A prominent elevation of glial fibrillary acidic protein in the cerebrospinal fluid during relapse in neuromyelitis optica.
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.
Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Rina
R
Takano
Tatsuro
T
Misu
Toshiyuki
T
Takahashi
Masahiro
M
Izumiyama
Kazuo
K
Fujihara
Yasuto
Y
Itoyama
D000328:Adult; D051401:Aquaporin 4 / Q000276:immunology / Q000378:metabolism; D015415:Biomarkers / Q000134:cerebrospinal fluid; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000134:cerebrospinal fluid*; D006801:Humans; D007275:Injections, Intravenous; D008775:Methylprednisolone / Q000627:therapeutic use; D009471:Neuromyelitis Optica / Q000134:cerebrospinal fluid* / Q000175:diagnosis / Q000276:immunology; D012008:Recurrence; D014786:Vision Disorders / Q000134:cerebrospinal fluid / Q000175:diagnosis
D051401:Aquaporin 4; D015415:Biomarkers; D005904:Glial Fibrillary Acidic Protein; D008775:Methylprednisolone