Uncommon inflammatory/immune-related myelopathies

J Neuroimmunol. 2021 Dec 15:361:577750. doi: 10.1016/j.jneuroim.2021.577750. Epub 2021 Oct 13.

Abstract

The differential diagnosis for immune-mediated myelopathies is broad. Although clinical manifestations overlap, certain presentations are suggestive of a particular myelopathy etiology. Spine MRI lesion characteristics including the length and location, and the pattern of gadolinium enhancement, help narrow the differential diagnosis and exclude an extrinsic compressive cause. The discovery of specific antibodies that serve as biomarkers of myelitis such as aquaporin-4-IgG and myelin-oligodendrocyte -glycoprotein-IgG (MOG-IgG), has improved our understanding of myelitis pathophysiology and facilitated diagnosis. In this review we will focus on the pathophysiology, clinical presentation, imaging findings and treatment and outcomes of uncommon immune-mediated myelopathies.

Keywords: Aquaporin 4; Autoimmune glial fibrillary acidic protein astrocytopathy; Myelin oligodendrocyte glycoprotein antibody-associated disease; Neuromyelitis optica spectrum disorders; Paraneoplastic myelopathy; Spinal cord sarcoidosis.

Publication types

  • Review

MeSH terms

  • Aquaporin 4
  • Autoantibodies / blood
  • Autoantibodies / immunology
  • Biomarkers
  • Contrast Media
  • Diagnosis, Differential
  • Gadolinium
  • Humans
  • Magnetic Resonance Imaging
  • Myelitis* / diagnosis
  • Myelitis* / drug therapy
  • Myelitis* / immunology
  • Myelitis* / physiopathology
  • Rare Diseases / diagnosis
  • Rare Diseases / drug therapy
  • Rare Diseases / immunology

Substances

  • AQP4 protein, human
  • Aquaporin 4
  • Autoantibodies
  • Biomarkers
  • Contrast Media
  • Gadolinium