A Case of Recurrent Cerebral Cortical Encephalitis in MOG Antibody-Associated Disease

Abstract

Objective NA. Background Cerebral cortical encephalitis is a recently recognized syndrome of myelin-oligodendrocyte-glycoprotein-antibody-associated disease (MOGAD), yet most descriptions report single episodes without recurrence. Design/Methods Case report of recurrent cerebral cortical encephalitis in MOGAD. Results This is a case report of a 32-year-old patient who initially developed meningoencephalitis when he was three. He was stable until age 31, when he developed headaches followed by status epilepticus secondary to cerebral cortical encephalitis with accompanying unilateral right temporal cerebral cortical T2-hyperintensity with leptomeningeal enhancement. Cerebrospinal fluid analysis revealed 505 white cells/µl (normal, 0-5) but oligoclonal bands were negative. Over the subsequent two weeks he developed bilateral severe vision loss (20/400 bilaterally) accompanied by bilateral optic disc edema, and MRI orbits showed bilateral anterior segment optic nerve enhancement extending >50% of the length of each nerve, confirming bilateral optic neuritis. MOG-IgG was tested in serum and was positive at a titer of 1:100. He was treated with intravenous methylprednisolone 1 gram daily for 5 days and a subsequent slow oral prednisone taper along with anti-epileptic treatment with levetiracetam, later transitioned to oxcarbazepine, although he later self-discontinued all anti-epileptics. He received maintenance intravenous immunoglobulins (IVIg) 0.4 g/Kg weekly, that was later weaned to 1 g/Kg every 4 weeks. He then developed recurrent cerebral cortical encephalitis 18 months after his prior episode manifesting with seizures and left occipito-temporal T2-hyperintensity and swelling with leptomeningeal enhancement. He was treated with high dose IV steroids, an oral prednisone taper and his IVIg dose was changed back to 0.4 g/Kg once weekly and anti-seizure medications reinitiated. Conclusions Cerebral cortical encephalitis in MOGAD can be recurrent. Close scrutiny of the MRI in patients with MOGAD who develop seizures after prior cerebral cortical encephalitis is important as it can discriminate a recurrent cerebral cortical encephalitis attack from a breakthrough seizure related to prior damage.