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Paraneoplastic neuromyelitis optica and ovarian teratoma: A case series

  • Author Footnotes
    1 Contributed equally to this work.
    Raphaël Bernard-Valnet
    Correspondence
    Corresponding author at: Medicine Department, Centre hospitalier universitaire vaudois (CHUV) CUTR Sylvana, Service de gériatrie et réadaptation gériatrique, Ch. de Sylvana 10, 1066 Epalinges, Switzerland.
    Footnotes
    1 Contributed equally to this work.
    Affiliations
    Center for pathophysiology Toulouse Purpan, INSERM U1043, CNRS 5282, Université Toulouse III, 31024 Toulouse, France

    CRC-SEP, Pôle Neurosciences, CHU de Toulouse, 31059 Toulouse, France

    Neurosciences and Medicine Departments, Centre hospitalier universitaire vaudois (CHUV), 1011 Lausanne, Switzerland
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  • Author Footnotes
    1 Contributed equally to this work.
    Alvaro Cobo-Calvo
    Footnotes
    1 Contributed equally to this work.
    Affiliations
    Service de neurologie, sclérose en plaques, pathologies de la myéline et neuro-inflammation and Centre de référence pour les maladies inflammatoires rares du cerveau et de la moelle (MIRCEM)– Hôpital Neurologique Pierre Wertheimer Hospices Civils de Lyon, Lyon, F-6977, France

    Lyon's Neuroscience Research Center, U1028 INSERM, UMR5292 CNRS, FLUID Team F-69008 Lyon, France
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  • Aurore Siegfried
    Affiliations
    Service d'anatomo-pathologie, CHU de Toulouse, IUCT Oncopôle, Toulouse, France
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  • Raluca Marasescu
    Affiliations
    Service de neurologie, CH Pau, 64000, Pau, France
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  • Mickael Bonnan
    Affiliations
    Service de neurologie, CH Pau, 64000, Pau, France
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  • Guillaume Ballan
    Affiliations
    Service de neurologie, CH de la Côte Basque, 64109, Bayonne, France
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  • Emmanuel Ellie
    Affiliations
    Service de neurologie, CH de la Côte Basque, 64109, Bayonne, France
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  • Jan Bauer
    Affiliations
    Center for Brain Research, Medical University of Vienna, Vienna, Austria
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  • Author Footnotes
    1 Contributed equally to this work.
    Emmanuelle Uro-Coste
    Footnotes
    1 Contributed equally to this work.
    Affiliations
    Service d'anatomo-pathologie, CHU de Toulouse, IUCT Oncopôle, Toulouse, France
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  • Author Footnotes
    1 Contributed equally to this work.
    Romain Marignier
    Footnotes
    1 Contributed equally to this work.
    Affiliations
    Service de neurologie, sclérose en plaques, pathologies de la myéline et neuro-inflammation and Centre de référence pour les maladies inflammatoires rares du cerveau et de la moelle (MIRCEM)– Hôpital Neurologique Pierre Wertheimer Hospices Civils de Lyon, Lyon, F-6977, France

    Lyon's Neuroscience Research Center, U1028 INSERM, UMR5292 CNRS, FLUID Team F-69008 Lyon, France
    Search for articles by this author
  • Author Footnotes
    1 Contributed equally to this work.
    David Brassat
    Footnotes
    1 Contributed equally to this work.
    Affiliations
    Center for pathophysiology Toulouse Purpan, INSERM U1043, CNRS 5282, Université Toulouse III, 31024 Toulouse, France

    CRC-SEP, Pôle Neurosciences, CHU de Toulouse, 31059 Toulouse, France
    Search for articles by this author
  • Author Footnotes
    1 Contributed equally to this work.

      Highlights

      • Paraneoplastic forms of neuromyelitis optica (NMOSD) may occur in rare cases.
      • We describe 3 cases of NMOSD in the context of underlying ovarian teratoma.
      • All three patients presented initially with area postrema symptomatology.
      • Strong immune infiltrates colocalize with AQP4+ glial component in the teratomas.

      Abstract

      Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease of the central nervous system, characterized by the presence of auto-antibodies directed against aquaporin-4 (AQP4) expressed on astrocyte end-feet. Despite NMOSD does not primarily belong to the spectrum of paraneoplastic neurological syndromes, rare cases of association with neoplasia have been outlined. Here, we report the association of NMOSD with ovarian teratoma in 3 cases. Pathological analysis of teratomas revealed glial component strongly expressing AQP4 and closely localized to immune infiltrates. Our series highlight the rare association of teratoma with NMOSD and the possible paraneoplastic mechanism.

      Keywords

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