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It tends to be monophasic, usually occurs in children and young adults. Magnetic resonance imaging (MRI) shows multiple lesions in deep and subcortical white matter, with very heterogeneous characteristics in the different published case reports.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Brain infection caused by JC virus in patients with severe drug- or infection-induced immunosuppression produces progressive multifocal leukoencephalopathy (PML) caused by the infection of the CNS oligodendrocytes. Unlike ADEM, the course of the disease is sub-acute and progressive, with multifocal, cortical and subcortical lesions in brain MRI without mass effect and usually without contrast uptake. The diagnosis of PML is established by proving JC virus DNA appears in brain biopsy or cerebrospinal fluid (CSF) by PCR. Currently there is no specific antiviral treatment against JC virus, so the therapeutic objective is restoring the immune system in immunocompromised patients. Its prognosis is usually fatal, with long-term survival being rare.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case report of a 63-year-old man, with a history of hypertension, hypercholesterolemia and ex-smoker under treatment with enalapril and simvastatin, who came to the emergency room hours after the onset of instability and sudden-onset binocular horizontal double vision, with subsequent right hemiplegia. It associated vomiting and changes in body temperature without stiff neck. On examination, the patient was afebrile and presented dysarthria, ophthalmoparesis, left facial weakness and right hemiplegia.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The blood test showed normocytic anemia, a moderate elevation of cholestasis enzymes and a C-reactive protein of 80<span class="elsevierStyleHsp" style=""></span>mg/L. The first brain computed tomography (CT) was normal. Lumbar puncture (LP) showed a clear CSF with hypercellularity, predominantly polymorphonuclear leukocytes, normal glycorrhachia and hyperproteinorrhachia with normal ADA levels. Brain MRI in FLAIR sequence showed extensive lesion in left frontotemporal white matter with no changes in diffusion sequence (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A) along with hyperintense lesions in medulla, pons, mesencephalon, and cerebellar peduncles (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B), with no contrast enhancement. Empirical treatment was started with combined antibiotic therapy, acyclovir and dexamethasone. Initial staining, culture and analysis of CSF ruled out the presence of any bacterial infection or herpes viruses. JC virus PCR was positive, finding 640<span class="elsevierStyleHsp" style=""></span>copies/ml.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The lab test was extended, finding a hemolytic anemia with high lactate dehydrogenase levels, indirect hyperbilirubinemia and positive Coombs test, finding agglutination of red blood cells secondary to cryoglobulins in the peripheral blood smear. The patient also presented hypogammaglobulinaemia and lower CD4 lymphocyte levels, these being 219<span class="elsevierStyleHsp" style=""></span>cell/μl with negative HIV, HBV and HCV serology, as well as tumor markers and autoimmunity studies. A thoracoabdominopelvic CT scan showed left supraclavicular, retroperitoneal and mesenteric adenopathies with a 2–3<span class="elsevierStyleHsp" style=""></span>cm size, slightly hypermetabolic, whose biopsy was consistent with the presence of a low grade non-Hodgkin's lymphoma (NHL). The steroid treatment was progressively withdrawn, with the patient remaining asymptomatic, from a neurological point of view, 4 months after onset of signs and symptoms, with undetectable JC in control LP and normalization of brain MRI.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Recently, new forms of JC virus infection have been reported, such as granule cell neuronopathy, with subacute or chronic cerebellar dysfunction, JC meningitis that occurs with a typical meningeal syndrome, and JC virus encephalopathy with gray matter infection.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> Cases of ADEM following a JC virus infection are rare, as well as cases of PML in immunocompetent patients.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> It is estimated that the PML incidence in patients with NHL is 8.3%, usually as a complication of the immunosuppressive treatment used, although this presentation has not been reported at the onset of the disease or prior to treatment.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> Our case report shows an apparently immunocompetent patient who develops a sudden-onset multifocal encephalopathy with an acute and monophasic course following JC virus infection. We consider that the atypical presentation of JC virus infection should lead us to include the performance of an extended systemic study to rule out the immune system compromise or alteration as in this case report.</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: Castro-Sánchez MV, Villagrán-García M, Romero-Imbroda J. Encefalomielitis aguda diseminada por virus JC como comienzo de linfoma no hodgkiniano. Med Clin (Barc). 2019;152:e49–e50.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1506 "Ancho" => 2500 "Tamanyo" => 334162 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Brain MRI in FLAIR sequence showing extensive lesion in left frontotemporal white matter (A) along with hyperintense lesions in medulla, pons, mesencephalon and cerebellar peduncles (B).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:3 [ "comentario" => "https://doi.org/10.1212/WNL. 0000000000002723" "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Acute disseminated encephalomyelitis in 228 patients: a retrospective, multicenter US study" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "D.L. 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Letter to the Editor
Acute disseminated encephalomyelitis due to JC virus infection as the onset of non-Hodgkin's lymphoma
Encefalomielitis aguda diseminada por virus JC como comienzo de linfoma no hodgkiniano
María Victoria Castro-Sánchez
, Macarena Villagrán-García, Jesús Romero-Imbroda
Corresponding author
Servicio de Neurología, Hospital Regional Universitario de Málaga, Málaga, Spain