TY - JOUR
T1 - A novel treatment-responsive encephalitis with frequent opsoclonus and teratoma
AU - Armangue, Thaís
AU - Titulaer, Maarten J.
AU - Sabater, Lidia
AU - Pardo-Moreno, Javier
AU - Gresa-Arribas, Nuria
AU - Barbero-Bordallo, Natalia
AU - Kelley, Gordon R.
AU - Kyung-Ha, Noh
AU - Takeda, Akitoshi
AU - Nagao, Takahiro
AU - Takahashi, Yukitoshi
AU - Lizcano, Angélica
AU - Carr, Aisling S.
AU - Graus, Francesc
AU - Dalmau, Josep
PY - 2014/3
Y1 - 2014/3
N2 - 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
AB - 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
UR - http://www.scopus.com/inward/record.url?scp=84897999009&partnerID=8YFLogxK
U2 - 10.1002/ana.23917
DO - 10.1002/ana.23917
M3 - Article
C2 - 23613036
AN - SCOPUS:84897999009
SN - 0364-5134
VL - 75
SP - 435
EP - 441
JO - Annals of Neurology
JF - Annals of Neurology
IS - 3
ER -