Clinical and Imaging Features of Patients With Encephalitic Symptoms and Myelin Oligodendrocyte Glycoprotein Antibodies

Abstract

BackgroundMyelin oligodendrocyte glycoprotein-antibody (MOG-ab)-associated disease (MOGAD) has highly heterogenous clinical and imaging presentations, in which encephalitis is an important phenotype. In recent years, some atypical presentations in MOG-ab-associated encephalitis (MOG-E) have been increasingly reported but have not yet been described well. The aim of the study was to describe the clinical and imaging features of patients with MOG-E in our center. Atypical phenotypes would be reported, which is expected to expand the spectrum of MOGAD.MethodsWe reviewed medical records of 59 patients with MOGAD diagnosed in our center and identified cases who had ever experienced encephalitic symptoms. Three hundred ten patients with autoimmune encephalitis (AE) were also reviewed, and cases with positive MOG-ab were identified. Besides, patients with chronically progressive encephalitis were identified from 13 MOG-E and 310 AE patients. We collected demographic, clinical, laboratory, radiological, and outcome data to explore clinical and imaging characteristics in MOG-E, especially in the atypical phenotype of chronically progressive encephalitis.ResultsWe identified 13 patients (7 males, 6 females) with MOG-E. The median age at onset was 33 years (range 13~62 years). Most (9/13, 69.2%) of patients showed acute or subacute onset of encephalitic symptoms. Brain MRI abnormalities were observed in all patients. The most common lesion locations on MRI were cortical/subcortical (11/13, 84.6%), deep/periventricular white matter (10/13, 76.9%) and corpus callosum (4/13, 30.8%). Brain MRI patterns were categorized into four phenotypes. The most common pattern was cortical encephalitis with leptomeningeal enhancement/brain atrophy (10/13, 76.9%). Eight (8/13, 61.5%) patients had a good response to immunotherapy. Four (4/13, 30.8%) patients with chronically progressive course were identified from MOG-E cohort. They showed leukodystrophy-like pattern, multifocal hazy lesions, or cortical encephalitis on MRI. With immunotherapy, they only showed mild or no improvement. We also identified four (4/310, 1.3%) patients with chronically progressive course from AE cohort. They had better outcomes than counterparts in MOG-E.ConclusionsThis study demonstrates that encephalitic presentations in MOGAD had complex clinical patterns. Chronically progressive encephalitis may be a new phenotype of MOGAD. We recommend to test MOG-ab in subacute and chronic progressive dementia with leukodystrophy-like MRI lesions.

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Review of Clinical and MRI Features, Diagnosis, and Management

“…Available data on the long-term clinical outcomes in MOGAD patients are in keeping with the above-mentioned MRI findings, showing complete or nearly complete recovery from attacks in most cases (82,84,100). However, residual cognitive deficits may occur even in children, and a minority of MOGAD patients have an unfavorable functional outcome (101)(102)(103)(104). In one series of 29 patients followed for a median of 14 (range, 9-31) years, the median EDSS at last clinical follow-up was 2, with only two cases (7%) having an EDSS of ≥6 (100, 101).…”

Corpus callosum involvement in MOG antibody-associated disease in comparison to AQP4-IgG-seropositive neuromyelitis optica spectrum disorder and multiple sclerosis

“…Callosal involvement occurred in 22% of MOGAD scans, which was similar to prior reports of 18%-33%. [3][4][5] Most had cerebral symptoms and few had isolated optic neuritis or myelitis. Unlike in MS and AQP4+NMOSD, 6,7 MOGAD callosal lesion characterization and comparison has not been reported.…”

Clinical features of the first attack with leukodystrophy‐like phenotype in children with myelin oligodendrocyte glycoprotein antibody‐associated disorders

“…Moreover, according to a previous study, adult leukodystrophy‐like MOGAD patients had subacute onset and were chronically progressive without acute attacks. Adult patients appeared to have a worse outcome than children patients, as effects of immunotherapy in adult on score was only mild or even deteriorated (Wang et al, 2021). Furthermore, MRI findings of predominantly confluent, bilateral white matter changes at onset showed dramatic resolution on follow‐up scans after acute treatment, as previously reported (Hacohen, Rossor, et al, 2018; Marignier et al, 2021; Zhou et al, 2019).…”