title: Confirmed enterovirus encephalitis with associated steroid-responsive acute disseminated encephalomyelitis: an overlapping infection and inflammation syndrome.
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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Australia; TY Nelson Department of Neurology, the Children's Hospital at Westmead, Australia.
authors.forename: Sekhar
authors.initials: S
authors.lastname: Pillai
authors.affiliation: 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.
authors.forename: Esther
authors.initials: E
authors.lastname: Tantsis
authors.affiliation: Department of Radiology, the Children's Hospital at Westmead, Australia.
authors.forename: Kristina
authors.initials: K
authors.lastname: Prelog
authors.affiliation: Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Australia; Discipline of Paediatrics and Child Health, University of Sydney, Australia.
authors.forename: Sudarshini
authors.initials: S
authors.lastname: Ramanathan
authors.affiliation: TY Nelson Department of Neurology, the Children's Hospital at Westmead, Australia.
authors.forename: Richard
authors.initials: R
authors.lastname: Webster
authors.affiliation: TY Nelson Department of Neurology, the Children's Hospital at Westmead, Australia.
authors.forename: Robert A
authors.initials: RA
authors.lastname: Ouvrier
authors.affiliation: 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.
authors.forename: Alison
authors.initials: A
authors.lastname: Kesson
authors.affiliation: Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Australia; Discipline of Paediatrics and Child Health, University of Sydney, Australia.
authors.forename: Fabienne
authors.initials: F
authors.lastname: Brilot
authors.affiliation: 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: russell.dale@health.nsw.gov.au.
authors.forename: Russell C
authors.initials: RC
authors.lastname: Dale
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001323:Autoantibodies / Q000032:analysis; D001327:Autoimmune Diseases / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D004673:Encephalomyelitis, Acute Disseminated / Q000134:cerebrospinal fluid / Q000150:complications*; D004769:Enterovirus Infections / Q000134:cerebrospinal fluid / Q000150:complications*; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007167:Immunotherapy; D007223:Infant; D007249:Inflammation / Q000134:cerebrospinal fluid / Q000150:complications*; D008297:Male; D063345:Oligodendrocyte-Myelin Glycoprotein / Q000097:blood / Q000276:immunology; D016133:Polymerase Chain Reaction; D013577:Syndrome
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D063345:Oligodendrocyte-Myelin Glycoprotein
keywords: ADEM; Autoimmune; Demyelination; Encephalitis; Enterovirus; Steroid