Expanding the MOG phenotype: Brainstem encephalitis with punctate and curvilinear enhancement

Myelin oligodendrocyte glycoprotein antibody (MOG-Ab)-associated demyelination has been associated with a range of clinical phenotypes, including acute disseminated encephalomyelitis (ADEM), ADEM followed by recurrent optic neuritis, aquaporin 4–negative neuromyelitis optica spectrum disorder, and less commonly, brainstem encephalitis.1 In contrast, chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a clinical, radiologic, and pathologic diagnosis without an associated antibody.2,3 There have been few reported cases of pediatric CLIPPERS,4 and most have not clearly met the strict case definition of CLIPPERS as proposed in 2017.3 Here, we present a pediatric case, ultimately diagnosed as MOG-Ab–associated demyelination, that was initially concerning for CLIPPERS due to subacute brainstem encephalitis with MRI showing curvilinear and punctate contrast enhancement in the pons.

Source: Neurology Neuroimmunology and Neuroinflammation - September 12, 2019 Category: Neurology Authors: Matesanz, S., Kotch, C., Perrone, C., Waanders, A. J., Hill, B., Narula, S. Tags: Encephalitis, All Pediatric, All Demyelinating disease (CNS) Clinical/Scientific Notes Source Type: research