Acute hemorrhagic leukoencephalitis with severe brainstem and spinal cord involvement: MRI features with neuropathological confirmation

Acute hemorrhagic leukoencephalitis (AHLE) is a rare and fulminant demyelinating disease considered to be the most severe form of acute disseminated encephalomyelitis (ADEM). A 70‐year‐old man was admitted to our emergency department (ED) after 1 week of unspecific abdominal symptoms and moderate fe...

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Bibliographic Details
Published in:Journal of magnetic resonance imaging 2011-04, Vol.33 (4), p.957-961
Main Authors: Pinto, Pedro S., Taipa, Ricardo, Moreira, Bruno, Correia, Carlos, Melo-Pires, Manuel
Format: Article
Language:English
Subjects:
acute hemorrhagic leukoencephalitis
Aged
Biopsy
Brain - pathology
Brain Stem - pathology
brainstem
Fatal Outcome
Humans
Leukoencephalitis, Acute Hemorrhagic - diagnosis
Leukoencephalitis, Acute Hemorrhagic - pathology
magnetic resonance imaging
Magnetic Resonance Imaging - methods
Male
pathological confirmation
Spinal Cord - pathology
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Summary:Acute hemorrhagic leukoencephalitis (AHLE) is a rare and fulminant demyelinating disease considered to be the most severe form of acute disseminated encephalomyelitis (ADEM). A 70‐year‐old man was admitted to our emergency department (ED) after 1 week of unspecific abdominal symptoms and moderate fever in the first 3 days. Within the ED he developed a rapid onset coma and flaccid tetraparesis. Cerebrospinal fluid (CSF) analysis showed mild polymorphonuclear pleocytosis and magnetic resonance imaging (MRI) revealed supratentorial focal white matter lesions and diffuse involvement of the medulla and spinal cord. A presumptive diagnosis of ADEM was made and the patient was treated with corticosteroids followed by intravenous immunoglobulin. His neurological state did not improve and the MRI on day 8 after admission showed an increased number of lesions, mainly in the brainstem, with hemorrhagic foci. The patient died the following day and pathological features confirmed the diagnosis of AHLE. This is a unique presentation of a rare disease with detailed MRI characteristics and pathological confirmation. Although this condition is usually fatal, early recognition and aggressive therapeutic management can facilitate survival. J. Magn. Reson. Imaging 2011;33:957–961. © 2011 Wiley‐Liss, Inc.
ISSN:1053-1807
1522-2586
DOI:10.1002/jmri.22505