A novel treatment-responsive encephalitis with frequent opsoclonus and teratoma

Among 249 patients with teratoma‐associated encephalitis, 211 had N‐methyl‐D‐aspartate receptor antibodies and 38 were negative for these antibodies. Whereas antibody‐positive patients rarely developed prominent brainstem-cerebellar symptoms, 22 (58%) antibody‐negative patients developed a brainstem...

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Detalles Bibliográficos
Autores: Armangué, Thaís, Titulaer, Maarten J., Sabater Baudet, Lidia, Pardo Moreno, Javier, Gresa Arribas, Nuria, Barbero Bordallo, Natalia, Kelley, Gordon R., Kyung-Ha, Noh, Takeda, Akitoshi, Nagao, Takahiro, Takahashi, Yukitoshi, Lizcano, Angélica, Carr, Aisling S., Graus Ribas, Francesc, Dalmau Obrador, Josep
Tipo de recurso: artículo
Estado:Versión aceptada para publicación
Fecha de publicación:2013
País:España
Institución:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
Repositorio:Recercat. Dipósit de la Recerca de Catalunya
OAI Identifier:oai:recercat.cat:2445/130725
Acceso en línea:https://hdl.handle.net/2445/130725
Access Level:acceso abierto
Palabra clave:Teratoma
Encefalitis
Tumors cerebrals
Encephalitis
Brain tumors
Descripción
Sumario:Among 249 patients with teratoma‐associated encephalitis, 211 had N‐methyl‐D‐aspartate receptor antibodies and 38 were negative for these antibodies. Whereas antibody‐positive patients rarely developed prominent brainstem-cerebellar symptoms, 22 (58%) antibody‐negative patients developed a brainstem-cerebellar syndrome, which in 45% occurred with opsoclonus. The median age of these patients was 28.5 years (range = 12-41), 91% were women, and 74% had full recovery after therapy and tumor resection. These findings uncover a novel phenotype of paraneoplastic opsoclonus that until recently was likely considered idiopathic or postinfectious. The triad of young age (teenager to young adult), systemic teratoma, and high response to treatment characterize this novel brainstem-cerebellar syndrome.