Early and widespread injury of astrocytes in the absence of demyelination in acute haemorrhagic leukoencephalitis

Christopher A. Robinson, Reginald C. Adiele, Mylyne Tham, Claudia F Lucchinetti, Bogdan F G H Popescu

Research output: Contribution to journalArticle

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Abstract

Acute hemorrhagic leukoencephalitis (AHL) is a fulminant demyelinating disease of unknown etiology. Most cases are fatal within one week from onset. AHL pathology varies with the acuteness of disease. Hemorrhages, vessel fibrinoid necrosis, perivascular fibrin exudation, edema and neutrophilic inflammation are early features, while perivascular demyelination, microglial foci and myelin-laden macrophages appear later. Reactive astrocytosis is not present in early hemorrhagic non-demyelinated lesions, but is seen in older lesions. This case report presents the pathology of an AHL case with fulminant course and fatal outcome within 48 hours from presentation. Severe hemorrhages, edema and neutrophilic inflammation in the absence of circumscribed perivascular demyelination affected the temporal neocortex and white matter, hippocampus, cerebellar cortex and white matter, optic chiasm, mammillary bodies, brainstem, cranial nerve roots and leptomeninges. Perivascular end-feet and parenchymal processes of astrocytes exhibited impressive swelling in haemorrhagic but non-demyelinated white matter regions. Astrocytes were dystrophic and displayed degenerating processes. Astrocytic swellings and remnants were immunoreactive for aquaporin-4, aquaporin-1 and glial fibrillary acidic protein. These morphological changes of astrocytes consistent with injury were also observed in haemorrhagic and normal appearing cortex. Our findings reinforce that perivascular demyelination is not present early in AHL. This is the first study that highlights the early and widespread astrocytic injury in the absence of demyelination in AHL, suggesting that, similarly to neuromyelitis optica and central pontine myelinolysis, demyelination in AHL is secondary to astrocyte injury.

Original languageEnglish (US)
Pages (from-to)52
Number of pages1
JournalActa neuropathologica communications
Volume2
DOIs
StatePublished - 2014

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Acute Hemorrhagic Leukoencephalitis
Demyelinating Diseases
Astrocytes
Wounds and Injuries
Edema
Central Pontine Myelinolysis
Mammillary Bodies
Aquaporin 1
Aquaporin 4
Neuromyelitis Optica
Pathology
Hemorrhage
Inflammation
Optic Chiasm
Cerebellar Cortex
Gliosis
Fatal Outcome
Cranial Nerves
Glial Fibrillary Acidic Protein
Neocortex

ASJC Scopus subject areas

  • Medicine(all)

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Early and widespread injury of astrocytes in the absence of demyelination in acute haemorrhagic leukoencephalitis. / Robinson, Christopher A.; Adiele, Reginald C.; Tham, Mylyne; Lucchinetti, Claudia F; Popescu, Bogdan F G H.

In: Acta neuropathologica communications, Vol. 2, 2014, p. 52.

Research output: Contribution to journalArticle

Robinson, Christopher A. ; Adiele, Reginald C. ; Tham, Mylyne ; Lucchinetti, Claudia F ; Popescu, Bogdan F G H. / Early and widespread injury of astrocytes in the absence of demyelination in acute haemorrhagic leukoencephalitis. In: Acta neuropathologica communications. 2014 ; Vol. 2. pp. 52.
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