Acute Hemorrhagic Leukoencephalopathy: Pathological Features and Cerebrospinal Fluid Cytokine Profiles - 30/10/19

Doi : 10.1016/j.pediatrneurol.2019.06.013

Michaela Waak, MD ^a,^b,^⁎ , Stephen Malone, MBBS ^a, Kate Sinclair, MBBS ^a, Gael Phillips, MBBS ^c, Sushil Bandodkar, PhD ^d, Louise Wienholt, BSc ^e, Thomas Robertson, MBBS ^c,^f, Ben Whitehead, MBBS ^g, Peter Trnka, MBBS ^h, Kavitha Kothur, PhD ⁱ, Russel C. Dale, PhD ⁱ
^a Department of Neuroscience, Queensland Children's Hospital, South Brisbane, Queensland, Australia
^b School of Medicine, University of Queensland, Brisbane, Queensland, Australia
^c Anatomical Pathology, Royal Brisbane & Women's Hospital, Herston, Queensland, Australia
^d Department of Neurochemistry, Children's Hospital at Westmead, Westmead, NSW, Australia
^e Department of Clinical Immunology, Royal Prince Alfred Hospital, Sydney, NSW, Australia
^f Department of Molecular and Cellular Pathology, School of Medicine, University of Queensland, Queensland, Australia
^g Queensland Children's Hospital, Rheumatology Department, South Brisbane, Queensland, Australia
^h Queensland Child and Adolescent Renal Service, Queensland Children's Hospital, South Brisbane, Queensland, Australia
ⁱ Neuroimmunology Group, Institute for Neuroscience and Muscle Research, The Children's Hospital at Westmead, University of Sydney, Sydney, NSW, Australia

^∗Communications should be addressed to: Waak; Department of Neuroscience; Queensland Children's Hospital; 501 Stanley Street, 4101 South Brisbane, Australia.Department of NeuroscienceQueensland Children's Hospital501 Stanley StreetSouth Brisbane4101Australia

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.

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

Plan

Introduction

Materials and methods

Patient descriptions

CSF findings

Discussion

Declarations of interest: none.

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Vol 100

P. 92-96 - novembre 2019 Retour au numéro

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Acute Hemorrhagic Leukoencephalopathy: Pathological Features and Cerebrospinal Fluid Cytokine Profiles - 30/10/19

Abstract

Background

Patients and Methods

Results

Conclusion

Plan

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