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Myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory demyelinating disease affecting the central nervous system. MOGAD is commonly associated with acute disseminated encephalomyelitis, optic neuritis, and transverse myelitis. Disease severity may be moderate to severe with an Expanded Disability Status Scale (EDSS) above 4 in half of patients, albeit most experience good to excellent motor recovery. 

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Herein, we present an exceptionally severe case of MOGAD with an unusual clinical course in a 50-year-old Caucasian female. Patient initially presented with diplopia, lower limb motor deficit and hypoesthesia which rapidly deteriorated into quadriplegia as well as a cerebrospinal fluid white blood cell count of 192x10⁶/L. High doses of intravenous corticosteroids, which ameliorated MRI findings, did not initially translate to significant clinical improvement (EDSS=9.0). Nevertheless, patient progressively improved over several months with courses of corticosteroids, maintenance Prednisone treatment and rehabilitation work, including physiotherapy and ergotherapy. At the 1 year follow up, patient had regained some mobility (EDSS=6.5) although with marked sequela. 

This exceptional case is an important reminder that MOGAD can present in atypical ways. Severe symptoms followed by an incomplete recovery as well as an abnormally high white blood cell count in cerebrospinal fluid were seen in this unusual presentation, making this case challenging to diagnose. Therefore, the development of formal criteria and more research into the pathophysiology of the disease will surely contribute to the timely diagnosis of MOGAD as well as expand on existing therapeutic options, notably in fulminant cases.