Longitudinally extensive transverse myelitis with AQP4 antibodies revealing ovarian teratoma.

Autores de IIS La Fe

• Marina Frasquet Carrera

  Autor

• Luís Bataller Alberola

  Autor

• Estefania Torres Vega

  Autor

• Maria Duran Moreno

  Autor

• José Manuel García Verdugo

  Autor

• 

  Teresa Sevilla Mantecón

  Autor

• Salvador Rivas Rodero

  Autor

• Francisco Carlos Pérez Miralles

  Autor

• Maria Simo Castello

  Autor

• Bonaventura Casanova Estruch

  Autor

Grupos

• Patología Neuromuscular y Ataxias

Abstract

Paraneoplastic myelitis is a rare inflammatory disorder most frequently associated with solid tumors or lymphoproliferative disorders. Patients often harbor onconeuronal antibodies and their prognosis is usually poor. Here we report a 42-year old woman with longitudinally extensive transverse myelitis and aquaporin-4 (AQP4) antibodies that led to the diagnosis of ovarian teratoma. After tumor removal and immune therapy (including corticosteroids, plasma exchange, intravenous immunoglobulins and rituximab) the patient progressively improved achieving complete recovery. Histological study of the teratoma demonstrated neural tissue containing AQP4 expressing cells and intense inflammatory infiltrates, providing evidence for a possible paraneoplastic link between both disorders.

Keywords

• Autoimmune diseases, Paraneoplastic syndrome, Transverse myelitis