Tubular Inclusions in Macrophages in the Brain of a Patient with Acute Hemorrhagic Leukoencephalitis (Weston-Hurst Syndrome)

1994 ◽  
Vol 18 (1-2) ◽  
pp. 19-22 ◽  
Author(s):  
Concettina G. Gillies ◽  
Margaret Grunnet ◽  
Cornelia W. Hamilton
2017 ◽  
Vol 7 (3) ◽  
pp. 487-493 ◽  
Author(s):  
Raheem Peerani ◽  
Meredith Berggren ◽  
Jayantha C. Herath

We report a case of acute hemorrhagic leukoencephalitis in an adult man with a prodrome of “feeling unwell” two days prior to this death. At autopsy, external examination revealed minor external injuries including contusions on the scalp and left thigh and abrasions on the forehead and right eyebrow. Gross examination of the brain after coronal sectioning revealed multiple petechial hemorrhages in the white matter in the cerebral hemispheres, corpus callosum, basal ganglia, brainstem, and cerebellum. Microscopic examination of these lesions revealed demyelination, hemorrhage, and necrosis with fibrin exudation in a perivenular distribution with radial extension into the white matter. The remainder of the autopsy was unremarkable. This case highlights the death of a young man by a rare fatal complication of a natural disease only identified by a singular set of gross and microscopic findings at autopsy in circumstances that would otherwise suggest a nonnatural death. The case demonstrates the importance of a thorough autopsy in settings where the clinical history, scene, and circumstances may be misleading or absent.


2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S38-S38
Author(s):  
H Tomac Pavosevic ◽  
S Zaheer ◽  
G M Kleinman

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.


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