A prominent elevation of glial fibrillary acidic protein in the cerebrospinal fluid during relapse in neuromyelitis optica

Tohoku J Exp Med. 2008 May;215(1):55-9. doi: 10.1620/tjem.215.55.


Neuromyelitis optica (NMO) is a neurologic disease characterized by severe optic neuritis, longitudinally extended, transverse myelitis and serum aquaporin-4 (AQP4) antibody. Our recent neuropathological study revealed the extensive loss of AQP4 and glial fibrillary acidic protein (GFAP), an astrocyte-specific protein, in NMO lesions, but not in MS lesions, suggesting that severe astrocytic damage or dysfunction may be related to the pathogenesis of NMO. Here we report a patient of NMO, in which the cerebrospinal fluid (CSF) levels of GFAP were measured both during relapse of myelitis and after high-dose intravenous methylprednisolone (HIMP). The patient was a 34-year old woman with two previous episodes of optic neuritis. She developed myelitis longitudinally extending from C3 to T12 with contrast enhancement, and was AQP4 antibody-positive. In the acute phase, the GFAP level in the cerebrospinal fluid (CSF) was prominently elevated (18,966.7 ng/ml) as compared with controls (0.6 +/- 0.33 ng/ml). However, following HIMP, the clinical and MRI findings improved, and the CSF-GFAP level was near-normal (2.1 ng/ml). The CSF of myelin basic protein was also elevated in relapse (1,016.0 pg/ml), and became lower but still remained high (158.7 pg/ml) after HIMP compared with controls (3.36 +/- 3.83 pg/ml). The prominent elevation of the CSF-GFAP level in relapse of NMO, followed by its sharp decline after therapy, suggests severe astrocytic damage with a temporal profile distinct from that of the demyelinating process in NMO. CSF-GFAP may be useful as a biomarker of NMO.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aquaporin 4 / immunology
  • Aquaporin 4 / metabolism
  • Biomarkers / cerebrospinal fluid
  • Female
  • Glial Fibrillary Acidic Protein / cerebrospinal fluid*
  • Humans
  • Injections, Intravenous
  • Methylprednisolone / therapeutic use
  • Neuromyelitis Optica / cerebrospinal fluid*
  • Neuromyelitis Optica / diagnosis
  • Neuromyelitis Optica / immunology
  • Recurrence
  • Vision Disorders / cerebrospinal fluid
  • Vision Disorders / diagnosis


  • Aquaporin 4
  • Biomarkers
  • Glial Fibrillary Acidic Protein
  • Methylprednisolone