Título

PARACENTRAL ACUTE MIDDLE MACULOPATHY IN A PATIENT WITH SEVERE BILATERAL CORTICOSTEROID-RESPONSIVE PRESUMABLY MOG-RELATED OPTIC NEURITIS

Objetivo

To document a patient with bilateral severe optic neuritis (ON) and paracentral acute middle maculopathy (PAMM) in one eye. Clinical and imaging data suggested she had MOG-related neuromyelitis optica spectrum disorder (NMOSD), making it a novel association.

Relato do Caso

53-year-old woman presented with periocular pain and progressive bilateral sequential blurry vision. Visual loss progressed to complete bilateral blindness over a 10-day period. At presentation, she had no light perception and optic disc edema OU, and was started on IV methylprednisolone one gram daily. Three days later, visual acuity (VA) improved to hand motion OD and finger counting OS. Pupils reacted sluggishly to light with a mild relative afferent pupillary defect OD. Fundus exam showed mild optic disc edema OU, with some small hemorrhages. A patchy whitish area, extending temporally from the disc to the fovea, was detected OS (Figure 1, arrows), along with a small intraretinal hemorrhage in the inferior border (arrowhead); OCT scan showed increased hyperreflectivity at the inner nuclear (INL) and both plexiform layers, compatible with PAMM. Orbital magnetic resonance imaging revealed signs of bilateral ON with perineuritis. Further investigation was unremarkable, including a negative serum anti aquaporin-4; however, anti-MOG testing was unavailable. Plasmapheresis led to further improvement. At the last exam, VA was 20/100 OD and 20/40 OS, optic disc edema resolved leaving temporal pallor OU, and the retinal lesion was not evident. OCT showed reduction of the hyperreflectiveness and thinning of the INL, and OCT angiography revealed reduced deep vascular complex perfusion, while en-face OCT structural analyses demarcated the affected area (figure 2).

Conclusão

This case is important to emphasize the possible occurrence of retinal vascular complications in NMOSD, which could be a relevant additional sign to help diagnose this condition and differentiate it from typical demyelinating ON.