A novel treatment-responsive encephalitis with frequent opsoclonus and teratoma

Thaís Armangue, Maarten J. Titulaer, Lidia Sabater, Javier Pardo-Moreno, Nuria Gresa-Arribas, Natalia Barbero-Bordallo, Gordon R. Kelley, Noh Kyung-Ha, Akitoshi Takeda, Takahiro Nagao, Yukitoshi Takahashi, Angélica Lizcano, Aisling S. Carr, Francesc Graus, Josep Dalmau*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

48 Citations (Scopus)

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. ANN NEUROL 2014;75:435-441

Original languageEnglish
Pages (from-to)435-441
Number of pages7
JournalAnnals of Neurology
Volume75
Issue number3
DOIs
Publication statusPublished - Mar 2014
Externally publishedYes

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