Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease in a patient with Sjogren's Syndrome: A Case Report and Literature Review
Mattia Wruble1, Rebecca Salky2, Tanuja Chitnis1, Michael Levy3, Shamik Bhattacharyya4
1Neurology, Mass General Brigham, 2Neurology, Massachusetts General Hospital, 3Massachusetts General Hospital/Harvard Medical School, 4Neurology, Brigham and Women's Hospital
Objective:
To report a case of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) in a patient with Sjogren's Syndrome (SS), and to perform a literature review examining cases of concurrent MOGAD and SS.
Background:
Few cases of co-occurrent MOGAD and SS have been reported, and the clinical significance of comorbid SS in patients with MOGAD remains poorly understood.
Design/Methods:
A PubMed search was performed using the following terms: “((myelin oligodendrocyte glycoprotein) OR (MOG) OR (MOGAD)) AND ((Sjogren's) OR (Sjogrens) OR (Sjögren's) OR (Sjögrens)).”
Results:
A 30-year-old woman first presented with acute disseminated encephalomyelitis (ADEM) at age 4. Over the next 18 years, she experienced over 10 relapses (involving unilateral or bilateral optic neuritis [ON], brainstem syndromes, and one cerebral syndrome) before achieving remission with intravenous immunoglobulin G. At age 16, she tested positive for serum anti-MOG antibodies. She was later diagnosed with psoriasis and SS.
Literature review identified four additional patients (three [75%] female) with concurrent MOGAD and SS, who ranged in age from 13-45 years at neurologic presentation. MOGAD syndromes included ON in two (50%) patients: one with longitudinally extensive ON, and one with bilateral ON then unilateral ON several years later. Two (50%) patients had transverse myelitis: one was longitudinally extensive, and the other involved the conus. Three (75%) patients had monophasic courses at 1-6 years of follow-up, while one (25%) patient experienced one relapse. Acute treatment involved steroids in all four cases, with the addition of PLEX in one case. Only one patient was treated with chronic immunotherapy (one year of azathioprine). Though our patient developed permanent visual deficits, outcomes in the other patients were favorable.
Conclusions:
This review identified five patients with concurrent SS and MOGAD, whose neurologic presentations and treatment responses were characteristic of MOGAD. These cases suggest that when present, comorbid SS does not significantly alter the course of MOGAD.
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