A case of unilateral recurrent cerebral cortical encephalitis with anti-myelin oligodendrocyte glycoprotein antibodies.

Myelin oligodendrocyte glycoprotein (MOG)-antibody-associated disease (MOGAD) is an independent inflammatory demyelinating disease. A rare phenotype of MOGAD is cerebral cortical encephalitis (CCE). This case report presents unilateral recurrent cerebral cortical encephalitis (CCE) with positive anti-MOG antibodies from a 55 year old man who was admitted with headache, fever and aphasia. This case highlights the findings of hyperintense lesions in the cortex of the right temporal gyrus with slight swelling on T2 FLAIR and anti MOG antibodies in serum (1:20) and CSF (1:80) when the patient presented again to hospital after the initial improvement with IVIG and glucocorticoids. In addition, the patient was found to have atrophy of the whole brain, especially the right temporal lobe, after becoming symptom-free with glucocorticoids. In summary, anti-MOG-associated CCE can be diagnosed with headache, fever, and seizures associated with the presence of anti-MOG antibodies. Unilateral CCE is a special clinical feature of MOGAD and cerebral atrophy can be found. Steroid therapy remains to be the standard treatment.

Guo L ，Yun HJ ，Tan X ，Geng X ，Ding Y ... -  《-》

Two case reports and a systematic review of the literature on adult cerebral cortical encephalitis with anti-myelin oligodendrocyte glycoprotein antibody.

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) has gained recognition in recent years as an immune-mediated inflammatory demyelinating disease of the central nervous system. The clinical features and prognosis of MOGAD adult cerebral cortical encephalitis (adult CCE) have not been fully elucidated. This study aims to further characterize the clinical symptoms, magnetic resonance imaging (MRI) findings, and prognosis of CCE with anti-MOG antibody. We present two adult cases of CCE with anti-MOG antibody and summarize the clinical symptoms, magnetic resonance imaging (MRI) findings, and prognosis of this phenotype as per a completed systematic review of the literature. We found a total of 39 cases of MOGAD adult CCE (36% females; average age of onset of 29 years). Among them, 85% had seizure, 82% had headache, 64% had cortical symptoms, 64% had fever, 54% had changes of consciousness, and 38% had ocular symptoms. All cases demonstrated cerebral cortical T2 fluid-attenuated inversion recovery (FLAIR) lesions on MRI. Of the 25 patients (with seizure or not) who had EEG reports, 76% of patients showed abnormal EEG. Cerebrospinal fluid (CSF) white blood cell count of 90% of patients and CSF total protein of 67% of patients were elevated. In 16 patients with available CSF cytology data, 11 (69%) had abnormal cytology findings with monocytic predominance. In the 15 cases for which MOG antibody IgG was tested in both serum and CSF, 14 (93%) demonstrated a higher positive MOG IgG titer in serum than CSF. The majority of patients were treated with immunosuppressive therapy (97% corticosteroids, 15% mycophenolate mofetil, 13% IVIg, 5% azathioprine, and 5% other). The majority of patients had a favorable prognosis after treatment, as exemplified by improved clinical symptoms and imaging. Two patients relapsed. The clinical presentation and prognosis of adult CCE remain less understood in comparison to more common MOGAD phenotypes. It is important to consider MOGAD as an underlying etiology for adult CCE, as early detection and immunotherapy may improve outcomes.

Xu M ，Ma C ，Dong M ，Guo C ，Yang S ，Liu Y ，Wang X ... -  《Frontiers in Immunology》

Unilateral cerebral cortical encephalitis (CCE) with positive anti-MOG antibodies: A case report.

Nowadays, myelin oligodendrocyte glycoprotein (MOG)-antibody-associated disease (MOGAD) is regarded as an independent inflammatory demyelinating disease. Here, we report a rare case of unilateral cerebral cortical encephalitis (CCE) with positive anti-MOG antibodies. A 19-year-old woman was admitted to our hospital owing to acute onset fever and headache. Four days later, she experienced a focal seizure that progressed to generalized tonic-clonic seizures. Brain magnetic resonance imaging (MRI) demonstrated cortical lesions in the left cerebral hemisphere on T2-weighted fluid-attenuated inversion recovery imaging. The patient was positive for anti-MOG antibodies in serum and diagnosed with anti-MOG antibody-associated unilateral CCE. She was administrated with intravenous methylprednisolone followed by oral corticosteroids. On day 14 after admission, a repeat MRI revealed partial resolution of the initial abnormalities. The patient received a quick recovery without residual symptoms. Unilateral CCE with positive anti-MOG antibodies has emerged as a special clinical phenotype of MOGAD. It should be emphasized that the characteristic neuroradiological features of CCE would be an important clue to the correct diagnosis of MOGAD.

Nie H ，Gao H ，Li Y ，Shen Y ... -  《-》

Myelin oligodendrocyte glycoprotein antibody-associated disease presenting as unilateral cerebral cortical encephalitis: a case report.

An JY ，Jang SI ，Choi SG ，Lee SN ，Lee EJ ，Kim KK ，Kim HR ... -  《-》

Clinical characteristic of myelin oligodendrocyte glycoprotein antibody associated cortical encephalitis in adults and outcomes following glucocorticoid therapy.

To describe the clinical and radiological features, as well as outcomes following glucocorticoid therapy and recurrence in adults suffering from cortical encephalitis associated with myelin oligodendrocyte glycoprotein (MOG) antibody. The clinical information of nine adult patients suffering from cortical encephalitis associated with MOG antibody admitted to the Affiliated Brain Hospital of Nanjing Medical University from 2020 to 2022 was systematically reviewed. The clinical symptoms, laboratory data, imaging results, outcomes following glucocorticoid therapy and recurrence were evaluated. A total of 9 patients positive for MOG antibody and suffering from cortical encephalitis were included in our study (55.6% men, median age 29 years, 15-57 years). The most common clinical symptoms included headache (77.8%), fever (66.7%), and generalized seizures (55.6%). Some patients also experienced limb shaking (22.2%), leg numbness (22.2%), transient motor aphasia (11.1%), and vision loss (11.1%). The main features of cerebrospinal fluid () examination were increased intracranial pressure, pleocytosis, and elevated cerebrospinal fluid (CSF) protein. In addition, N-methyl-D-aspartate receptor (NMDAR) and MOG antibodies were found in the CSF of 3 patients, and NMDAR, MOG, and glial fibrillary acidic protein antibodies were found in the CSF of 1 patient. All patients were subjected to magnetic resonance imaging (MRI) and the images of eight of them showed T2 and/flair image hyperintense lesions, three showed meningeal or lesion enhancement and four showed white matter lesions, which were mostly located in the midline structures (75%). All patients received glucocorticoid therapy in the acute phase and in remission, and eight of them received an intravenous high dose of methylprednisolone, including one patient who received a simultaneous immunoglobulin therapy. One patient was treated with low-dose prednisolone tablets. Seven (77.8%) patients were wholly recovered at discharge, and 2 (22.2%) patients were left with slight symptoms. During the median 9-month follow-up (range: 2-36 months), 2 (22.2%) patients developed recurrence. The clinical manifestations of adult MOG antibody-associated cortical encephalitis were significantly different from those of the typical MOG antibody-associated disease (MOGAD). Patients in the acute phase of the disease were prone to show signs similar to central nervous system infection, requiring clinicians to have the ability to recognize the disease to avoid misdiagnosis. In addition, seizures were common in MOG antibody-related encephalitis, and the type of seizures was age-related. Brain MRI results showed that the distribution of cerebral cortex lesions was closely related to the classification of cortical encephalitis. Based on the patient's response to the treatment, glucocorticoid therapy was effective against MOG antibody-associated cortical encephalitis, which is consistent with the treatment response and clinical prognosis of MOGAD. Therefore, our opinion was that MOG antibody might be the "responsible antibody" in MOG antibody-associated cortical encephalitis, although further studies are needed to confirm this hypothesis.

Wu Y ，Zhou H ，Ci X ，Lin L ，Zhang D ，Lu J ... -  《-》