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RM axial FLAIR (12 meses después del inicio de los síntomas): áreas difusas hiperintensas corticosubcorticales en los lóbulos parietal y temporal. 2.a, 2.b, 3.a. y 3.b) RM axial FLAIR (26 meses y 8 años después del inicio de la clínica, respectivamente): evolución progresiva, con atrofia cerebral y de tronco severas y lesión difusa y extensa de la sustancia blanca periventricular.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "R. Martins, J. Peres, C. Casimiro, A. Valverde" "autores" => array:4 [ 0 => array:2 [ "nombre" => "R." "apellidos" => "Martins" ] 1 => array:2 [ "nombre" => "J." "apellidos" => "Peres" ] 2 => array:2 [ "nombre" => "C." "apellidos" => "Casimiro" ] 3 => array:2 [ "nombre" => "A." 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"apellidos" => "Ruiz-Morales" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0213485317300312" "doi" => "10.1016/j.nrl.2017.01.008" "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/S0213485317300312?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S217358081830018X?idApp=UINPBA00004N" "url" => "/21735808/0000003300000003/v1_201804060407/S217358081830018X/v1_201804060407/en/main.assets" ] "en" => array:15 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Letter to the Editor</span>" "titulo" => "Subacute sclerosing panencephalitis presenting as rapidly progressive dementia in a young adult" "tieneTextoCompleto" => true "saludo" => "Dear Editor:" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "206" "paginaFinal" => "207" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "R. Martins, J. Peres, C. Casimiro, A. Valverde" "autores" => array:4 [ 0 => array:4 [ "nombre" => "R." "apellidos" => "Martins" "email" => array:1 [ 0 => "ritadossantosmartins@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "J." "apellidos" => "Peres" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "C." "apellidos" => "Casimiro" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "A." "apellidos" => "Valverde" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Prof. Doutor Fernando Fonseca, Amadora, Lisboa, Portugal" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Neurorradiología, Hospital Prof. Doutor Fernando Fonseca, Amadora, Lisboa, Portugal" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "CEDOC, Nova Medical School, Universidade Nova de Lisboa, Lisboa, Portugal" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Demencia rápidamente progresiva en adulto joven como forma de presentación de panencefalitis esclerosante subaguda" ] ] "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" => 1542 "Ancho" => 1867 "Tamanyo" => 362325 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(1a and 1b) Axial FLAIR MR images (12 months after symptom onset) showing diffuse hyperintensities in cortical and subcortical regions of the parietal and temporal lobes. (2a/b, 3a/b) Axial FLAIR MR images (26 months and 8 years after symptom onset, respectively) revealing disease progression, with severe cerebellar and brainstem atrophy and a diffuse, extensive lesion to the periventricular white matter.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Subacute sclerosing panencephalitis (SSP) is a neurodegenerative disorder caused by persistent infection with measles virus.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a> It is characterised by progressive cognitive and motor impairment and associated with myoclonus.<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">1,2</span></a> The condition is rarely observed in adult patients; when this does occur, SSP manifests with atypical symptoms.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of a 25-year-old man with no relevant medical history who developed behavioural alterations and cognitive deficits over the course of 3 years due to SSP. The initial neurological examination revealed inattention, impaired executive function, poor language fluency, motor perseveration, predominantly right-sided ideomotor apraxia, asomatognosia, agraphaesthesia, and right hemiparesis. A brain MRI scan revealed an extensive left-sided cortico-subcortical temporoparietal lesion, as well as other smaller lesions in the bilateral subcortical white matter on T2-weighted and FLAIR sequences; lesions showed no contrast uptake and the results of a cerebral angiography study were normal (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>: 1a and 1b). Serial electroencephalography revealed diffuse slowing, with theta rhythm in posterior regions and no periodic activity.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">We excluded prion disease, autoimmune encephalopathy, inflammatory vasculopathy, and progressive multifocal leukoencephalopathy. The results from serology tests and autoimmune studies were negative. A lumbar puncture yielded the following results: one red blood cell, 51<span class="elsevierStyleHsp" style=""></span>mg/dL glucose, 54<span class="elsevierStyleHsp" style=""></span>mg/dL proteins, and a high IgG index (1.74) with presence of oligoclonal bands. Results from CSF polymerase chain reaction for <span class="elsevierStyleItalic">Tropheryma whippelii</span> and herpes simplex, varicella-zoster, and JC viruses; syphilis serology; and 14-3-3 protein determination were all negative. Radiological studies and CSF anti-neuronal antibody determinations revealed no tumours.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The clinical, imaging, and laboratory findings led to suspicion of SSP; diagnosis was confirmed by high anti-measles virus antibody titres in the serum and CSF (1:10 and 1:134, respectively). The patient had no history of infection or exposure to measles virus and had been vaccinated against the virus. In the following 6 months, the patient's clinical symptoms worsened progressively: he displayed myoclonus in the form of periodic supraversion eye movements and refractory tonic-clonic seizures. A follow-up MRI scan revealed severe cerebellar and brainstem atrophy (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>: 2a, 2b, 3a, and 3b). The patient is currently in a vegetative state, displaying akinetic mutism.</p><p id="par0025" class="elsevierStylePara elsevierViewall">SSP typically presents during the first and second decades of life; only 100 cases of adult-onset SSP are described in the literature.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">4</span></a> Incidence of the condition decreased by 90% after the introduction of the measles vaccine into the vaccination schedule.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">5</span></a> A causal relationship between measles vaccination and SSP has not been determined. The cases of SSP described in patients vaccinated against measles virus are thought to be due to subclinical measles infection within the first year of life.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">As in our case, most cases of adult-onset SSP are not associated with a history of infection or exposure to measles virus.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a> Atypical symptoms are also more frequent in adult-onset SSP.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">2,3</span></a> Our patient experienced progressive motor and cognitive impairment without generalised myoclonus or periodic EEG activity; these findings are typical in advanced stages of the disease.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a> High anti-measles virus antibody titres in the serum and CSF confirm the diagnosis of SSP due to their high sensitivity, specificity, and positive predictive value.<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">1,2</span></a> Death usually occurs 1-3 years after symptom onset; there is currently no effective treatment for the condition.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">2</span></a> A combination of intrathecal interferon alfa and oral isoprinosine has shown an efficacy of 30% to 35% in clinical trials.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">6,7</span></a> Our patient did not start treatment due to the advanced stage of the condition.</p><p id="par0035" class="elsevierStylePara elsevierViewall">In conclusion, a diagnosis of SSP must be considered in young patients with progressive cognitive impairment. Atypical symptoms are frequent in adult-onset SSP, especially in cases with compatible serology and imaging findings.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Martins R, Peres J, Casimiro C, Valverde A. Demencia rápidamente progresiva en adulto joven como forma de presentación de panencefalitis esclerosante subaguda. Neurología. 2018;33:206–207.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1542 "Ancho" => 1867 "Tamanyo" => 362325 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(1a and 1b) Axial FLAIR MR images (12 months after symptom onset) showing diffuse hyperintensities in cortical and subcortical regions of the parietal and temporal lobes. (2a/b, 3a/b) Axial FLAIR MR images (26 months and 8 years after symptom onset, respectively) revealing disease progression, with severe cerebellar and brainstem atrophy and a diffuse, extensive lesion to the periventricular white matter.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:7 [ 0 => array:3 [ "identificador" => "bib0040" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Subacute sclerosing panencephalitis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "R. 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Year/Month | Html | Total | |
---|---|---|---|
2024 November | 13 | 1 | 14 |
2024 October | 50 | 9 | 59 |
2024 September | 62 | 15 | 77 |
2024 August | 43 | 18 | 61 |
2024 July | 42 | 7 | 49 |
2024 June | 31 | 12 | 43 |
2024 May | 24 | 5 | 29 |
2024 April | 26 | 7 | 33 |
2024 March | 35 | 7 | 42 |
2024 February | 47 | 14 | 61 |
2024 January | 87 | 9 | 96 |
2023 December | 103 | 15 | 118 |
2023 November | 105 | 19 | 124 |
2023 October | 107 | 9 | 116 |
2023 September | 38 | 2 | 40 |
2023 August | 74 | 8 | 82 |
2023 July | 107 | 8 | 115 |
2023 June | 168 | 5 | 173 |
2023 May | 160 | 8 | 168 |
2023 April | 193 | 6 | 199 |
2023 March | 115 | 4 | 119 |
2023 February | 84 | 4 | 88 |
2023 January | 85 | 7 | 92 |
2022 December | 86 | 9 | 95 |
2022 November | 89 | 13 | 102 |
2022 October | 64 | 12 | 76 |
2022 September | 63 | 28 | 91 |
2022 August | 93 | 8 | 101 |
2022 July | 62 | 8 | 70 |
2022 June | 61 | 10 | 71 |
2022 May | 62 | 11 | 73 |
2022 April | 55 | 16 | 71 |
2022 March | 69 | 12 | 81 |
2022 February | 78 | 12 | 90 |
2022 January | 71 | 12 | 83 |
2021 December | 87 | 34 | 121 |
2021 November | 63 | 10 | 73 |
2021 October | 67 | 19 | 86 |
2021 September | 73 | 12 | 85 |
2021 August | 76 | 7 | 83 |
2021 July | 37 | 9 | 46 |
2021 June | 47 | 15 | 62 |
2021 May | 60 | 10 | 70 |
2021 April | 117 | 20 | 137 |
2021 March | 80 | 27 | 107 |
2021 February | 33 | 25 | 58 |
2021 January | 34 | 13 | 47 |
2020 December | 49 | 13 | 62 |
2020 November | 46 | 8 | 54 |
2020 October | 43 | 6 | 49 |
2020 September | 20 | 15 | 35 |
2020 August | 35 | 6 | 41 |
2020 July | 24 | 6 | 30 |
2020 June | 35 | 9 | 44 |
2020 May | 25 | 13 | 38 |
2020 April | 26 | 5 | 31 |
2020 March | 22 | 8 | 30 |
2020 February | 41 | 17 | 58 |
2020 January | 18 | 10 | 28 |
2019 December | 25 | 15 | 40 |
2019 November | 16 | 14 | 30 |
2019 October | 18 | 3 | 21 |
2019 September | 21 | 7 | 28 |
2019 August | 25 | 3 | 28 |
2019 July | 16 | 14 | 30 |
2019 June | 47 | 12 | 59 |
2019 May | 163 | 15 | 178 |
2019 April | 64 | 5 | 69 |
2019 March | 2 | 4 | 6 |
2019 February | 7 | 3 | 10 |
2019 January | 7 | 3 | 10 |
2018 December | 9 | 6 | 15 |
2018 November | 8 | 7 | 15 |
2018 October | 14 | 11 | 25 |
2018 September | 27 | 3 | 30 |
2018 August | 5 | 1 | 6 |
2018 July | 13 | 1 | 14 |
2018 June | 9 | 0 | 9 |
2018 May | 19 | 1 | 20 |
2018 April | 9 | 2 | 11 |
2018 March | 1 | 2 | 3 |