Teenager With Recurrent Ataxia, Ophthalmoplegia, and Encephalopathy Associated With Demyelination: From the National Multiple Sclerosis Society Case Conference Proceedings

Abstract

A 15-year-old adolescent boy developed subacute ataxia, encephalopathy, ophthalmoplegia, and dysarthria following a sore throat. An MRI examination revealed multifocal enhancing and nonenhancing supratentorial white matter and symmetric brainstem lesions. After 2 additional presentations with worsening symptoms and lesion accumulation, he was ultimately successfully treated with rituximab for his condition.

Figure 1. Brain MRI at First Presentation

Axial T2/FLAIR demonstrating multifocal hyperintensities in the periventricular, subcortical, infratentorial, and basal ganglia regions (A-C). Nonenhancing and multiple punctate and ring-enhancing (arrow) lesions noted (D). Extent of symmetric midbrain lesions extending into cerebral peduncles shown in coronal T2 (E). Few T1 hypointensities in subcortical (arrow), juxtacortical, and periventricular regions were present (F).

Figure 2. Subsequent Brain MRIs

Six weeks from initial presentation, axial T2/FLAIR demonstrates increasing confluence of brainstem involvement, with new basal ganglia and periventricular lesions (A.a–A.c). New enhancing lesions are noted in the left internal capsule and left centrum semiovale (arrow) with open-ring configuration (A.d). Three weeks later at next admission, T2/FLAIR shows further progression in extent of brainstem, basal ganglia, and deep white matter hyperintensities (B.a–B.c) with 3 new rim-enhancing lesions in the deep white matter (arrows, B.d). Six months later after rituximab initiation, axial T2/FLAIR MRI shows resolution of all enhancement and decreasing extent of hyperintensities throughout (C.a–C.c). Coronal T1 imaging highlights T1 hypointensities (arrows at juxtacortical and left cerebral peduncle hypointensities, C.d).