Acute Hemorrhagic Leukoencephalopathy: Pathological Features and Cerebrospinal Fluid Cytokine Profiles

Michaela Waak; Stephen Malone; Kate Sinclair; Gael Phillips; Sushil Bandodkar; Louise Wienholt; Thomas Robertson; Ben Whitehead; Peter Trnka; Kavitha Kothur; Russel C Dale

doi:10.1016/j.pediatrneurol.2019.06.013

Acute Hemorrhagic Leukoencephalopathy: Pathological Features and Cerebrospinal Fluid Cytokine Profiles

Pediatr Neurol. 2019 Nov:100:92-96. doi: 10.1016/j.pediatrneurol.2019.06.013. Epub 2019 Jun 28.

Authors

Affiliations

¹ Department of Neuroscience, Queensland Children's Hospital, South Brisbane, Queensland, Australia; School of Medicine, University of Queensland, Brisbane, Queensland, Australia. Electronic address: Michaela.waak@health.qld.gov.au.
² Department of Neuroscience, Queensland Children's Hospital, South Brisbane, Queensland, Australia.
³ Anatomical Pathology, Royal Brisbane & Women's Hospital, Herston, Queensland, Australia.
⁴ Department of Neurochemistry, Children's Hospital at Westmead, Westmead, NSW, Australia.
⁵ Department of Clinical Immunology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
⁶ Anatomical Pathology, Royal Brisbane & Women's Hospital, Herston, Queensland, Australia; Department of Molecular and Cellular Pathology, School of Medicine, University of Queensland, Queensland, Australia.
⁷ Queensland Children's Hospital, Rheumatology Department, South Brisbane, Queensland, Australia.
⁸ Queensland Child and Adolescent Renal Service, Queensland Children's Hospital, South Brisbane, Queensland, Australia.
⁹ Neuroimmunology Group, Institute for Neuroscience and Muscle Research, The Children's Hospital at Westmead, University of Sydney, Sydney, NSW, Australia.

PMID: 31376926
DOI: 10.1016/j.pediatrneurol.2019.06.013

Abstract

Background: Acute hemorrhagic leukoencephalopathy is a rare encephalopathy of unknown etiology, causing fulminant, hemorrhagic central nervous system demyelination with high mortality. It is unclear whether acute hemorrhagic leukoencephalopathy is an entirely distinct entity from acute disseminated encephalomyelitis.

Patients and methods: We report two patients with rapidly progressive neurological illness resulting in raised intracranial pressure and coma, with biopsy-proven acute hemorrhagic leukoencephalopathy (perivascular hemorrhages and demyelination, predominantly neutrophil infiltrates).

Results: Acute cerebrospinal fluid showed pronounced T cell-associated cytokine elevation (interleukins 6, 8, and 17A) and CCL2 or CCL3, higher than in patients with acute disseminated encephalomyelitis, but no B cell-associated cytokine elevation.

Conclusion: Improved understanding of the immune process may provide rationale for use of anticytokine biologic agents.

Keywords: Acute hemorrhagic leukoencephalopathy (AHLE); Cerebrospinal fluid; Chemokines; Cytokines; Histopathology.

Publication types

Case Reports
Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Cytokines / cerebrospinal fluid*
Humans
Leukoencephalitis, Acute Hemorrhagic* / cerebrospinal fluid
Leukoencephalitis, Acute Hemorrhagic* / immunology
Leukoencephalitis, Acute Hemorrhagic* / pathology
Leukoencephalitis, Acute Hemorrhagic* / physiopathology
Magnetic Resonance Imaging
Male

Substances

Cytokines