Confirmed enterovirus encephalitis with associated steroid-responsive acute disseminated encephalomyelitis: an overlapping infection and inflammation syndrome

Sekhar Pillai; Esther Tantsis; Kristina Prelog; Sudarshini Ramanathan; Richard Webster; Robert A Ouvrier; Alison Kesson; Fabienne Brilot; Russell C Dale

doi:10.1016/j.ejpn.2014.12.015

Confirmed enterovirus encephalitis with associated steroid-responsive acute disseminated encephalomyelitis: an overlapping infection and inflammation syndrome

Eur J Paediatr Neurol. 2015 Mar;19(2):266-70. doi: 10.1016/j.ejpn.2014.12.015. Epub 2014 Dec 24.

Authors

Sekhar Pillai¹, Esther Tantsis², Kristina Prelog³, Sudarshini Ramanathan⁴, Richard Webster⁵, Robert A Ouvrier⁵, Alison Kesson⁶, Fabienne Brilot⁴, Russell C Dale⁷

Affiliations

¹ Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Australia; TY Nelson Department of Neurology, the Children's Hospital at Westmead, Australia.
² Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Australia; TY Nelson Department of Neurology, the Children's Hospital at Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney, Australia.
³ Department of Radiology, the Children's Hospital at Westmead, Australia.
⁴ Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Australia; Discipline of Paediatrics and Child Health, University of Sydney, Australia.
⁵ TY Nelson Department of Neurology, the Children's Hospital at Westmead, Australia.
⁶ Department of Infectious Diseases and Microbiology, the Children's Hospital at Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney, Australia; Marie Bashir Institute, University of Sydney, Australia.
⁷ Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Australia; TY Nelson Department of Neurology, the Children's Hospital at Westmead, Australia; Discipline of Paediatrics and Child Health, University of Sydney, Australia. Electronic address: [email protected].

PMID: 25576193
DOI: 10.1016/j.ejpn.2014.12.015

Abstract

Background: Inflammatory disorders of the central nervous system have generally been separated into infectious or immune-mediated aetiologies. However, there are emerging examples of confirmed infectious viral infection of the brain followed by secondary inflammation or autoimmunity that is amenable to immune suppressive therapies.

Methods: We report four children with confirmed enterovirus encephalitis (CSF enterovirus PCR positivity), who had MRI evidence of inflammatory demyelination compatible with ADEM.

Results: Two patients had a monophasic course, whereas two had a biphasic course. Serum myelin oligodendrocyte glycoprotein antibodies were negative in two tested patients, although all patients had mirrored CSF and serum oligoclonal bands. All four patients only improved with introduction of immune therapy (corticosteroids in three, corticosteroid and intravenous immunoglobulin in one).

Conclusion: These cases provide a further example of the overlap between CNS infection and immune mediated CNS disease. Randomised controlled trials investigating immune therapies in encephalitis are required.

Keywords: ADEM; Autoimmune; Demyelination; Encephalitis; Enterovirus; Steroid.

Publication types

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

MeSH terms

Adrenal Cortex Hormones / therapeutic use
Autoantibodies / analysis
Autoimmune Diseases / etiology
Child
Child, Preschool
Encephalomyelitis, Acute Disseminated / cerebrospinal fluid
Encephalomyelitis, Acute Disseminated / complications*
Enterovirus Infections / cerebrospinal fluid
Enterovirus Infections / complications*
Female
Humans
Immunoglobulins, Intravenous / therapeutic use
Immunotherapy
Infant
Inflammation / cerebrospinal fluid
Inflammation / complications*
Male
Oligodendrocyte-Myelin Glycoprotein / blood
Oligodendrocyte-Myelin Glycoprotein / immunology
Polymerase Chain Reaction
Syndrome

Substances

Adrenal Cortex Hormones
Autoantibodies
Immunoglobulins, Intravenous
Oligodendrocyte-Myelin Glycoprotein