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Review
. 2017 Sep;37(3):291-299.
doi: 10.1097/WNO.0000000000000508.

Neuromyelitis Optica: Deciphering a Complex Immune-Mediated Astrocytopathy

Affiliations
Review

Neuromyelitis Optica: Deciphering a Complex Immune-Mediated Astrocytopathy

Jeffrey L Bennett et al. J Neuroophthalmol. 2017 Sep.
No abstract available

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Conflict of interest statement

The authors report no conflicts of interest.

Figures

FIG. 1
FIG. 1
Inflammatory and noninflammatory mechanisms contributing to astrocyte injury in neuromyelitis optica. Inflammatory mechanisms include complement-dependent cytotoxicity (CDC), antibody-dependent cell-mediated cytotoxicity (ADCC), opsonization, and complement-induced degranulation. Potential noninflammatory mechanisms contributing to injury include aquaporin-4 (AQP4) and glutamate transporter (EAAT2) internalization and direct inhibition of AQP4-mediated water transport. AP, alternative pathway; C2aC4bC3b, C5 convertase; C4bC2a, C3 convertase; CP, classical pathway; MAC, membrane attack complex; NK, natural killer.
FIG. 2
FIG. 2
Myelinolysis and progressive axonal swelling following astrocyte injury in neuromyelitis optica lesions. Astrocyte destruction mediated by AQP4-IgG results in early myelinolysis and progressive axonal swelling. The potential mechanisms driving these pathologies include inflammatory, metabolic, ionic, and excitotoxic mechanisms. Progressive axonal swellings identified in intravital microscopy may represent periaxonal swelling or myelin injury (splitting or focal bulging). Adapted from (27). Ca2+, calcium.

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