Premium
Acute hemorrhagic leukoencephalitis with severe brainstem and spinal cord involvement: MRI features with neuropathological confirmation
Author(s) -
Pinto Pedro S.,
Taipa Ricardo,
Moreira Bruno,
Correia Carlos,
MeloPires Manuel
Publication year - 2011
Publication title -
journal of magnetic resonance imaging
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.563
H-Index - 160
eISSN - 1522-2586
pISSN - 1053-1807
DOI - 10.1002/jmri.22505
Subject(s) - medicine , acute disseminated encephalomyelitis , pathology , pleocytosis , cerebrospinal fluid , magnetic resonance imaging , spinal cord , myelitis , coma (optics) , leukoencephalopathy , pathological , radiology , disease , physics , optics , psychiatry
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.