title: A novel treatment-responsive encephalitis with frequent opsoclonus and teratoma.
abstract: 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.
authors.affiliation: Neurology Service, Hospital Clinic, August Pi i Sunyer Biomedical Research Institute University of Barcelona, Barcelona, Spain; Pediatric Neurology Service, Vall d'Hebron Maternal-Infant Hospital, Autonomous University of Barcelona, Barcelona, Spain.
authors.forename: Thaís
authors.initials: T
authors.lastname: Armangue
authors.forename: Maarten J
authors.initials: MJ
authors.lastname: Titulaer
authors.forename: Lidia
authors.initials: L
authors.lastname: Sabater
authors.forename: Javier
authors.initials: J
authors.lastname: Pardo-Moreno
authors.forename: Nuria
authors.initials: N
authors.lastname: Gresa-Arribas
authors.forename: Natalia
authors.initials: N
authors.lastname: Barbero-Bordallo
authors.forename: Gordon R
authors.initials: GR
authors.lastname: Kelley
authors.forename: Noh
authors.initials: N
authors.lastname: Kyung-Ha
authors.forename: Akitoshi
authors.initials: A
authors.lastname: Takeda
authors.forename: Takahiro
authors.initials: T
authors.lastname: Nagao
authors.forename: Yukitoshi
authors.initials: Y
authors.lastname: Takahashi
authors.forename: Angélica
authors.initials: A
authors.lastname: Lizcano
authors.forename: Aisling S
authors.initials: AS
authors.lastname: Carr
authors.forename: Francesc
authors.initials: F
authors.lastname: Graus
authors.forename: Josep
authors.initials: J
authors.lastname: Dalmau
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000276:immunology; D020295:Brain Stem Neoplasms / Q000150:complications / Q000276:immunology* / Q000601:surgery; D002528:Cerebellar Neoplasms / Q000150:complications / Q000276:immunology / Q000601:surgery; D002648:Child; D004660:Encephalitis / Q000150:complications* / Q000276:immunology / Q000628:therapy*; D005260:Female; D006801:Humans; D008297:Male; D015835:Ocular Motility Disorders / Q000150:complications* / Q000276:immunology; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology; D063189:Symptom Assessment; D013577:Syndrome; D013724:Teratoma / Q000150:complications* / Q000276:immunology / Q000601:surgery
chemical_list: D001323:Autoantibodies; D016194:Receptors, N-Methyl-D-Aspartate