Clinical and Radiological Features of Anti-MOG Associated Cerebral Cortical Encephalitis
Hazal Ceren Manazoğlu1, İpek Güngör Doğan3, Serkan Demir3, Ahmet Kasım Kılıç4, Serkan Özakbaş5, Furkan Sarıdaş6, Rabia Koç6, Belgin Petek Balcı7, Esra Taşkıran8, İbrahim Acır9, Nermin Tepe10, Pınar Acar Özen11, Aslı Tuncer11, Ahmed Serkan Emekli2, Tuncay Gündüz2, Murat Kürtüncü2
1Neurology Clinic, Ümraniye Training and Research Hospital, University of Health Sciences, Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, 2Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, 3Neurology Clinic, Şehit Prof. Dr. İlhan Varank Sancaktepe Training and Research Hospital, University of Health Sciences, 4Neurology Clinic, Kartal Dr. Lütfi Kırdar City Hospital, University of Health Sciences, 5Department of Neurology, Faculty of Medicine, İzmir University of Economics, 6Department of Neurology, Faculty of Medicine, Bursa Uludağ University, 7Neurology Clinic, Haseki Training and Research Hospital, University of Health Sciences, 8Neurology Clinic, Antalya Training and Research Hospital, University of Health Sciences, 9Neurology Clinic, Bakırköy Dr. Sadi Konuk Training and Research Hospital, University of Health Sciences, 10Department of Neurology, Faculty of Medicine, Balıkesir University, 11Department of Neurology, Faculty of Medicine, Hacettepe University
Objective:
This study aims to describe the clinical and radiological features of anti-MOG associated CCE and provide guidance for treatment and follow-up.
Background:
Cerebral cortical encephalitis (CCE) is a recently identified phenotype of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
Design/Methods:
This multicenter observational study included patients with symptomatic cerebral cortical lesions meeting the 2023 MOGAD diagnostic criteria. Data on demographics, clinical and radiological findings, treatment responses, and prognosis were collected.
Results:
A total of 18 patients (10 males, 8 females) from 11 centers were included in the study, with a median age at onset of 22 years (IQR: 16,3). The median follow-up duration was three years (IQR: 7,5). At presentation, cortical findings were observed in four patients (22%), optic neuritis in seven (38%), encephalopathy in two (11%), brainstem involvement in 12 (56%), and spinal involvement in two (11%) patients. Acute treatments consisted of intravenous methylprednisolone (IVMP) in 11 patients (61%), plasma exchange in three (17%), and a combination of IVMP, plasma exchange, and IVIG in one patient (6%). Complete recovery was achieved in 13 patients (72%), partial recovery in four (22%), while one patient did not show improvement. During the follow-up period, 22% of the patients experienced a single attack, 56% had two attacks, and 22% had more than two attacks. Seizures occurred in 78% of patients, optic neuritis in 39%, and spinal attacks in 28%. Spinal lesions were identified in 33% of the patients, with 22% presenting cervicothoracic lesions, and 5.6% having either cervical or full spinal lesions. After six months of treatment, cortical lesions resolved in 14 patients (78%). The median final EDSS score was 2.0 (IQR: 3,5).
Conclusions:
Anti-MOG associated CCE cases are limited in the literature. Early recognition in young patients with seizures, headaches, and altered consciousness is crucial for proper management and differentiating CCE from other encephalitis forms.
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