Netrin-1 receptor antibodies in thymoma-associated neuromyotonia with myasthenia gravis

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Autores de IIS La Fe

Participantes ajenos a IIS La Fe

  • Cebrian-Silla, A
  • Herranz-Perez, V
  • Moris, G
  • Joubert, B
  • Honnorat, J
  • Dalmau, J
  • Graus, F
  • Garcia-Verdugo, JM

Grupos

Abstract

Objective: To identify cell-surface antibodies in patients with neuromyotonia and to describe the main clinical implications. Methods: Sera of 3 patients with thymoma-associated neuromyotonia and myasthenia gravis were used to immunoprecipitate and characterize neuronal cell-surface antigens using reported techniques. The clinical significance of antibodies against precipitated proteins was assessed with sera of 98 patients (neuromyotonia 46, myasthenia gravis 52, thymoma 42; 33 of them with overlapping syndromes) and 219 controls (other neurologic diseases, cancer, and healthy volunteers). Results: Immunoprecipitation studies identified 3 targets, including the Netrin-1 receptors DCC (deleted in colorectal carcinoma) and UNC5A (uncoordinated-5A) as well as Caspr2 (contactin-associated protein-like 2). Cell-based assays with these antigens showed that among the indicated patients, 9 had antibodies against Netrin-1 receptors (7 with additional Caspr2 antibodies) and 5 had isolated Caspr2 antibodies. Only one of the 219 controls had isolated Caspr2 antibodies with relapsing myelitis episodes. Among patients with neuromyotonia and/or myasthenia gravis, the presence of Netrin-1 receptor or Caspr2 antibodies predicted thymoma (p < 0.05). Coexisting Caspr2 and Netrin-1 receptor antibodies were associated with concurrent thymoma, myasthenia gravis, and neuromyotonia, often with Morvan syndrome (p = 0.009). Expression of DCC, UNC5A, and Caspr2 proteins was demonstrated in paraffin-embedded thymoma samples (3) and normal thymus. Conclusions: Antibodies against Netrin-1 receptors (DCC and UNC5a) and Caspr2 often coexist and associate with thymoma in patients with neuromyotonia and myasthenia gravis.

Datos de la publicación

ISSN/ISSNe:
0028-3878, 1526-632X

Neurology  LIPPINCOTT WILLIAMS & WILKINS

Tipo:
Article
Páginas:
1235-1242
PubMed:
28251919
Factor de Impacto:
3,399 SCImago
Cuartil:
Q1 SCImago

Citas Recibidas en Web of Science: 26

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