The pathological features of MOG antibody-positive cerebral cortical encephalitis as a new spectrum associated with MOG antibodies: A case report

Ikeda, Toshimasa; Yamada, Kentaro; Ogawa, Ryo; Tokura, Y.; Kaneko, Kimihiko; Misu, Tatsuro; Kamimoto, Kaoru; Matsukawa, Noriyuki; Yoshida, Mari

doi:10.1016/j.jns.2018.06.028

Cited by 63 publications

(74 citation statements)

References 10 publications

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“…Ikeda et al described a similar case of a 29-year-old female presenting with generalized seizures, unilateral cerebral cortical lesions, and a bilateral optic neuritis, who underwent a brain biopsy before steroid treatment. This revealed only mild inflammatory changes in the cortex and subcortex without distinct demyelination (8) and did not fulfill the previously described MOG-Abs-associated histopathological features (9)(10)(11)(12)(13). The authors ascribe this to the most likely very early stage of demyelination, when the biopsy was taken.…”

Section: Discussioncontrasting

confidence: 53%

A Fulminant Case of Demyelinating Encephalitis With Extensive Cortical Involvement Associated With Anti-MOG Antibodies

et al. 2020

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Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies (MOG-Abs) are commonly associated with clinical presentations as acute disseminated encephalomyelitis (ADEM) in both adults and children and anti-aquaporin 4 antibody-seronegative neuromyelitis optica spectrum disorder (NMOSD) and related syndromes such as optic neuritis, myelitis, and brainstem encephalitis. Most often, the presence of MOG-Abs is associated with a more benign clinical course and a good response to steroids. Here, we present a case report of a previously healthy 52-year-old female patient with fulminant demyelinating encephalitis, leading to death within a week after the first presenting symptoms from a massive brain edema irresponsive to high-dose intravenous steroids as well as osmotic therapy. The final diagnosis was only made postmortem after serum anti-MOG-Abs results were available. Histopathological analysis of the brain revealed extensive, predominantly cortical demyelinating lesions in the frontal, temporal, and parietal lobes with intracortical, leukocortical, and subpial plaques, associated with pronounced perivenous deposition of activated complement complex as well as features of acute MS characterized by destructive lesions.A 52-year-old woman, a cashier at a supermarket, presented in our outpatient department with difficulties in speaking. Furthermore, she complained of headache in the last 2 weeks. At neurological examination, she showed features of an encephalitic syndrome (disorientation, agitation, poor memory functioning) and continuously perseverated the same sentences. Gaze was preferable to the right, but there were no motor signs on admittance. Meningeal signs were positive. Past medical history was remarkable for hypothyreodism, a previous gastric-banding surgery in 2011, and depression. She was on a permanent antidepressive medication (Sertralin/Trazodon) as well as thyroid hormone replacement. Allergies were documented against Diclofenac and Penicillin.An initially performed CT of the brain (CCT) revealed a newly formed hypodensity in the left occipital region, which was not present on an archival CCT scan from the year before. A subsequently performed MRI of the brain furthermore revealed multiple, contrast-enhancing

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Section: Discussioncontrasting

confidence: 53%

A Fulminant Case of Demyelinating Encephalitis With Extensive Cortical Involvement Associated With Anti-MOG Antibodies

et al. 2020

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“…However, in contrast to MS, intracortical rather than leukocortical demyelinated lesions predominate. Although two prior MOGAD biopsied cases reported cortical encephalitis without demyelination, this may have potentially been due to a very early stage of the disease process or an effect of treatment prior to biopsy [21,26].…”

Section: Discussionmentioning

confidence: 98%

The pathology of central nervous system inflammatory demyelinating disease accompanying myelin oligodendrocyte glycoprotein autoantibody

et al. 2020

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We sought to define the pathological features of myelin oligodendrocyte glycoprotein (MOG) antibody associated disorders (MOGAD) in an archival autopsy/biopsy cohort. We histopathologically analyzed 2 autopsies and 22 brain biopsies from patients with CNS inflammatory demyelinating diseases seropositive for MOG-antibody by live-cell-based-assay with full length MOG in its conformational form. MOGAD autopsies (ages 52 and 67) demonstrate the full spectrum of histopathological features observed within the 22 brain biopsies (median age, 10 years; range, 1-66; 56% female). Clinical, radiologic, and laboratory characteristics and course (78% relapsing) are consistent with MOGAD. MOGAD pathology is dominated by coexistence of both perivenous and confluent white matter demyelination, with an over-representation of intracortical demyelinated lesions compared to typical MS. Radially expanding confluent slowly expanding smoldering lesions in the white matter as seen in MS, are not present. A CD4+ T-cell dominated inflammatory reaction with granulocytic infiltration predominates. Complement deposition is present in all active white matter lesions, but a preferential loss of MOG is not observed. AQP4 is preserved, with absence of dystrophic astrocytes, and variable oligodendrocyte and axonal destruction. MOGAD is pathologically distinguished from AQP4-IgG seropositive NMOSD, but shares some overlapping features with both MS and ADEM, suggesting a transitional pathology. Complement deposition in the absence of selective MOG protein loss suggest humoral mechanisms are involved, however argue against endocytic internalization of the MOG antigen. Parallels with MOG-EAE suggest MOG may be an amplification factor that augments CNS demyelination, possibly via complement mediated destruction of myelin or ADCC phagocytosis.

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“…Wang et al 106 reported that 21% of MOG-IgG-positive patients presented with encephalitis, and one-third of these patients also showed anti-N-methyl d-aspartase receptor antibody. 105,107,108 Optic nerve lesions MRI abnormalities of the optic nerve have been found in 44-83% of patients. 105,107,108 Optic nerve lesions MRI abnormalities of the optic nerve have been found in 44-83% of patients.…”

Section: Brain Lesionsmentioning

confidence: 99%

“…MOG antibody-positive cortical encephalitis might have other distinct mechanisms beyond simple MOG-related immunopathology. 105,107,108 Optic nerve lesions MRI abnormalities of the optic nerve have been found in 44-83% of patients. 102,109,111 Bilateral, longitudinally extensive, symmetrical optic neuritis in the intraorbital segments is characteristic.…”

Section: Brain Lesionsmentioning

confidence: 99%

Magnetic resonance imaging diagnosis of demyelinating diseases: An update

Miki

2019

Clinical & Exp Neuroim

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Keywords acute disseminating encephalomyelitis (ADEM); demyelinating diseases; multiple sclerosis (MS); myelin oligodendrocyte glycoprotein (MOG) encephalomyelitis; neuromyelitis optica spectrum disorder (NMOSD) Correspondence AbstractMajor demyelinating diseases include multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), acute disseminated encephalomyelitis (ADEM) and myelin oligodendrocyte glycoprotein (MOG) encephalomyelitis. As treatment strategies differ among these diseases, precise diagnosis of demyelinating diseases is crucial, and magnetic resonance imaging (MRI) plays a pivotal role in the diagnosis. In the present review, MRI appearances of characteristic lesions of these demyelinating diseases, and differentiation between MS and other demyelinating diseases based on MRI findings are discussed.

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The pathological features of MOG antibody-positive cerebral cortical encephalitis as a new spectrum associated with MOG antibodies: A case report

Cited by 63 publications

References 10 publications

A Fulminant Case of Demyelinating Encephalitis With Extensive Cortical Involvement Associated With Anti-MOG Antibodies

A Fulminant Case of Demyelinating Encephalitis With Extensive Cortical Involvement Associated With Anti-MOG Antibodies

The pathology of central nervous system inflammatory demyelinating disease accompanying myelin oligodendrocyte glycoprotein autoantibody

Magnetic resonance imaging diagnosis of demyelinating diseases: An update

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