Progressive cerebral atrophy in neuromyelitis optica

Mult Scler. 2015 Dec;21(14):1872-5. doi: 10.1177/1352458515600246. Epub 2015 Sep 10.


We report two cases of neuromyelitis optica patients with progressive cerebral atrophy. The patients exhibited characteristic clinical features, including elderly onset, secondary progressive tetraparesis and cognitive impairment, abnormally elevated CSF protein and myelin basic protein levels, and extremely highly elevated serum anti-AQP-4 antibody titer. Because neuromyelitis optica pathology cannot switch from an inflammatory phase to the degenerative phase until the terminal phase, neuromyelitis optica rarely appears as a secondary progressive clinical course caused by axonal degeneration. However, severe intrathecal inflammation and massive destruction of neuroglia could cause a secondary progressive clinical course associated with cerebral atrophy in neuromyelitis optica patients.

Keywords: Multiple sclerosis; anti-aquaporin-4 antibody; astrocyte; axonal degeneration; inflammation; neuromyelitis optica; progressive cerebral atrophy; secondary progressive.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Atrophy / pathology
  • Cerebrum / pathology*
  • Disease Progression
  • Female
  • Humans
  • Middle Aged
  • Neuromyelitis Optica / blood
  • Neuromyelitis Optica / cerebrospinal fluid
  • Neuromyelitis Optica / pathology*
  • Neuromyelitis Optica / physiopathology
  • White Matter / pathology*