A novel treatment-responsive encephalitis with frequent opsoclonus and teratoma

Authors

  • Thaís Armangue MD,

    1. Neurology Service, Hospital Clinic, August Pi i Sunyer Biomedical Research Institute University of Barcelona, Barcelona, Spain
    2. Pediatric Neurology Service, Vall d'Hebron Maternal–Infant Hospital, Autonomous University of Barcelona, Barcelona, Spain
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  • Maarten J. Titulaer MD, PhD,

    1. Neurology Service, Hospital Clinic, August Pi i Sunyer Biomedical Research Institute University of Barcelona, Barcelona, Spain
    2. Department of Neurology, University of Pennsylvania, Philadelphia, PA
    Current affiliation:
    1. Department of Neurology, Erasmus Medical Center, Rotterdam, the Netherlands
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  • Lidia Sabater PhD,

    1. Neurology Service, Hospital Clinic, August Pi i Sunyer Biomedical Research Institute University of Barcelona, Barcelona, Spain
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  • Javier Pardo-Moreno MD,

    1. Neurology Service, Rey Juan Carlos Hospital–Infanta Elena Hospital, Madrid, Spain
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  • Nuria Gresa-Arribas PhD,

    1. Neurology Service, Hospital Clinic, August Pi i Sunyer Biomedical Research Institute University of Barcelona, Barcelona, Spain
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  • Natalia Barbero-Bordallo MD,

    1. Neurology Service, Rey Juan Carlos Hospital–Infanta Elena Hospital, Madrid, Spain
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  • Gordon R. Kelley MD,

    1. Service of Neurology Service, Shawnee Mission Medical Center, Shawnee Mission, KS
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  • Noh Kyung-Ha MD,

    1. Department of Neurology, Pusan National University Yangsan Hospital, Yangsan, South Korea
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  • Akitoshi Takeda MD, PhD,

    1. Department of Geriatric Medicine and Neurology, Osaka City University Graduate School of Medicine, Osaka, Japan
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  • Takahiro Nagao MD,

    1. Internal Medicine Service, Toukatsu Hospital, Chiba, Japan
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  • Yukitoshi Takahashi MD,

    1. Department of Neurology, National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan
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  • Angélica Lizcano MD,

    1. Department of Neurology, University of Campinas, Campinas, Brazil
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  • Aisling S. Carr MRCP, PhD,

    1. Department of Neurology, Royal Victoria Hospital, Belfast, North Ireland
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  • Francesc Graus MD,

    1. Neurology Service, Hospital Clinic, August Pi i Sunyer Biomedical Research Institute University of Barcelona, Barcelona, Spain
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  • Josep Dalmau MD, PhD

    Corresponding author
    1. Neurology Service, Hospital Clinic, August Pi i Sunyer Biomedical Research Institute University of Barcelona, Barcelona, Spain
    2. Department of Neurology, University of Pennsylvania, Philadelphia, PA
    3. Catalan Institution for Research and Advanced Studies (ICREA), University of Barcelona, Barcelona, Spain
    • Address correspondence to Dr Dalmau, Neurology Service, IDIBAPS-Hospital Clínic, University of Barcelona, Casanova 143, CELLEX, P3A, Barcelona 08036, Spain. E-mail: Josep.dalmau@uphs.upenn.edu

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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

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