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Acute hemorrhagic leukoencephalitis

Case contributed by Andrew Dixon
Diagnosis almost certain

Presentation

2 week history of lower respiratory tract infection. 3 days confusion and visual changes. Now left hemiplegia.

Patient Data

Age: 30
Gender: Male

Day 1

ct

Initial CT Brain demonstrates diffuse cerebral white matter hypoattenuation including involvement of the corpus callosum.  There is associated generalized cerebral sulcal effacement. Brain biopsy was performed the following day showing acute hemorrhagic leukoencephalitis (see path report below).

Day 5

ct

CT Brain 4 days later shows interval progression of the white matter hypoattenuation and the development of basal cistern effacement (descending transtentorial herniation) from raised intracranial pressure.

Day 9

ct

Urgent decompressive bilateral fronto-temporal craniectomies were performed. CT Brain 9 days following the initial presentation shows established bilateral posterior cerebral artery territory infarcts as a result of the previous descending transtentorial herniation.   

Case Discussion

MICROSCOPIC DESCRIPTION: Sections show white matter, within which there is patchy areas within which here appears to be myelin loss in association with a moderately florid neutrophilic infiltrate which in areas is perivascular centric in association with red cell extravasation and scattered reactive changes in oligodendrocytes. No viral inclusions are identified. There is no significant lymphocytic infiltrate.

DIAGNOSIS: Deep white matter - features in keeping with acute hemorrhagic leucoencephalitis within the early stage of the spectrum of acute demyelinating encephalomyelitis (ADEM).

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