Hemicerebral atrophy as an unusual central nervous system manifestation of Neurofibromatosis type 1: A case report

Abstract

Neurofibromatosis type 1 is a widely known phakomatosis with highly variable manifestations. However, its presentation with hemicerebral atrophy resulting from an intracranial vasculopathy is uncommon, yet it poses a major health threat. We hereby report the case of a 6-year-old boy with the clinical features of Neurofibromatosis type 1 and imaging evidence of right internal carotid artery narrowing, with subsequent ipsilateral hemicerebral atrophy, after presenting with progressive left-sided body weakness and epilepsy. This case represents a rare manifestation within the vast imaging spectrum of Neurofibromatosis type 1, highlighting how unpredictable the condition can be.