Abstract
Isolated brain stem lesions presenting with acute neurologic findings create a major diagnostic dilemma in children. Although the brain stem is frequently involved in ADEM, solitary brain stem lesions are unusual. We performed a retrospective review in 6 children who presented with an inflammatory lesion confined to the brain stem. Two children were diagnosed with connective tissue disorder, CNS lupus, and localized scleroderma. The etiology could not be determined in 1, and clinical features suggested monophasic demyelination in 3. In these 3 children, initial lesions demonstrated vasogenic edema; all showed dramatic response to high-dose corticosteroids and made a full clinical recovery. Follow-up MRI showed complete resolution of lesions, and none had relapses at >2 years of follow-up. In retrospect, these cases are best regarded as a localized form of ADEM. We conclude that though ADEM is typically a disseminated disease with multifocal lesions, it rarely presents with monofocal demyelination confined to the brain stem.
Highlights
We report the clinical and radiologic findings of children presenting with acute brain stem inflammation and discuss the possibility of ADEM in the differential diagnosis
A challenging case of a child presenting with a large solitary brain stem lesion with subsequent diagnosis of multiple sclerosis has been reported.[15]
Diffusion characteristics were analyzed in children with ADEM diagnosed by IPMSSG criteria, and the study demonstrated that ADC is increased in ADEM lesions, whereas isotropic diffusion maps appear to have normal findings, consistent with vasogenic edema in most patients.[18]
Summary
We report the clinical and radiologic findings of children presenting with acute brain stem inflammation and discuss the possibility of ADEM in the differential diagnosis. Among the 112 patients presenting with acute CNS inflammation between January 2003 and December 2011, six children were identified with isolated brain stem syndrome, both clinically and radiologically.
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