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Small Vessel Vasculitis in Biopsies Of Anti-mog Encephalitis

Published online by Cambridge University Press:  29 July 2021

Bradley M. Chaharyn
Affiliation:
Division of Neuropathology, University of British Columbia, Vancouver, BC
Christopher P. Dunham
Affiliation:
Division of Neuropathology, University of British Columbia, Vancouver, BC
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Abstract

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We report the neuropathology of two pediatric brain biopsy cases associated with serum anti-myelin oligodendrocyte glycoprotein (MOG) positivity. Descriptions of anti-MOG associated neuropathology are limited, with initial reports describing various patterns of inflammatory demyelination. Our first patient presented with confusion, speech abnormalities and personality changes following a treated strep throat infection. Our second patient had a past medical history of neurofibromatosis type 1 (NF1) and presented with hypersomnolence and focal neurological deficits. MRI abnormalities included diffuse scattered T2 FLAIR hyperintensities +/- enhancement. CSF was positive for anti-MOG antibodies in both cases, while one case exhibited additional anti-NMDA-R antibodies. Brain biopsies revealed vasocentric mononuclear inflammation featuring a predominance of lymphocytes that included intramural forms, as well as diffuse microglial activation, but no evidence of microglial nodules or microorganisms. One case demonstrated mild perivascular demyelination. The prevailing pattern in both cases was suggestive of “small vessel childhood primary angiitis of the central nervous system” (SVcPACNS). Our results parallel recent reports of anti-MOG neuropathology describing small vessel vasculitis, contrary to initial and subsequent reports that describe “encephalitis”. The foregoing suggests that the neuropathology associated with serum anti-MOG positivity may be broader than first appreciated. Moreover, this pattern of vasculitis might have implications for the natural history of this nascent disorder.

Learning Objectives

  • Define anti-MOG encephalitis.

  • Recognize the pathologic spectrum of reported cases of anti-MOG encephalitis.

  • Contrast the pathologic features of pediatric and adult CNS vasculitis.

  • Describe the histologic overlap of vasculitis and encephalitis.

Type
Abstracts
Copyright
© The Canadian Journal of Neurological Sciences Inc. 2021