Clinicopathological features in anterior visual pathway in neuromyelitis optica

Abstract

Severe and widespread neuroaxonal damage and unique dynamics of astrocytes/Müller cells with alterations of AQP4 were prominent in the AVP and may be associated with poor visual function and prognosis in NMOsd.

Neurodegeneration in multiple sclerosis and neuromyelitis optica

“…Involvement of the optic nerves and the spinal cord in NMO is associated with severe visual impairment and poor prognosis of optic neuritis79 or more severe motor/sensory disabilities for myelitis, in comparison to the respective changes seen in MS. A severe phenotype of NMO is associated with profound tissue damage including neuroaxonal injury, occurring as a secondary consequence of astrocyte damage by AQP4 antibodies. Related to optic neuritis as a representative NMO lesion, the pathological correlates of the severity in NMO have been provided as follows:79 (1) Longitudinally extensive optic neuritis.…”

“…Related to optic neuritis as a representative NMO lesion, the pathological correlates of the severity in NMO have been provided as follows:79 (1) Longitudinally extensive optic neuritis. (2) Unique dynamics of AQP4, including (i) loss of AQP4 immunoreactivity on astrocytes and complement activation in optic neuritis lesions, (ii) loss of AQP4 immunoreactivity on Müller cells and no complement activation and (iii) densely packed AQP4 immunoreactivity on astrocytes in gliosis at sites of secondary anterograde/retrograde degeneration in the optic nerves and RNFL.…”

“…(2) Unique dynamics of AQP4, including (i) loss of AQP4 immunoreactivity on astrocytes and complement activation in optic neuritis lesions, (ii) loss of AQP4 immunoreactivity on Müller cells and no complement activation and (iii) densely packed AQP4 immunoreactivity on astrocytes in gliosis at sites of secondary anterograde/retrograde degeneration in the optic nerves and RNFL. (3) More severe neurodegenerative processes with accumulation of damaged mitochondria and transient receptor potential melastatin 4 channel (TRPM4) in axons with astrocyte pathology via CDC in optic neuritis lesions 79. RNFL thinning and loss of ganglion cells in the presence of AQP4 + astrocytes indicate secondary retrograde degeneration after optic neuritis 79.…”

“…(3) More severe neurodegenerative processes with accumulation of damaged mitochondria and transient receptor potential melastatin 4 channel (TRPM4) in axons with astrocyte pathology via CDC in optic neuritis lesions 79. RNFL thinning and loss of ganglion cells in the presence of AQP4 + astrocytes indicate secondary retrograde degeneration after optic neuritis 79. Furthermore, in vivo imaging of the spinal cord to study the formation of experimental NMO-related lesions caused by human AQP4 antibody in mice demonstrated that human AQP4 antibody results in acute depletion of astrocytes with initial oligodendrocyte survival 96.…”

The 3rd MS Summer College in Kobe (6–7 August 2016)Practical issues and new horizons in MS, NMOSD and related disorders

“…Finally, she demonstrated severe and widespread neuroaxonal damage, including increased mitochondrial content with unique dynamics of AQP4 expression on astrocytes in the optic nerve and M€ uller cells in the retina. 2 These findings suggested that neurodegeneration in the optic nerve might be associated with the poor recovery of visual function in patients with NMO. The next session was a poster session for submitted abstracts.…”