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"tienePdf" => "es" "tieneTextoCompleto" => "es" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "413" "paginaFinal" => "415" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Advanced interatrial block as a predictor of cardioembolic stroke: is it time to change our clinical practice?" ] ] "contieneTextoCompleto" => array:1 [ "es" => true ] "contienePdf" => array:1 [ "es" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figura 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1289 "Ancho" => 751 "Tamanyo" => 72259 ] ] "descripcion" => array:1 [ "es" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Activación auricular en sujetos sanos (A) y en pacientes con bloqueo interauricular (B).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "C.R. Lopez Perales, A. Perez Guerrero, D. Grados Saso, J.M. Salvador Casabona" "autores" => array:4 [ 0 => array:2 [ "nombre" => "C.R." "apellidos" => "Lopez Perales" ] 1 => array:2 [ "nombre" => "A." "apellidos" => "Perez Guerrero" ] 2 => array:2 [ "nombre" => "D." "apellidos" => "Grados Saso" ] 3 => array:2 [ "nombre" => "J.M." "apellidos" => "Salvador Casabona" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2173580822000311" "doi" => "10.1016/j.nrleng.2021.06.005" "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/S2173580822000311?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0213485321001109?idApp=UINPBA00004N" "url" => "/02134853/0000003700000005/v1_202205210936/S0213485321001109/v1_202205210936/es/main.assets" ] "en" => array:16 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Letter to the Editor</span>" "titulo" => "Rapidly progressive dementia with generalized myoclonus in an adult: Do not forget subacute sclerosing panencephalitis" "tieneTextoCompleto" => true "saludo" => "Dear Editor," "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "415" "paginaFinal" => "418" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "R. Ghosh, S. Dubey, A. Mukherjee, J. Benito-León" "autores" => array:4 [ 0 => array:3 [ "nombre" => "R." "apellidos" => "Ghosh" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 1 => array:3 [ "nombre" => "S." "apellidos" => "Dubey" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "A." "apellidos" => "Mukherjee" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:4 [ "nombre" => "J." "apellidos" => "Benito-León" "email" => array:1 [ 0 => "jbenitol67@gmail.com" ] "referencia" => array:4 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">d</span>" "identificador" => "aff0020" ] 2 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">e</span>" "identificador" => "aff0025" ] 3 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] ] "afiliaciones" => array:5 [ 0 => array:3 [ "entidad" => "Department of General Medicine, Burdwan Medical College & Hospital, Burdwan, West Bengal, India" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Department of Neuromedicine, Bangur Institute of Neurosciences, Kolkata, India" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Department of Neurology, University Hospital “12 de Octubre”, Madrid, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] 3 => array:3 [ "entidad" => "Department of Neurology, Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain" "etiqueta" => "d" "identificador" => "aff0020" ] 4 => array:3 [ "entidad" => "Department of Medicine, Complutense University, Madrid, Spain" "etiqueta" => "e" "identificador" => "aff0025" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Demencia rápidamente progresiva con mioclonías generalizadas en un adulto: no olvides la panencefalitis esclerosante subaguda" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 684 "Ancho" => 1505 "Tamanyo" => 246223 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Electroencephalogram revealing Radermecker complexes (high-voltage periodic repetitive polyphasic sharps and slow wave complexes of 0.5- to 2-second duration that recur every 4–15 seconds).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Persistence of measles infection in children and young adults can rarely lead to a fatal progressive neurodegenerative disorder known as subacute sclerosing panencephalitis (SSPE).<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">1–4</span></a> Amongst the other known neurological manifestations of measles infection, SSPE is the rarest and most severe.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">1–4</span></a> However, measles infection is amenable to prevention by highly effective live vaccination.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">1–5</span></a> The latency period between measles infection and development of clinical features of SSPE is variable and can be up to 2–3 decades.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">1–4</span></a> Earlier affection and intrafamilial cases of SSPE have a shorter latency.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">1–4</span></a> The clinical course is characterized by subacute to chronic progressive cognitive impairment, behavioral abnormalities, movement disorders (especially myoclonus), seizures, visual disturbances, and ataxia.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">1–3</span></a> Although few reports of spontaneous remissions of SSPE have been documented,<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">6,7</span></a> this disease is considered universally fatal. Diagnosis of SSPE is difficult in developed countries because of extreme rarity of the condition and atypical and non-specific initial presentation because the sufferers come rather early in the course of disease.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">1–3,8</span></a> On the contrary, in underdeveloped/developing countries, the delay in diagnosis occurs due to low health-related literacy in general population, significant lack of knowledge and training of primary care physicians regarding these disorders, inadequate health-seeking behavior of the general population, and inadequate investigational backup in peripheral health care setups .<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">1–3,8</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We herein report a case of SSPE in a 36-year-old previously healthy man with questionable vaccination status in childhood who presented with rapidly progressive early onset dementia and subcortical myoclonus. Neuroimaging revealed altered signals involving bilateral thalamus and brainstem widening the list of differential diagnoses; however, clinical history, examination findings, and high anti-measles IgG titers in serum and cerebrospinal fluid (CSF) clinched the diagnosis, which was supported by classical Radermecker's complex in electroencephalography (EEG).<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">9</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">A 36-year-old previously healthy man from a middle socio-economic family of rural West Bengal (India) was brought to the out-patient department with complaints of rapidly progressive behavioral abnormalities (inattentiveness, forgetfulness, mood disturbances, aggressive behavior, lack of interest in surroundings, calculation difficulties, difficulties in recognizing relatives and common objects, and loosing ways during navigation) and visual difficulties for last 3–4 months. The family members also gave history of objects falling from his hands unknowingly, and gradually evolving abnormal involuntary jerky “shock-like” movements involving all the limbs and torso for the last 2 months. Past medical history and history of long-term drug exposure or addiction in any form were unyielding. The family members revealed that one of his brothers also had succumbed to a similar illness within 2 years of developing similar symptoms at the age of 18 years. On enquiry, his mother described an episode of “fever with confluent rash” with spontaneous recovery in all her children, in their childhood, over a period of 2 weeks suggestive of measles. Neurological examination revealed multi-domain cognitive impairments with subcortical myoclonus.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Brain magnetic resonance imaging (MRI) showed non-enhancing bilaterally symmetrical altered intensity lesions, hyperintense on T2-weighted imaging and FLAIR sequences, involving thalamus and brainstem (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). EEG revealed periodic discharges suggestive of Radermecker complexes (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Contrast enhanced MR angiography and MR venography excluded common vascular insults. Accordingly, relevant tests were done, which excluded metabolic disorders (blood glucose, thyroid, liver and renal function tests, electrolytes, vitamin B1 and B12, arterial blood gas analysis, serum lactate, CSF lactate, lactate/pyruvate ratio, and anti-thyroperoxidase/thyroglobulin antibody were normal), toxic encephalopathy (toxicology screen), acute infectious encephalopathies (neuro-viruses panel, including SARS-CoV-2 and HIV 1, 2, were negative). Autoimmune/paraneoplastic encephalitis panel was negative. CSF study revealed lymphocyte-predominant pleocytosis (cell count: 25/μl), increased total protein (108<span class="elsevierStyleHsp" style=""></span>mg/dL), and raised IgG index. CSF and serum were tested for anti-measles IgG antibody titers and they came out to be extremely high confirming the diagnosis of SSPE further (fulfillment of Dyken's criteria).<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">8</span></a></p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Quite expectedly, resource-constrained countries with a high burden of measles encounter a higher incidence of SSPE.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">1–3</span></a> Besides, significant number of cases remain undiagnosed/misdiagnosed and unreported.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">1–3,8</span></a> However, measles vaccine has a preventive effect against SSPE by simply protecting against measles.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">5</span></a> Countries with high vaccination gap and recent outbreaks is associated with higher incidence of SSPE.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">1–5</span></a> Provided the potential lethality of long-term infection of measles, SSPE should be diagnosed early and reported.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Of note is that MRI patterns of SSPE may mimic metabolic disorders.<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">10</span></a> Symmetrical thalamic and brainstem involvement without affection of periventricular sub-cortical white matter and cortex is extremely rare in SSPE.<a class="elsevierStyleCrossRefs" href="#bib0110"><span class="elsevierStyleSup">10–12</span></a> However, neuroimaging findings of SSPE have typically a dynamic and evolving course. And in this case, though the cortical involvement was not evident on conventional imaging, clinical picture suggested involvement of fronto-parietal and occipital cortices (inattentiveness, forgetfulness, mood disturbances, aggressive behavior, apathy, dyscalculia, prosopagnosia, and visual object agnosia). Cortical and sub-cortical white matter intensity changes are expected to be visualized in the follow-up scans. Symmetrical thalamic involvement made it difficult for us to diagnose the case as similar neuroimaging findings can be observed in many other neurological disorders.<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">13</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">In closing, SSPE is almost universally fatal and even symptomatic treatment does not help much leading to a disastrous end of a young productive life. Measles vaccination is the only definite way to prevent SSPE until date hence all children and eligible adolescents should get it without hesitancy. SSPE should be kept in the list of differential diagnoses, while dealing a case of subacute-to-acute onset rapidly evolving cognitive decline with myoclonic jerks.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Ethics statement</span><p id="par0040" class="elsevierStylePara elsevierViewall">Informed written consent was obtained from the patient involved in this study.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Disclosures</span><p id="par0045" class="elsevierStylePara elsevierViewall">R. Ghosh reports no disclosures relevant to the manuscript.</p><p id="par0050" class="elsevierStylePara elsevierViewall">S. Dubey reports no disclosures relevant to the manuscript.</p><p id="par0055" class="elsevierStylePara elsevierViewall">A .K. Mukherjee reports no disclosures relevant to the manuscript.</p><p id="par0060" class="elsevierStylePara elsevierViewall">J. Benito-León reports no disclosures relevant to the manuscript.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Funding</span><p id="par0065" class="elsevierStylePara elsevierViewall">None.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Conflict of interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors have no conflict of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:6 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Ethics statement" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Disclosures" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Funding" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Conflict of interest" ] 4 => array:2 [ "identificador" => "xack605015" "titulo" => "Acknowledgments" ] 5 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1526 "Ancho" => 2925 "Tamanyo" => 793737 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Axial T2-weighted (A) and FLAIR-weighted (B) images revealing bilateral symmetrical hyperintense lesions involving thalami and brainstem (some motion-related artifacts can be noted, as the patient was moving involuntarily during imaging procedure.</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" => 684 "Ancho" => 1505 "Tamanyo" => 246223 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Electroencephalogram revealing Radermecker complexes (high-voltage periodic repetitive polyphasic sharps and slow wave complexes of 0.5- to 2-second duration that recur every 4–15 seconds).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:13 [ 0 => array:3 [ "identificador" => "bib0065" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Subacute sclerosing panencephalitis: an update" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "J. 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Benito-León is supported by the <span class="elsevierStyleGrantSponsor" id="gs1">National Institutes of Health</span>, Bethesda, MD, USA (NINDS <span class="elsevierStyleGrantNumber" refid="gs1">#R01 NS39422</span>), <span class="elsevierStyleGrantSponsor" id="gs2">European Commission</span> (grant <span class="elsevierStyleGrantNumber" refid="gs2">ICT-2011-287739</span>, NeuroTREMOR), the <span class="elsevierStyleGrantSponsor" id="gs3">Ministry of Economy and Competitiveness</span> (grant <span class="elsevierStyleGrantNumber" refid="gs3">RTC-2015-3967-1</span>, NetMD—platform for the tracking of movement disorder), and the <span class="elsevierStyleGrantSponsor" id="gs4">Spanish Health Research Agency</span> (grant <span class="elsevierStyleGrantNumber" refid="gs4">FIS PI12/01602</span> and grant <span class="elsevierStyleGrantNumber" refid="gs4">FIS PI16/00451</span>).</p>" "vista" => "all" ] ] ] "idiomaDefecto" => "en" "url" => "/02134853/0000003700000005/v1_202205210936/S0213485321002620/v1_202205210936/en/main.assets" "Apartado" => array:4 [ "identificador" => "9412" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Cartas al Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/02134853/0000003700000005/v1_202205210936/S0213485321002620/v1_202205210936/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0213485321002620?idApp=UINPBA00004N" ]
Year/Month | Html | Total | |
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2024 November | 15 | 0 | 15 |
2024 October | 66 | 9 | 75 |
2024 September | 71 | 16 | 87 |
2024 August | 74 | 12 | 86 |
2024 July | 96 | 6 | 102 |
2024 June | 67 | 8 | 75 |
2024 May | 64 | 4 | 68 |
2024 April | 48 | 2 | 50 |
2024 March | 69 | 14 | 83 |
2024 February | 75 | 7 | 82 |
2024 January | 97 | 6 | 103 |
2023 December | 82 | 13 | 95 |
2023 November | 65 | 7 | 72 |
2023 October | 156 | 10 | 166 |
2023 September | 63 | 5 | 68 |
2023 August | 67 | 5 | 72 |
2023 July | 78 | 6 | 84 |
2023 June | 65 | 3 | 68 |
2023 May | 91 | 12 | 103 |
2023 April | 33 | 3 | 36 |
2023 March | 22 | 3 | 25 |
2023 February | 21 | 3 | 24 |
2023 January | 11 | 12 | 23 |
2022 December | 21 | 6 | 27 |
2022 November | 31 | 10 | 41 |
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