Pediatric Acute Hemorrhagic Leukoencephalitis (Weston-Hurst syndrome): A Rare Entity

Abstract

Abstract Introduction/Objective Acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome) is a rare, fulminant inflammatory hemorrhagic demyelination of the brain white matter, and it is considered to be the most severe form of acute disseminated encephalomyelitis (ADEM). Herein we describe a case of 6-year-old boy presenting with elevated intracranial pressure and uncal herniation. Brain MRI showed a large right temporoparietal T2 hyperintense lesion with edema, abnormal perivascular enhancement and midline shift. Emergent decompressive craniotomy with brain biopsy was performed. Results H&E sections of the brain lesion revealed neutrophilic small vessel vasculitis with extravasation of blood and fibrin. Predominantly in a perivascular distribution were infiltrates of neutrophils and in areas foamy macrophages. Additionally, the white matter was studded with hemorrhages of varying size. Sections stained with Luxol Fast Blue for myelin and with a neurofilament immunostain for axons were compared to reveal selective perivascular loss of myelin with relative sparing of axons. Conclusion The constellation of acute hemorrhages, neutrophilic infiltration and selective loss of myelin with relative sparing of axons is characteristic of this exceptional, hyperacute variant of acute disseminated encephalomyelitis. Histomorphologic diagnosis is characteristic and may be crucial for timely decision for treatment of this often fatal disease.