Michaela Waak1, Stephen Malone2, Kate Sinclair2, Gael Phillips3, Sushil Bandodkar4, Louise Wienholt5, Thomas Robertson6, Ben Whitehead7, Peter Trnka8, Kavitha Kothur9, Russel C Dale9. 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.
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. Crown
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. Crown
Authors: Maria L Benevides; Stefany Elias; Diego A Fagundes; Rafael F Martins; Michel M Dutra; Maria E Rodrigues de Oliveira Thais; Gabriel M Rodrigues; Jean C Nunes; Gladys L Martins Journal: Neurohospitalist Date: 2022-03-03