Multicystic demyelinating myelopathy: widening spectrum of pediatric aquaporin-4 autoimmunity

Neurology. 2014 Mar 11;82(10):902-3. doi: 10.1212/WNL.0000000000000192.

Abstract

A 10-year-old girl presented with subacute lower limb weakness and gait ataxia. MRI revealed a large multicystic spinal cord lesion with patchy enhancement (figure 1, A–B) and 3 small (<6 mm) periventricular and deep white matter brain lesions. The presence of serum anti-aquaporin-4 immunoglobulin G (AQP4) (ELISA assay) and compatible neuropathologic features from neurosurgical specimens1 (figure 2) suggested the diagnosis of a neuromyelitis optica spectrum disorder.2 Targeted immunotherapy was started with partial lesion resolution (figure 1C).

Publication types

  • Case Reports

MeSH terms

  • Aquaporin 4 / immunology*
  • Autoimmunity / immunology*
  • Brain / pathology*
  • Child
  • Demyelinating Diseases / immunology
  • Demyelinating Diseases / pathology*
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Neuromyelitis Optica / immunology
  • Neuromyelitis Optica / pathology*
  • Spinal Cord / pathology*

Substances

  • AQP4 protein, human
  • Aquaporin 4