dc.contributor | Vall d'Hebron Barcelona Hospital Campus |
dc.contributor.author | Spatola, Marianna |
dc.contributor.author | Petit-Pedrol, Mar |
dc.contributor.author | Simabukuro, Mateus Mistieri |
dc.contributor.author | Armangue, Thaís |
dc.contributor.author | Castro, Fernanda J |
dc.contributor.author | Barcelo Artigues, Maria I |
dc.contributor.author | Felipe Rucián, Ana |
dc.date.accessioned | 2019-03-22T07:06:20Z |
dc.date.available | 2019-03-22T07:06:20Z |
dc.date.issued | 2017-02-15 |
dc.identifier.citation | Spatola 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.issn | 0028-3878 |
dc.identifier.uri | https://hdl.handle.net/11351/3855 |
dc.description | GABA-A receptor; Encephalitis; Antibodies |
dc.description.abstract | OBJECTIVE:
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.iso | eng |
dc.publisher | American Academy of Neurology |
dc.relation.ispartofseries | Neurology;88(11) |
dc.rights | Attribution-NonCommercial-NoDerivatives 4.0 International |
dc.rights.uri | http://creativecommons.org/licenses/by-nc-nd/4.0/ |
dc.source | Scientia |
dc.subject | Encefalitis |
dc.subject | GABA - Receptors |
dc.subject | Antígens |
dc.subject.mesh | Encephalitis |
dc.subject.mesh | /immunology |
dc.subject.mesh | Receptors, GABA-A |
dc.subject.mesh | Antibodies |
dc.title | Investigations in GABAA receptor antibody-associated encephalitis |
dc.type | info:eu-repo/semantics/article |
dc.identifier.doi | 10.1212/WNL.0000000000003713 |
dc.subject.decs | encefalitis |
dc.subject.decs | /inmunología |
dc.subject.decs | receptores de GABA-A |
dc.subject.decs | anticuerpos |
dc.relation.publishversion | https://n.neurology.org/content/88/11/1012 |
dc.type.version | info:eu-repo/semantics/publishedVersion |
dc.audience | Professionals |
dc.contributor.organismes | Institut 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.pmid | 28202703 |
dc.identifier.wos | WOS:000397383100005 |
dc.rights.accessrights | info:eu-repo/semantics/openAccess |