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

Abstract

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.

Weston-Hurst syndrome: a rare fulminant form of acute disseminated encephalomyelitis (ADEM)

“…There is only one case in the literature as yet that denotes involvement of the spinal cord 9. On microscopy, it is characterised by fibrinoid venous necrosis, neutrophilic debris, haemorrhages and demyelination 7.…”

Basal ganglia necrosis: a ‘best-fit’ approach

“…Acute haemorrhagic leukoencephalitis, a severe form of acute disseminated encephalomyelitis, is a disease process that has been linked with Epstein-Barr virus,5 however, there are usually microhaemorrhages within white matter lesions on MRI6 and our patient did not have these. CSF findings are also inconsistent, typically showing raised white and red blood cell counts 2.…”

Fatal Central Nervous System Disease Following First Infliximab Infusion in a Child With Inflammatory Bowel Disease

“…AHLE is a rare catastrophic CNS demyelinating disease of unclear origin that most commonly follows a respiratory infection; it has been considered to reside on a spectrum with acute disseminated encephalomyelitis, which also has an abrupt onset. 17 AHLE rarely occurs in patients with IBD, 18 and it has not been attributed to anti-TNF-a therapy. Acute disseminated encephalomyelitis, however, has been reported in pediatric patients with IBD.…”