Letter to the Editor
Morvan syndrome: case report
Síndrome de Morvan: Presentación de un caso
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López-Núñez" "autores" => array:3 [ 0 => array:4 [ "nombre" => "Anna" "apellidos" => "Esquerrà" "email" => array:1 [ 0 => "anna.esquerra.molas@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Marta" "apellidos" => "Álvarez-Larruy" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "Juan J." "apellidos" => "López-Núñez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio Medicina Interna. Hospital Universitario Germans Trias i Pujol, Badalona, Barcelona, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio Neurología. Hospital Universitario Germans Trias i Pujol, Badalona, Barcelona, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome de Morvan: Presentación de un caso" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 59-year-old patient with a history of hypertension was admitted to Internal Medicine for the study of a one-month long toxic syndrome, accompanied, in the last 15 days, by symptoms of disorientation, complex visual hallucinations, secondary scratching lesions, nocturnal agitation, daytime somnolence, dysarthria, gait instability, “tremors” in the legs and neuropathic pain in the hands and feet. Physical examination revealed myokymia in the chin and in both calves, unsteady gait with increased base of support, stocking hypesthesia, pallhypesthesia in the malleoli, patellar and achilles hyporeflexia, facial telangiectasias and parotid hypertrophy.</p><p id="par0010" class="elsevierStylePara elsevierViewall">A complete laboratory analysis was performed, as well as neuroimaging tests that showed no significant findings; electromyogram compatible with muscular hyperactivity syndrome and electroencephalogram showing an organised waking background tracing, with no interictal epileptiform discharges.</p><p id="par0015" class="elsevierStylePara elsevierViewall">On suspicion of autoimmune encephalitis, a cerebrospinal fluid (CSF) study was performed, which confirmed the absence of oligoclonal bands, but was positive for <span class="elsevierStyleItalic">anti</span>-<span class="elsevierStyleItalic">contactin-associated protein-like 2</span> (CASPR2) and <span class="elsevierStyleItalic">anti</span>-<span class="elsevierStyleItalic">leucine rich glioma inactivated protein 1</span> (LGI1) antibodies (Ab), leading to a diagnosis of probable Morvan syndrome (MS). Treatment was started with intravenous (IV) immunoglobulins at a dose of 2 g/kg for three days with little clinical improvement, so corticotherapy was started with IV methylprednisolone bolus (1g/day) for five days and then maintenance with prednisone at a dose of 1 mg/kg/day orally.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Given the presence of toxic syndrome and the association between autoimmune encephalitis and underlying neoplasm, a screening for malignancy was performed, which was negative. The patient progresses unfavourably, so it is agreed to start treatment with i.v. rituximab (2g distributed over two weeks, then 1g every six months) without complications. After the first cycle, the symptoms resolved, although they returned later.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The MS is a rare channelopathy with a high mortality that gives rise to heterogeneous subacute clinical features such as the presence of myokymia, insomnia, dysautonomia and central nervous system involvement<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a>. It mainly affects middle-aged men and its prevalence is unknown, with less than 100 cases in the literature, none in the Spanish literature so far.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Although its etiopathogenesis is not well defined, the presence of Ab against voltage-gated potassium channels and the response to immunosuppressive treatment support the autoimmune aetiology of the syndrome<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a>. Specifically, two neuronal proteins have been identified as targets: CASPR2 and LGI1. In a review of 29 patients with MS, positive anti-VGKC was identified in 24 patients (79%), anti-CASPR2 in six patients, anti-LGI1 in three patients, anti-CASPR2 and anti-LGI1 in 12 patients, and anti-CASPR2, anti-LGI1 and anti-contactin 2 in three patients<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a>.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Clinically it manifests as the association of multifocal or diffuse encephalitis, hyperexcitability of the peripheral nerve in the form of neuromyotonia and dysautonomia<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3</span></a>. Sleep disorders and neuropathic pain are common, with areflexia and stocking and glove hypesthesia. The presence of epileptic seizures of diverse semiology is not uncommon<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a>.</p><p id="par0040" class="elsevierStylePara elsevierViewall">While the diagnosis is clinical, 25% of patients have CSF abnormalities and neuroimaging is usually normal<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2–4</span></a>.</p><p id="par0045" class="elsevierStylePara elsevierViewall">With regard to tumour association, the data available in the literature are mixed: some groups identify an underlying tumour in around 20-41% of cases, predominantly thymomas, while other groups suggest that they are less common<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3</span></a>.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Although there is little literature on treatment, experts recommend immunosuppressive treatment<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,5</span></a>, with steroids, IV immunoglobulins and plasma exchange being the first-line therapies. In the absence of a clinical response, therapies such as rituximab and/or cyclophosphamide are then considered. Overall, the prognosis is good, but recurrence is not uncommon<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a>.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Out of a published series of 12 cases<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a>, most patients responded to immunosuppressive treatment (plasmapheresis or immunoglobulins, steroid-associated or not), although most required chronic treatment to remain stable. None of them improved spontaneously before starting treatment<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,5</span></a>.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Early detection of this syndrome is paramount, in order to initiate treatment and tumour search within an appropriate therapeutic window. Regarding our patient, despite the multiple complementary examinations carried out, to date, no concomitant malignancy has been identified.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflict of interests</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors declare no conflict of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflict of interests" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Esquerrà A, Álvarez-Larruy M, López-Núñez JJ. Síndrome de Morvan: Presentación de un caso. Med Clin (Barc). 2021;157:e311–e312.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Morvan syndrome: clinical and serological observations in 29 cases" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "S.R. Irani" 1 => "P. Pettingill" 2 => "K.A. Kleopa" 3 => "N. Schiza" 4 => "P. Waters" 5 => "C. 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Martinez-Hernandez" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1002/ana.22297" "Revista" => array:6 [ "tituloSerie" => "Ann Neurol" "fecha" => "2011" "volumen" => "69" "paginaInicial" => "303" "paginaFinal" => "311" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21387375" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Management of voltage-gated potassium channel antibody disorders" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "M.P. Merchut" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.ncl.2010.03.024" "Revista" => array:6 [ "tituloSerie" => "Neurol Clin" "fecha" => "2010" "volumen" => "28" "paginaInicial" => "941" "paginaFinal" => "959" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20816272" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000015700000009/v1_202111060623/S2387020621005404/v1_202111060623/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000015700000009/v1_202111060623/S2387020621005404/v1_202111060623/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020621005404?idApp=UINPBA00004N" ]
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