInicial" => "1809"
                        "paginaFinal" => "1811"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23147344"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            8 => array:3 [
              "identificador" => "bib0210"
              "etiqueta" => "9"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Sturge&#8211;Weber-syndrome with extreme ocular manifestation and rare association of upper airway angioma with anticipated difficult airway"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "H&#46;S&#46; Wong"
                            1 => "R&#46; Abdul Rahman"
                            2 => "S&#46;Y&#46; Choo"
                            3 => "N&#46; Yahya"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Med J Malaysia"
                        "fecha" => "2012"
                        "volumen" => "67"
                        "paginaInicial" => "435"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23082461"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            9 => array:3 [
              "identificador" => "bib0215"
              "etiqueta" => "10"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Atypical Sturge&#8211;Weber syndrome requires confirmation"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "C&#46; Zhou"
                            1 => "L&#46; Zhu"
                            2 => "J&#46; Wu"
                            3 => "S&#46; Fang"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:2 [
                        "tituloSerie" => "Eur J Paediatr Neurol"
                        "fecha" => "2013"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            10 => array:3 [
              "identificador" => "bib0220"
              "etiqueta" => "11"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Upregulation of hypoxia-inducible factor &#40;HIF&#41;-1alpha and HIF-2alpha in leptomeningeal vascular malformations of Sturge&#8211;Weber syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "A&#46; JanComati"
                            1 => "H&#46; Beck"
                            2 => "W&#46; Halliday"
                            3 => "G&#46;J&#46; Snipes"
                            4 => "K&#46;H&#46; Plate"
                            5 => "T&#46; Acker"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1097/nen.0b013e31802d9011"
                      "Revista" => array:5 [
                        "tituloSerie" => "J Neuropathol Exp Neurol"
                        "fecha" => "2007"
                        "volumen" => "66"
                        "paginaInicial" => "86"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17204940"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            11 => array:3 [
              "identificador" => "bib0225"
              "etiqueta" => "12"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Congenital glaucoma from Sturge&#8211;Weber syndrome&#58; a modified surgical approach"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:2 [
                            0 => "N&#46;S&#46; Yuen"
                            1 => "I&#46;Y&#46; Wong"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.3341/kjo.2012.26.6.481"
                      "Revista" => array:6 [
                        "tituloSerie" => "Korean J Ophthalmol"
                        "fecha" => "2012"
                        "volumen" => "26"
                        "paginaInicial" => "481"
                        "paginaFinal" => "484"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23204808"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            12 => array:3 [
              "identificador" => "bib0230"
              "etiqueta" => "13"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Sturge&#8211;Weber syndrome&#58; age of onset of seizures and glaucoma and the prognosis for affected children"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:2 [
                            0 => "E&#46; Sujansky"
                            1 => "S&#46; Conradi"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1177/088307389501000113"
                      "Revista" => array:5 [
                        "tituloSerie" => "J Child Neurol"
                        "fecha" => "1995"
                        "volumen" => "10"
                        "paginaInicial" => "49"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/7769179"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            13 => array:3 [
              "identificador" => "bib0235"
              "etiqueta" => "14"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Multifocal photodynamic therapy for diffuse choroidal hemangioma"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:2 [
                            0 => "M&#46; Ang"
                            1 => "S&#46;Y&#46; Lee"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.2147/OPTH.S35471"
                      "Revista" => array:6 [
                        "tituloSerie" => "Clin Ophthalmol"
                        "fecha" => "2012"
                        "volumen" => "6"
                        "paginaInicial" => "1467"
                        "paginaFinal" => "1469"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23055665"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            14 => array:3 [
              "identificador" => "bib0240"
              "etiqueta" => "15"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Updates and future horizons on the understanding&#44; diagnosis&#44; and treatment of Sturge&#8211;Weber syndrome brain involvement"
                      "autores" => array:1 [
                        0 => array:3 [
                          "colaboracion" => "Brain Vascular Malformation Consortium National Sturge Weber Syndrome Workgroup"
                          "etal" => true
                          "autores" => array:6 [
                            0 => "W&#46; Lo"
                            1 => "D&#46;A&#46; Marchuk"
                            2 => "K&#46;L&#46; Ball"
                            3 => "C&#46; Juhasz"
                            4 => "L&#46;C&#46; Jordan"
                            5 => "J&#46;B&#46; Ewen"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1111/j.1469-8749.2011.04169.x"
                      "Revista" => array:6 [
                        "tituloSerie" => "Dev Med Child Neurol"
                        "fecha" => "2012"
                        "volumen" => "54"
                        "paginaInicial" => "214"
                        "paginaFinal" => "223"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22191476"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            15 => array:3 [
              "identificador" => "bib0245"
              "etiqueta" => "16"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Focal cortical dysplasia type IIa underlying epileptogenesis in patients with epilepsy associated with Sturge&#8211;Weber syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "M&#46; Murakami"
                            1 => "T&#46; Morioka"
                            2 => "S&#46;O&#46; Suzuki"
                            3 => "K&#46; Hashiguchi"
                            4 => "T&#46; Amano"
                            5 => "A&#46; Sakata"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1111/j.1528-1167.2012.03628.x"
                      "Revista" => array:6 [
                        "tituloSerie" => "Epilepsia"
                        "fecha" => "2012"
                        "volumen" => "53"
                        "paginaInicial" => "e184"
                        "paginaFinal" => "e188"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22905723"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            16 => array:3 [
              "identificador" => "bib0250"
              "etiqueta" => "17"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Medically intractable epilepsy in Sturge&#8211;Weber syndrome is associated with cortical malformation&#58; implications for surgical therapy"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "B&#46; Maton"
                            1 => "P&#46; Krsek"
                            2 => "P&#46; Jayakar"
                            3 => "T&#46; Resnick"
                            4 => "M&#46; Koehn"
                            5 => "G&#46; Morrison"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1111/j.1528-1167.2009.02304.x"
                      "Revista" => array:6 [
                        "tituloSerie" => "Epilepsia"
                        "fecha" => "2010"
                        "volumen" => "51"
                        "paginaInicial" => "257"
                        "paginaFinal" => "267"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/19780796"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            17 => array:3 [
              "identificador" => "bib0255"
              "etiqueta" => "18"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Pediatric functional hemispherectomy&#58; outcome in 92 patients"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "J&#46; Schramm"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "Acta Neurochir &#40;Wien&#41;"
                        "fecha" => "2012"
                        "volumen" => "154"
                        "paginaInicial" => "2017"
                        "paginaFinal" => "2028"
                        "itemHostRev" => array:3 [
                          "pii" => "S0955395913001679"
                          "estado" => "S300"
                          "issn" => "09553959"
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            18 => array:3 [
              "identificador" => "bib0260"
              "etiqueta" => "19"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Sturge&#8211;Weber syndrome&#58; introduction and overview"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:2 [
                            0 => "J&#46;B&#46; Bodensteiner"
                            1 => "E&#46;S&#46; Roach"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "LibroEditado" => array:3 [
                        "titulo" => "Sturge&#8211;Weber syndrome"
                        "edicion" => "2nd ed&#46;"
                        "serieFecha" => "2010"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            19 => array:3 [
              "identificador" => "bib0265"
              "etiqueta" => "20"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Aspirin use in Sturge&#8211;Weber syndrome&#58; side effects and clinical outcomes"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "E&#46;I&#46; Lance"
                            1 => "A&#46;K&#46; Sreenivasan"
                            2 => "T&#46;A&#46; Zabel"
                            3 => "E&#46;H&#46; Kossoff"
                            4 => "A&#46;M&#46; Comi"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1177/0883073812463607"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Child Neurol"
                        "fecha" => "2013"
                        "volumen" => "28"
                        "paginaInicial" => "213"
                        "paginaFinal" => "218"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23112247"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            20 => array:3 [
              "identificador" => "bib0270"
              "etiqueta" => "21"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Survey of aspirin use in Sturge&#8211;Weber syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "M&#46;J&#46; Bay"
                            1 => "C&#46;U&#46; Lehmann"
                            2 => "T&#46;A&#46; Zabel"
                            3 => "A&#46;M&#46; Comi"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1177/0883073810388646"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Child Neurol"
                        "fecha" => "2011"
                        "volumen" => "26"
                        "paginaInicial" => "692"
                        "paginaFinal" => "702"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21427442"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            21 => array:3 [
              "identificador" => "bib0275"
              "etiqueta" => "22"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Case report of subdural hematoma in a patient with Sturge&#8211;Weber syndrome and literature review&#58; questions and implications for therapy"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "J&#46; Lopez"
                            1 => "K&#46;W&#46; Yeom"
                            2 => "A&#46;M&#46; Comi"
                            3 => "K&#46; Van Haren"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1177/0883073812449514"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Child Neurol"
                        "fecha" => "2013"
                        "volumen" => "28"
                        "paginaInicial" => "672"
                        "paginaFinal" => "675"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22805242"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            22 => array:3 [
              "identificador" => "bib0280"
              "etiqueta" => "23"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Sturge&#8211;Weber syndrome&#58; study of 40 patients"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "I&#46; Pascual-Castroviejo"
                            1 => "C&#46; D&#237;az-Gonzalez"
                            2 => "R&#46;M&#46; Garc&#237;a-Melian"
                            3 => "I&#46; Gonz&#225;lez Casado"
                            4 => "E&#46; Mu&#241;oz Hiraldo"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Pediat Neurol"
                        "fecha" => "1993"
                        "volumen" => "9"
                        "paginaInicial" => "283"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/8216540"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            23 => array:3 [
              "identificador" => "bib0285"
              "etiqueta" => "24"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Prognosis in Sturge&#8211;Weber disease&#58; comparison of unihemispheric and bihemispheric involvement"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:2 [
                            0 => "E&#46;M&#46; Bebin"
                            1 => "M&#46;R&#46; Gomez"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1177/088307388800300306"
                      "Revista" => array:5 [
                        "tituloSerie" => "J Child Neurol"
                        "fecha" => "1988"
                        "volumen" => "3"
                        "paginaInicial" => "181"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/3209844"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            24 => array:3 [
              "identificador" => "bib0290"
              "etiqueta" => "25"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Psychological functioning in children and adolescents with Sturge&#8211;Weber syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "L&#46; Chapieski"
                            1 => "A&#46; Friedman"
                            2 => "D&#46; Lachar"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1177/088307380001501004"
                      "Revista" => array:5 [
                        "tituloSerie" => "J Child Neurol"
                        "fecha" => "2000"
                        "volumen" => "15"
                        "paginaInicial" => "660"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/11063079"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            25 => array:3 [
              "identificador" => "bib0295"
              "etiqueta" => "26"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Behavioral and academic problems in children with Sturge&#8211;Weber syndrome&#58; differences between children with and without seizures"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "D&#46; Raches"
                            1 => "M&#46; Hiscock"
                            2 => "L&#46; Chapieski"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.yebeh.2012.06.004"
                      "Revista" => array:6 [
                        "tituloSerie" => "Epilepsy Behav"
                        "fecha" => "2012"
                        "volumen" => "25"
                        "paginaInicial" => "457"
                        "paginaFinal" => "463"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23000106"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            26 => array:3 [
              "identificador" => "bib0300"
              "etiqueta" => "27"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Growth hormone deficiency in Sturge&#8211;Weber syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "R&#46;S&#46; Miller"
                            1 => "K&#46;L&#46; Ball"
                            2 => "A&#46;M&#46; Comi"
                            3 => "E&#46;L&#46; Germain-Lee"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1136/adc.2005.082578"
                      "Revista" => array:5 [
                        "tituloSerie" => "Arch Dis Child"
                        "fecha" => "2006"
                        "volumen" => "91"
                        "paginaInicial" => "340"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16551788"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            27 => array:3 [
              "identificador" => "bib0305"
              "etiqueta" => "28"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Sturge&#8211;Weber syndrome&#58; presentation with partial hypopituitarism"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
                            0 => "S&#46;K&#46; Kota"
                            1 => "L&#46;K&#46; Meher"
                            2 => "S&#46;K&#46; Kota"
                            3 => "S&#46; Jammula"
                            4 => "S&#46;V&#46; Krishna"
                            5 => "K&#46;D&#46; Modi"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1515/jpem-2012-0056"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Pediatr Endocrinol Metab"
                        "fecha" => "2012"
                        "volumen" => "25"
                        "paginaInicial" => "785"
                        "paginaFinal" => "789"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23155711"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            28 => array:3 [
              "identificador" => "bib0310"
              "etiqueta" => "29"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Importance of utilizing a sensitive free thyroxine assay in Sturge&#8211;Weber syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "L&#46; Siddique"
                            1 => "A&#46;M&#46; Comi"
                            2 => "E&#46;L&#46; Germain-Lee"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1177/0883073812463606"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Child Neurol"
                        "fecha" => "2013"
                        "volumen" => "28"
                        "paginaInicial" => "269"
                        "paginaFinal" => "274"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23112245"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            29 => array:3 [
              "identificador" => "bib0315"
              "etiqueta" => "30"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Magnetic resonance imaging&#44; its role in the neuroradiologic evaluation of neurofibromatosis&#44; tuberous sclerosis&#44; and Sturge&#8211;Weber syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:2 [
                            0 => "A&#46;P&#46; Truhan"
                            1 => "P&#46;A&#46; Filipek"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Arch Dermatol"
                        "fecha" => "1993"
                        "volumen" => "129"
                        "paginaInicial" => "219"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/8434982"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            30 => array:3 [
              "identificador" => "bib0320"
              "etiqueta" => "31"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Pulsed dye laser for Sturge&#8211;Weber syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "C&#46; L&#233;aut&#233;-Labr&#233;ze"
                            1 => "F&#46; Boralevi"
                            2 => "J&#46;M&#46; Pedespan"
                            3 => "Y&#46; Meymat"
                            4 => "A&#46; Taieb"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "Arch Dis Child"
                        "fecha" => "2002"
                        "volumen" => "87"
                        "paginaInicial" => "434"
                        "paginaFinal" => "435"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12390926"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            31 => array:3 [
              "identificador" => "bib0325"
              "etiqueta" => "32"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Topical rapamycin combined with pulsed dye laser in the treatment of capillary vascular malformations in Sturge&#8211;Weber syndrome&#58; phase II&#44; randomized&#44; double-blind&#44; intraindividual placebo-controlled clinical trial"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "L&#46; Marqu&#233;s"
                            1 => "J&#46;M&#46; N&#250;&#241;ez-C&#243;rdoba"
                            2 => "L&#46; Aguado"
                            3 => "M&#46; Pretel"
                            4 => "P&#46; Boixeda"
                            5 => "E&#46; Nagore"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.jaad.2014.10.011"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Am Acad Dermatol"
                        "fecha" => "2015"
                        "volumen" => "72"
                        "paginaInicial" => "151"
                        "paginaFinal" => "158"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/25455610"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            32 => array:3 [
              "identificador" => "bib0330"
              "etiqueta" => "33"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "S&#237;ndrome de Sturge&#8211;Weber&#46; Presentaci&#243;n de un caso con manifestaciones dermatol&#243;gicas m&#237;nimas"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "C&#46; Rodofile"
                            1 => "S&#46;A&#46; Grees"
                            2 => "L&#46;E&#46; Valle"
                            3 => "G&#46; Martino"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1590/S0325-00752011000200016"
                      "Revista" => array:6 [
                        "tituloSerie" => "Arch Argent Pediatr"
                        "fecha" => "2011"
                        "volumen" => "109"
                        "paginaInicial" => "e42"
                        "paginaFinal" => "e45"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21465064"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
          ]
        ]
      ]
    ]
  ]
  "idiomaDefecto" => "en"
  "url" => "/21735808/0000003200000006/v1_201707180027/S2173580817300913/v1_201707180027/en/main.assets"
  "Apartado" => array:4 [
    "identificador" => "9491"
    "tipo" => "SECCION"
    "en" => array:2 [
      "titulo" => "Original Articles"
      "idiomaDefecto" => true
    ]
    "idiomaDefecto" => "en"
  ]
  "PDF" => "https://static.elsevier.es/multimedia/21735808/0000003200000006/v1_201707180027/S2173580817300913/v1_201707180027/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/"
  "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173580817300913?idApp=UINPBA00004N"
]
Article information
ISSN: 21735808
Original language: English
The statistics are updated each day
Year/Month Html Pdf Total
2024 November 9 1 10
2024 October 78 11 89
2024 September 117 16 133
2024 August 74 16 90
2024 July 56 17 73
2024 June 68 7 75
2024 May 64 18 82
2024 April 72 15 87
2024 March 91 12 103
2024 February 111 12 123
2024 January 106 14 120
2023 December 101 34 135
2023 November 103 13 116
2023 October 124 27 151
2023 September 75 16 91
2023 August 63 8 71
2023 July 93 14 107
2023 June 93 8 101
2023 May 144 37 181
2023 April 107 33 140
2023 March 122 35 157
2023 February 86 24 110
2023 January 87 27 114
2022 December 62 15 77
2022 November 77 22 99
2022 October 78 30 108
2022 September 85 36 121
2022 August 95 37 132
2022 July 72 20 92
2022 June 73 13 86
2022 May 114 20 134
2022 April 108 20 128
2022 March 143 20 163
2022 February 162 9 171
2022 January 103 11 114
2021 December 69 21 90
2021 November 54 17 71
2021 October 105 28 133
2021 September 76 20 96
2021 August 93 16 109
2021 July 68 22 90
2021 June 110 21 131
2021 May 105 16 121
2021 April 369 66 435
2021 March 274 19 293
2021 February 227 20 247
2021 January 91 30 121
2020 December 85 17 102
2020 November 82 10 92
2020 October 94 14 108
2020 September 79 11 90
2020 August 75 17 92
2020 July 91 22 113
2020 June 76 20 96
2020 May 65 19 84
2020 April 72 17 89
2020 March 84 10 94
2020 February 90 19 109
2020 January 71 13 84
2019 December 67 22 89
2019 November 62 25 87
2019 October 62 16 78
2019 September 62 18 80
2019 August 49 13 62
2019 July 56 21 77
2019 June 127 38 165
2019 May 306 41 347
2019 April 129 29 158
2019 March 35 27 62
2019 February 39 18 57
2019 January 39 17 56
2018 December 22 10 32
2018 November 31 5 36
2018 October 29 24 53
2018 September 39 9 48
2018 August 17 9 26
2018 July 20 7 27
2018 June 27 4 31
2018 May 21 6 27
2018 April 11 10 21
2018 March 17 4 21
2018 February 13 4 17
2018 January 17 2 19
2017 December 16 7 23
2017 November 20 2 22
2017 October 9 4 13
2017 September 21 6 27
2017 August 30 9 39
2017 July 25 9 34
2017 June 0 12 12
Show all

Follow this link to access the full text of the article

es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos