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dc.contributorVall d'Hebron Barcelona Hospital Campus
dc.contributor.authorSpatola, Marianna
dc.contributor.authorPetit-Pedrol, Mar
dc.contributor.authorSimabukuro, Mateus Mistieri
dc.contributor.authorArmangue, Thaís
dc.contributor.authorCastro, Fernanda J
dc.contributor.authorBarcelo Artigues, Maria I
dc.contributor.authorFelipe Rucián, Ana
dc.date.accessioned2019-03-22T07:06:20Z
dc.date.available2019-03-22T07:06:20Z
dc.date.issued2017-02-15
dc.identifier.citationSpatola M, Petit-Pedrol M, Simabukuro MM, Armangue T, Castro FJ, Barcelo Artigues MI, et al. Investigations in GABAA receptor antibody-associated encephalitis. Neurology. 2017;88(11):1012–20.
dc.identifier.issn0028-3878
dc.identifier.urihttp://hdl.handle.net/11351/3855
dc.descriptionGABA-A receptor; Encephalitis; Antibodies
dc.description.abstractOBJECTIVE: To report the clinical features, comorbidities, receptor subunit targets, and outcome in patients with anti-GABAA receptor (GABAAR) encephalitis. METHODS: Clinical study of 26 patients, including 17 new (April 2013-January 2016) and 9 previously reported patients. Antibodies to α1, β3, and γ2 subunits of the GABAAR were determined using reported techniques. RESULTS: Patients' median age was 40.5 years (interquartile range 48.5 [13.75-62.35] years; the youngest 2.5 months old; 13 female). Symptoms included seizures (88%), alteration of cognition (67%), behavior (46%), consciousness (42%), or abnormal movements (35%). Comorbidities were identified in 11 (42%) patients, including 7 tumors (mostly thymomas), 2 herpesvirus encephalitis (herpes simplex virus 1, human herpesvirus 6; coexisting with NMDAR antibodies), and 2 myasthenia without thymoma. Brain MRI was abnormal in 23 (88%) patients, showing in 20 (77%) multifocal, asynchronous, cortical-subcortical T2/fluid-attenuated inversion recovery abnormalities predominantly involving temporal (95%) and frontal (65%) lobes, but also basal ganglia and other regions. Immunologic or tumor therapy resulted in substantial improvement in 18/21 (86%) assessable patients; the other 3 (14%) died (2 status epilepticus, 1 sepsis). Compared with adults, children were more likely to have generalized seizures (p = 0.007) and movement disorders (p = 0.01) and less likely to have a tumor (p = 0.01). The main epitope targets were in the α1/β3 subunits of the GABAAR. CONCLUSIONS: Anti-GABAAR encephalitis is characterized by frequent seizures and distinctive multifocal cortical-subcortical MRI abnormalities that provide an important clue to the diagnosis. The frequency of symptoms and comorbidities differ between children (more viral-related) and adults (more tumor-related). The disorder is severe but most patients respond to treatment.
dc.language.isoeng
dc.publisherAmerican Academy of Neurology
dc.relation.ispartofseriesNeurology;88(11)
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 International
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/
dc.sourceScientia
dc.subjectEncefalitis
dc.subjectGABA - Receptors
dc.subjectAntígens
dc.subject.meshEncephalitis
dc.subject.mesh/immunology
dc.subject.meshReceptors, GABA-A
dc.subject.meshAntibodies
dc.titleInvestigations in GABAA receptor antibody-associated encephalitis
dc.typeinfo:eu-repo/semantics/article
dc.identifier.doi10.1212/WNL.0000000000003713
dc.subject.decsencefalitis
dc.subject.decs/inmunología
dc.subject.decsreceptores de GABA-A
dc.subject.decsanticuerpos
dc.relation.publishversionhttps://n.neurology.org/content/88/11/1012
dc.type.versioninfo:eu-repo/semantics/publishedVersion
dc.audienceProfessionals
dc.contributor.organismesInstitut Català de la Salut
dc.contributor.authoraffiliation[Spatola M] Institut d'Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Barcelona, Spain. University of Lausanne, Lausanne, Switzerland. [Petit-Pedrol M] Institut d'Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Barcelona, Spain. Centro de Investigación Biomédica en Red de Enfermedades Raras Madrid, Spain. [Simabukuro MM] Neurology Division, Hospital das Clínicas, São Paulo, Brazil. São Paulo University, São Paulo, Brazil. [Armangue T] Institut d'Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Barcelona, Spain. Centro de Investigación Biomédica en Red de Enfermedades Raras Madrid, Spain. Neurology Service, Hospital Sant Joan de Déu, Barcelona, Spain. [Castro FJ] Hospital de Base, Brasília, Brazil. [Barcelo Artigues MI] Service of Neurology, University Hospital of Son Espases, Mallorca, Spain. [Felipe A] Hospital Universitari Vall d'Hebron, Barcelona, Spain.
dc.identifier.pmid28202703
dc.identifier.wosWOS:000397383100005
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess


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