Cerebral Cortical Encephalitis Research Articles

MOG antibody‐positive cerebral cortical encephalitis: Two case reports and literature review

To investigate the clinical and MRI features of myelin oligodendrocyte glycoprotein (MOG) antibody-positive cerebral cortical encephalitis. To summarize the clinical manifestations, magnetic resonance imaging (MRI) features, and diagnosis and treatment of two children diagnosed with MOG antibody-positive cortical encephalitis in our hospital, and to analyze all of the literature on MOG antibody-positive associated cerebral cortical encephalitis published in the last 10years. A total of 24 patients, 20 adults, and 4 children were included. The main symptoms of all observed patients were as follows: seizures 20/24 (83%), headache 18/24 (75%), fever 13/24 (54%), and increased cell number in cerebrospinal fluid 22/24 (92%). The main radiological sign was enhanced FLAIR signal in cortical 24/24 (100%), including enhanced fluid-attenuated inversion recovery (FLAIR) signal in unilateral cortical 20/24 (83%). The clinical manifestations of MOG antibody-positive cerebral cortical encephalitis are complex and varied, and long-term follow-up is needed to clarify the prognosis and recurrence, providing reference for the clinical treatment.

Neuromyelitis optica spectrum disorder in Asia: Epidemiology and risk factors

AbstractIn Asia, prior to the discovery of aquaporin 4 (AQP4) antibody, many cases of neuromyelitis optica spectrum disorder (NMOSD) were misdiagnosed as multiple sclerosis (MS) or the so‐called opticospinal multiple sclerosis (OSMS). The AQP4 antibody discovery was an important milestone in the field of NMOSD, leading to correct diagnosis and reclassification of NMOSD cases, and the subsequent appropriate therapy. This is especially pertinent in Asia where the prevalence of MS is generally much lower than the Caucasian populations. East Asians have a higher NMOSD prevalence rate (~4/100 000) than Caucasians (~1‐1.5/100 000) and other Asian racial groups (Austronesians: 1.2‐1.5/100 000; South Asians: ~1/100 000; Arabs: ~1‐1.5/100 000). Myelin oligodendrocyte glycoprotein (MOG) antibody‐associated disease (MOGAD) comprises cases of AQP4‐antibody‐negative NMOSD, optic neuritis, myelitis, and certain unique phenotypes such as cases with acute disseminated encephalomyelitis (ADEM)‐like presentation, and cerebral cortical encephalitis. MOGAD appears not too uncommon among Japanese, Chinese, Sri Lankans and Indians, but data in different Asian populations and geographical locations are needed. There exist some differences in clinical features of NMOSD and MOGAD between Asians and other populations. As for risk factors, genetic studies have identified certain HLA associations with NMOSD among Asians that were different from other populations. North‐South latitudinal gradient as seen in MS was not observed in NMOSD in Japan and China. Seasonal variation and preceding infection are among some risk factors worth further investigations. Population‐based MOGAD study in Asia, and further genetic and environmental studies are useful to further inform the epidemiology and potential pathogenetic mechanism of this unique neuroinflammatory disease.

Clinical and Radiological Features of Adult Onset Bilateral Medial Frontal Cerebral Cortical Encephalitis With Anti-myelin Oligodendrocyte Glycoprotein Antibody.

Objective: To clarify the clinical and radiological features of adult onset anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated bilateral medial frontal cerebral cortical encephalitis (BFCCE).Methods: We systematically reviewed the literature for patients with anti-MOG antibody-associated BFCCE. Patients who were also positive for other encephalitis-related autoantibodies were excluded from the study. The frequency of several characteristic neurological symptoms and lesion distributions were analyzed.Results: We identified six patients with anti-MOG antibody-associated BFCCE. Among them, 6/6 had headache, 4/6 had fever, 3/6 had seizure, 2/6 had paraparesis, 2/6 had lethargy, and 2/6 had memory disturbance. CSF pleocytosis was observed in 5/6 patients, while CSF myelin basic protein was not elevated in any of the six patients. On brain MRI, 6/6 had bilateral medial frontal cortical lesions, 3/6 had corpus callosum lesions, and 3/6 had leptomeningeal enhancements. Most of the lesions were distributed in the territory of the anterior cerebral artery (ACA).Conclusion: Our results indicate that anti-MOG antibody-associated BFCCE presents with characteristic clinical symptoms and MRI findings, which might reflect lesion formation in the ACA territory.

Unilateral chronic pulsatile headache as the single manifestation of anti-MOG antibody-associated unilateral cerebral cortical encephalitis

Unilateral cerebral cortical encephalitis (UCCE) with myelin oligodendrocyte glycoprotein (MOG)-antibody comprises a new spectrum of disease entities generally presenting seizures. Here, we report a case of a young adult with anti-MOG antibody-associated UCCE who only presented persistent left pulsatile headache. Neurological examination revealed no deficits. Brain MRI showed a fluid-attenuated inversion recovery hyperintense lesion along the swollen left cerebral cortex. The patient was positive for anti-MOG antibodies. We diagnosed him with anti-MOG antibody-associated UCCE. Immediately after the administration of high-dose IV methylprednisolone, the headache diminished. Anti-MOG antibody-associated UCCE is a new differential diagnosis in patients with unilateral chronic pulsatile headache.

Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide.

Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon inflammatory disease of the central nervous system, manifesting clinically as optic neuritis, myelitis, and certain brain and brainstem syndromes. Cases clinically diagnosed as NMOSD may include aquaporin 4 (AQP4)-antibody-seropositive autoimmune astrocytopathic disease, myelin oligodendrocyte glycoprotein (MOG)-antibody-seropositive inflammatory demyelinating disease, and double-seronegative disease. AQP4-antibody disease has a high female-to-male ratio (up to 9:1), and its mean age at onset of ~40 years is later than that seen in multiple sclerosis. For MOG-antibody disease, its gender ratio is closer to 1:1, and it is more common in children than in adults. Its clinical phenotypes differ but overlap with those of AQP4-antibody disease and include acute disseminated encephalomyelitis, brainstem and cerebral cortical encephalitis, as well as optic neuritis and myelitis. Double-seronegative disease requires further research and clarification. Population-based studies over the past two decades report the prevalence and incidence of NMOSD in different populations worldwide. One relevant finding is the varying prevalence observed in different racial groups. Consistently, the prevalence of NMOSD among Whites is ~1/100,000 population, with an annual incidence of <1/million population. Among East Asians, the prevalence is higher, at ~3.5/100,000 population, while the prevalence in Blacks may be up to 10/100,000 population. For MOG-antibody disease, hospital-based studies largely do not observe any significant racial preponderance so far. This disorder comprises a significant proportion of NMOSD cases that are AQP4-antibody-seronegative. A recent Dutch nationwide study reported the annual incidence of MOG-antibody disease as 1.6/million population (adult: 1.3/million, children: 3.1/million). Clinical and radiological differences between AQP4-antibody and MOG-antibody associated diseases have led to interest in the revisions of NMOSD definition and expanded stratification based on detection of a specific autoantibody biomarker. More population-based studies in different geographical regions and racial groups will be useful to further inform the prevalence and incidence of NMOSD and their antibody-specific subgroups. Accessibility to AQP4-antibody and MOG-antibody testing, which is limited in many centers, is a challenge to overcome. Environmental and genetic studies will be useful accompaniments to identify other potential pathogenetic factors and specific biomarkers in NMOSD.

Improvement in recurrent anti-myelin oligodendrocyte glycoprotein antibody - positive cerebral cortical encephalitis not requiring anti - inflammatory therapy following the decrease in cytokine/chemokine levels

Improvement in recurrent anti-myelin oligodendrocyte glycoprotein antibody - positive cerebral cortical encephalitis not requiring anti - inflammatory therapy following the decrease in cytokine/chemokine levels

MOG antibody-associated cerebral cortical encephalitis in females

MOG antibody-associated cerebral cortical encephalitis in females

MOG antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy.

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.