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The pathology of central nervous system inflammatory demyelinating disease accompanying myelin oligodendrocyte glycoprotein autoantibody
- Source :
- Acta Neuropathologica
- Publication Year :
- 2020
- Publisher :
- Springer Berlin Heidelberg, 2020.
-
Abstract
- 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.
- Subjects :
- 0301 basic medicine
Pathology
medicine.medical_specialty
CNS demyelination
Biopsy
Pathology and Forensic Medicine
Myelin oligodendrocyte glycoprotein
White matter
Multiple sclerosis
03 medical and health sciences
Cellular and Molecular Neuroscience
Myelin
0302 clinical medicine
immune system diseases
Central Nervous System Diseases
Acute disseminated encephalomyelitis
medicine
MOG
Humans
Aged
Autoantibodies
Original Paper
biology
business.industry
Neuromyelitis Optica
Middle Aged
medicine.disease
White Matter
Hyperintensity
Oligodendrocyte
Oligodendroglia
030104 developmental biology
medicine.anatomical_structure
Astrocytes
Immunoglobulin G
biology.protein
Female
Myelin-Oligodendrocyte Glycoprotein
Neurology (clinical)
Autopsy
Demyelination
business
030217 neurology & neurosurgery
Subjects
Details
- Language :
- English
- ISSN :
- 14320533 and 00016322
- Volume :
- 139
- Issue :
- 5
- Database :
- OpenAIRE
- Journal :
- Acta Neuropathologica
- Accession number :
- edsair.doi.dedup.....88f6e714f1e08a5137e0986aad01331d