Spontaneous remission lasting 3 decades in myelin oligodendrocyte glycoprotein disease

In February 2017, a 52-year-old woman was brought to our clinic after abrupt right vision loss. Four days later, both eyes were affected, bilateral optic neuritis (ON) was diagnosed, and the patient was treated with a short course of corticosteroids. Her medical history revealed she was admitted at the age of 18 years with severe paraparesis, followed 4 weeks later by right vision loss. With a short course of corticosteroids, the patient achieved full recovery, but no etiology was found. Myelin oligodendrocyte glycoprotein (MOG)-IgG positivity and aquaporine-4 antibodies (AQP4-IgG) absence (cell-based immunoassay, analysed by microscopy) were confirmed in the present attack (figure for case description).

Figure. Timeline with full description of the patient attacks.

AQP4-IgG = aquaporine-4 antibodies; MOG-IgG = myelin oligodendrocyte glycoprotein antibodies.

We report a patient with MOG-IgG disease with a spontaneous remission lasting 34 years. Extended periods of spontaneous remissions were previously reported in AQP4-IgG disease,¹ but this benign course would be extremely unlikely in AQP4-IgG-associated demyelination. Although samples of serum or CSF were not available to confirm MOG-IgG seropositivity at the age of 18 years, the previous attacks are likely part of the clinical syndrome. In our patient, myelitis was the presenting attack despite ON being the most frequent phenotype at disease onset.² In line with results from the MOGADOR study,² our patient experienced variable intervals between attacks and a good recovery from them. Our case highlights that a new attack may occur many years after being in remission without immunosuppression in MOG-IgG disease. The need of long-term immunosuppression and its duration in this and other similar patients remains uncertain.

Author contributions

D. Gabriel and S. Duarte: drafting and revising the manuscript. R. Felgueiras: drafting and revising the manuscript, data acquisition, study concept or design, and analysis or interpretation of data. A.M. Silva: drafting and revising the manuscript and analysis or interpretation of data. E. Santos: drafting and revising the manuscript, data acquisition, study concept or design, analysis or interpretation of data, and study supervision.

Study funding

No targeted funding reported.

Disclosure

The authors report no disclosures relevant to the manuscript. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp.