Abstract
Objective: Neuromyelitis optica spectrum disorder (NMOsd) is an autoimmune disorder of the CNS characterized by aquaporin-4 (AQP4) autoantibodies. The aim of this study was to elucidate the characteristics of involvement of the anterior visual pathway (AVP) and neurodegeneration via glia-neuron interaction in NMOsd.
Methods: Thirty Japanese patients with serologically verified NMOsd were assessed with a neuro-ophthalmological study. Using 27 tissue blocks from 13 other cases of NMOsd, we performed neuropathological analysis of glial and neuroaxonal involvement in the AVP.
Results: The AVP involvement in NMOsd was characterized by the followings, compared to multiple sclerosis: 1) longitudinally extensive optic neuritis (ON); 2) more severe visual impairment and worse prognosis for ON; 3) unique AQP4 dynamics – (i) loss of AQP4 immunoreactivity on astrocytes with complement activation in ON lesions, (ii) loss of AQP4 immunoreactivity on Müller cells with no deposition of complement in the retinas, and (iii) densely packed AQP4 immunoreactivity on astrocytes in gliosis of secondary antero/retrograde degeneration in the optic nerves and retinal nerve fiber layer (RNFL); and 4) more severe neurodegeneration – (i) axonal accumulation of degenerative mitochondria and transient receptor potential melastatin 4 channel with complement-dependent astrocyte pathology in ON lesions, (ii) mild loss of horizontal cells, and (iii) RNFL thinning and loss of ganglion cells with abundance of AQP4+ astrocytes, indicating secondary retrograde degeneration after ON.
Interpretation: Severe and widespread neuroaxonal damage and unique dynamics of astrocytes/Müller cells with alterations of AQP4 was prominent in the AVP and may be associated with poor visual function and prognosis in NMOsd. This article is protected by copyright. All rights reserved.
from #MedicinebyAlexandrosSfakianakis via xlomafota13 on Inoreader http://ift.tt/1UGfowe
via IFTTT
Δεν υπάρχουν σχόλια:
Δημοσίευση σχολίου