Paraneoplastic neuromyelitis optica spectrum disorder: A case report and review of the literature

J Clin Neurosci. 2018 Feb:48:7-10. doi: 10.1016/j.jocn.2017.10.030. Epub 2017 Nov 10.

Abstract

Neuromyelitis optica spectrum disorders (NMOSD) are demyelinating, autoimmune diseases affecting the central nervous system. Typically, recurrent optic neuritis and longitudinal extensive transverse myelitis dominates the clinical picture. In most cases NMOSD are associated with autoantibodies targeting the water channel aquaporin-4 (AQP-4). NMOSD usually present in young adults. Clinical findings suggestive of NMOSD in elderly patients should raise the suspicion of a paraneoplastic etiology. To our knowledge, we report the first case of a 66 year-old female patient with paraneoplastic NMOSD that is associated with squamous cell lung carcinoma. Anti-AQP-4 was present in both the serum and cerebrospinal fluid of the patient. However, immunhistological staining of the malignant tissue did not show presence of AQP-4 on the surface of tumour cells.

Keywords: Aquaporin-4; Neuromyelitis optica; Paraneoplastic.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Aquaporin 4 / immunology
  • Autoantibodies / analysis
  • Autoantibodies / blood
  • Autoantibodies / cerebrospinal fluid
  • Carcinoma, Squamous Cell / complications
  • Female
  • Humans
  • Immunohistochemistry
  • Lung Neoplasms / complications
  • Magnetic Resonance Imaging
  • Neuromyelitis Optica / complications
  • Neuromyelitis Optica / diagnostic imaging
  • Neuromyelitis Optica / therapy*
  • Paraneoplastic Polyneuropathy / diagnostic imaging
  • Paraneoplastic Polyneuropathy / etiology
  • Paraneoplastic Polyneuropathy / therapy*
  • Smoking
  • Spine / diagnostic imaging

Substances

  • AQP4 protein, human
  • Aquaporin 4
  • Autoantibodies