Small Vessel Vasculitis In Biopsies Of Anti-mog Encephalitis

Abstract 3 – 0930-0945
Category: Clinical
At the end of the session, participants will be able to:
  • 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
Presentor
Dr. Bradley Chaharyn is a PGY4 Neuropathology resident at the University of British Columbia in Vancouver, BC, Canada. He received his MD in 2017 from the University of Saskatchewan where he also completed his undergraduate studies in Anatomy and Cell Biology.
Authors
Bradley M. Chaharyn1 and Christopher P. Dunham1 1Division of Neuropathology, University of British Columbia, Vancouver, BC.
Target Audience: Pathologists, Residents
CanMEDS: Medical Expert (the integrating role), Scholar
Description 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.
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