MOG antibody–positive, benign, unilateral, cerebral cortical encephalitis with epilepsy

Abstract

Objective:To describe the features of adult patients with benign, unilateral cerebral cortical encephalitis positive for the myelin oligodendrocyte glycoprotein (MOG) antibody.Methods:In this retrospective, cross-sectional study, after we encountered an index case of MOG antibody–positive unilateral cortical encephalitis with epileptic seizure, we tested for MOG antibody using our in-house, cell-based assay in a cohort of 24 consecutive adult patients with steroid-responsive encephalitis of unknown etiology seen at Tohoku University Hospital (2008–2014). We then analyzed the findings in MOG antibody–positive cases.Results:Three more patients, as well as the index case, were MOG antibody–positive, and all were adult men (median age 37 years, range 23–39 years). The main symptom was generalized epileptic seizure with or without abnormal behavior or consciousness disturbance. Two patients also developed unilateral benign optic neuritis (before or after seizure). In all patients, brain MRI demonstrated unilateral cerebral cortical fluid-attenuated inversion recovery hyperintense lesions, which were swollen and corresponded to hyperperfusion on SPECT. CSF studies showed moderate mononuclear pleocytosis with some polymorphonuclear cells and mildly elevated total protein levels, but myelin basic protein was not elevated. A screening of encephalitis-associated autoantibodies, including aquaporin-4, glutamate receptor, and voltage-gated potassium channel antibodies, was negative. All patients received antiepilepsy drugs and fully recovered after high-dose methylprednisolone, and the unilateral cortical MRI lesions subsequently disappeared. No patient experienced relapse.Conclusions:These MOG antibody–positive cases represent unique benign unilateral cortical encephalitis with epileptic seizure. The pathology may be autoimmune, although the findings differ from MOG antibody–associated demyelination and Rasmussen and other known immune-mediated encephalitides.

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease: practical considerations

“…MR scan of the brain in the acute phase usually shows unilateral mildly oedematous cortical lesion(s) best seen on fluid-attenuated inversion recovery images (figure 3). The prognosis is typically good, with the seizures and MRI brain lesions resolving,14 although there are exceptions 15…”

Is MOG-IgG another biomarker for neuromyelitis optica?

“…The spectrum of MOG-IgG-related diseases seems to be much broader than we had previously thought, suggesting that MOG-IgG is much more important in paediatric patients who develop encephalopathy as acute disseminated encephalomyelitis (ADEM) rather than in patients with NMOSD 2. Some cases of adults with cortical encephalitis and epilepsy related to MOG-IgG have been reported 3. We may overlook the true characteristics of MOG-IgG-related diseases when only looking at limited forms of the disease.…”

Clinical Reasoning: A patient with a history of encephalomyelitis and recurrent optic neuritis

“…Interestingly, seizures associated with ON, as noted in our patient, have been associated with anti-MOG antibodies in a few patients in Japan. 10 While testing is currently only available in specialized research settings, it is expected that rigorous testing for anti-IgG1 isotype of anti-MOG antibodies will become commercially available in the near future.…”