Summary

A healthy 38-year-old woman developed sudden unilateral vision loss due to retrobulbar optic neuritis in the wake of varicella-zoster virus infection. She had no further central nervous system (CNS) lesions. Antinuclear antibodies (ANA) and anti-aquaporin 4 antibodies were found, consistent with neuromyelitis optica (NMO). Later, serial MRIs showed dynamic short-segment and long-segment myelitis lesions, ANA titre increased and additional autoantibodies were found including anti-dsDNA, anti-chromatin/nucleosome and antiphospholipid antibodies. In that setting, NMO can be regarded a rare presenting manifestation of systemic lupus erythematosus (SLE). The relevant literature is reviewed and the implications of NMO spectrum disorder demyelinating syndromes as the first manifestation of SLE (with or without antiphospholipid syndrome) (APS) or their later development (in a patient diagnosed with SLE) as part of the spectrum of neuropsychiatric SLE are analysed in view of recent research developments in the field.