Case report: The histopathological analyses of two myelin oligodendrocyte glycoprotein antibody-associated diseases with a distinctive linear radiating gadolinium enhancement on MRI

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) has highly heterogeneous clinical presentations, in which encephalitis is an important phenotype. Moreover, MOGAD has been reported to exhibit diverse imaging findings. However, there have been no previous reports of cases with perivascular radial gadolinium enhancement in periventricular regions, commonly reported in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. In this paper, we present two cases of MOGAD with this MRI feature, both of which underwent brain biopsy for the lesions. Brain biopsies revealed perivenous demyelination and inflammation consistent with acute disseminated encephalomyelitis (ADEM), with pronounced axonal damage in Case 1 and minimal axonal involvement in Case 2. Case 1 exhibited more severe cerebral atrophy than Case 2, correlating with the extent of axonal damage. Through these cases, we highlight the heterogeneity of radiological manifestations of MOGAD, expanding the spectrum beyond previously defined MRI patterns. Furthermore, histopathological analysis revealed distinct axonal involvement as a potential prognostic marker of brain atrophy. These observations emphasize the importance of considering MOGAD in the differential diagnosis, even in cases with atypical imaging findings, and highlight the significance of brain biopsy in guiding both diagnosis and prognosis.

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Publication Details

Journal
Frontiers in Immunology
Published
2024-08-29
DOI
https://doi.org/10.3389/fimmu.2024.1426236
Primary Topic
Multiple Sclerosis Research Studies
Type
article
Field-Weighted Citation Impact
0.00

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article

Case report: The histopathological analyses of two myelin oligodendrocyte glycoprotein antibody-associated diseases with a distinctive linear radiating gadolinium enhancement on MRI

Goichi Beck, Tatsusada Okuno, Shigeo Murayama, Taku Hoshi et al.
Frontiers in Immunology
Multiple Sclerosis Research Studies
article

Case report: The histopathological analyses of two myelin oligodendrocyte glycoprotein antibody-associated diseases with a distinctive linear radiating gadolinium enhancement on MRI

Goichi Beck, Tatsusada Okuno, Shigeo Murayama, Taku Hoshi, Yuki Shimada, Hideki Mochizuki, Nozomi Nagashima, Mikito Shimizu, Manabu Sakaguchi, Naoki Hatayama, Isao Fukasaka, Hiroyuki Sumikura, Kyoko Higashida, Tomohiro Fujioka
article en

Abstract

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) has highly heterogeneous clinical presentations, in which encephalitis is an important phenotype. Moreover, MOGAD has been reported to exhibit diverse imaging findings. However, there have been no previous reports of cases with perivascular radial gadolinium enhancement in periventricular regions, commonly reported in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. In this paper, we present two cases of MOGAD with this MRI feature, both of which underwent brain biopsy for the lesions. Brain biopsies revealed perivenous demyelination and inflammation consistent with acute disseminated encephalomyelitis (ADEM), with pronounced axonal damage in Case 1 and minimal axonal involvement in Case 2. Case 1 exhibited more severe cerebral atrophy than Case 2, correlating with the extent of axonal damage. Through these cases, we highlight the heterogeneity of radiological manifestations of MOGAD, expanding the spectrum beyond previously defined MRI patterns. Furthermore, histopathological analysis revealed distinct axonal involvement as a potential prognostic marker of brain atrophy. These observations emphasize the importance of considering MOGAD in the differential diagnosis, even in cases with atypical imaging findings, and highlight the significance of brain biopsy in guiding both diagnosis and prognosis.

Frontiers in ImmunologyVol. 15
Osaka Prefectural Medical Center (JP), Osaka City General Hospital (JP), Tokyo Metropolitan Geriatric Hospital (JP), The University of Osaka (JP)
Japan Agency for Medical Research and Development, Japan Society for the Promotion of Science
Good health and well-being
Openalex Percentile: Top 77%
Multiple Sclerosis Research Studies
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