Paracentral Acute Middle Maculopathy Associated with Severe Anti-Mog (Myelin Oligodendrocyte Glycoprotein)-Positive Optic Neuritis

Abstract

Retinal complications in patients with inflammatory optic neuritis (ON) are generally related to post-infectious neuroretinitis and are considered uncommon in autoimmune/demyelinating ON, whether isolated or caused by multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD). More recently, however, cases with retinal complications have been reported in subjects positive for myelin oligodendrocyte glycoprotein (MOG) antibodies. We report a 53-year-old woman presenting with severe bilateral ON associated with a focal area of paracentral acute middle maculopathy (PAMM) in one eye. Visual loss recovered remarkably after high-dose intravenous corticosteroid treatment and plasmapheresis, but the PAMM lesion remained visible on both optical coherence tomography and angiography as an ischaemic lesion affecting the middle layers of the retina. The report emphasises the possible occurrence of retinal vascular complications in MOG-related optic neuritis, an important addition to the diagnosis of, and possible differentiation from, MS-related or NMOSD-related ON.

Keywords: Optic Neuritis; myelin oligodendrocyte glycoprotein; neuromyelitis optica spectrum disorder; paracentral acute middle maculopathy; retinal ischemia.

Figure 1.

Fundus photographs at the first neuro-ophthalmological evaluation, 3 days after onset of methylprednisolone treatment. (a) Right eye (left image) showing mild optic disc oedema. Left eye (right image) showing mild optic disc oedema, small inferior peripapillary splinter haemorrhage, small macular intraretinal haemorrhage (arrowhead) and an area of perifoveal retinal pallor (between arrows). Note the cilioretinal artery projecting from the left optic disc at the 5 o’clock meridian. (b) Fundus photographs of both eyes at the last neuro-ophthalmological evaluation after 5 days of methylprednisolone, five sessions of plasmapheresis and oral prednisone treatment. Right eye (left image) with no signs of optic disc oedema. Left eye (right image) showing no signs of optic disc oedema, resolution of peripapillary and intraretinal haemorrhages, and reduction of the area of perifoveal retinal pallor.

Figure 2.

Spectral-domain optical coherence tomography line scan passing through the macula (right), as indicated by the scanning laser ophthalmoscopy image (left). (a) At presentation, showing perifoveal hyperreflectivity in the inner nuclear and plexiform layers (arrows), compatible with paracentral acute middle maculopathy. (b) At the follow-up examination after treatment, showing reduction of paracentral hyperreflectivity and areas of inner nuclear layer thinning (arrows).

Figure 3.

Magnetic resonance imaging. (a) Axial T2-weighted image showing hyperintensity in the anterior portion of both optic nerves (arrows). (b) Axial post-contrast T1-weighted image showing enhancement of both optic nerves, together with perineural enhancement signalling perineuritis (arrows). (c) Coronal T2-weighted image and (d) post-contrast T1-weighted image with similar findings (arrows). T1-weighted images also show mild enhancement of the orbital fat (b and d).

Figure 4.

Optical coherence tomography (OCT) angiography (OCTA) of the superficial (a) and deep (b) vascular complexes showing reduced perfusion inferior and nasal to the avascular foveal zone in the deep vascular complex. In (a) and (b) the upper image shows the level OCTA segmentation, the left image the superficial (a) and deep (b) vascular complexes and the right image shows the corresponding en-face spectral domain OCT image at each of the two levels showing patchy paracentral area of increased reflectivity.