BBA Clin. 2015 Jan 15;3:126-34. doi: 10.1016/j.bbacli.2015.01.003. eCollection 2015. Kariya Y1, Kariya Y1, Saito T1, Nishiyama S2, Honda T3, Tanaka K4, Yoshida M5, Fujihara K2,…
The aquaporin-4 (AQP4) water channel antibody is used in the diagnosis of neuromyelitis optica (NMO) due to its high sensitivity and high specificity. However, some patients are reported to have neither optic neuritis nor myelitis despite being positive for the AQP4-autoantibody (AQP4-Ab). Therefore, recent reports suggest that such patients should be diagnosed as having ‘AQP4-autoimmune syndrome’.
Background: Neuromyelitis optica (NMO) is a human inflammatory/demyelinating disease of the central nervous system characterized by optic neuritis and longitudinally extensive transverse myelitis.
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).
INTRODUCTION: Loss of aquaporin 4 and glial fibrillary acidic protein (GFAP) with necrosis and demyelination is a prominent pathologic feature of neuromyelitis optica (NMO). However, the clinicopathologic significance of astrocytic damage and its relation with demyelination are unknown