Myelin oligodendrocyte glycoprotein antibody-associated cerebral cortical encephalitis with super-refractory status epilepticus.

A 9-year-old boy with fever, headache, and altered consciousness developed super-refractory status epilepticus due to myelin oligodendrocyte glycoprotein antibody-associated cerebral cortical encephalitis (MOG-CCE). Seizures were initially controlled with high-dose thiopental but recurred upon dose reduction, and long-term thiopental caused adverse effects. Continuous infusion of ketamine and intrathecal dexamethasone therapy promptly arrested the seizures. The patient tested positive for anti-MOG antibodies in cerebrospinal fluid and serum. After three courses of intravenous methylprednisolone pulse therapy followed by a 6-month taper of oral prednisolone, he had no relapse for 6 months. Ketamine and intrathecal dexamethasone may be useful for seizure control in MOG-CCE with super-refractory status epilepticus.

Study at a glance

Abstract

Seizures are commonly reported in patients with myelin oligodendrocyte glycoprotein antibody-associated cerebral cortical encephalitis (MOG-CCE). However, seizure management during the acute phase has not been established. A 9-year-old previously healthy boy presented with fever persisting for approximately 6 days, along with headache and altered consciousness. Plain T2-weighted and fluid-attenuated inversion recovery imaging showed swelling and abnormal hyperintense lesions in the bilateral frontal, parietal, temporal, and insular cortices with left hemisphere predominance. Consciousness disturbance persisted, and focal myoclonic seizures clustered hourly. Seizures were arrested by titrating the thiopental dose but recurred with dose reduction, and the patient exhibited super refractory status epilepticus. Adverse effects due to long-term use of thiopental became apparent. Hence, continuous infusion of ketamine and intrathecal dexamethasone therapy (IT-DEX) was started. After administration of ketamine and IT-DEX, his seizure was arrested promptly. The cerebrospinal fluid and serum at the time of transfer were clear positive for ani-MOG antibody; therefore, the patient was diagnosed with MOG-CCE. The patient received three courses of intravenous methylprednisolone pulse therapy, followed by oral prednisolone gradually tapered over 6 months. He did not experience any relapse for 6 months. In MOG-CCE, some cases may present with super-refractory status epilepticus (SRSE) in the acute phase and be refractory to anti-seizure medication, analogous to febrile infection-related epilepsy syndrome. IT-DEX and continuous infusion ketamine are useful for seizure control in MOG-CCE.