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Pediatrics. 2015 Apr;135(4):e974-84. doi: 10.1542/peds.2014-2702.

Infectious and autoantibody-associated encephalitis: clinical features and long-term outcome.

Author information

1
Neuroimmunology Group, Institute of Neuroscience and Muscle Research at the Kids Research Institute, Children's Hospital at Westmead, University of Sydney, Australia; TY Nelson Department of Neurology and Neurosurgery and.
2
Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, England;
3
Departments of Medical Imaging.
4
Neuroimmunology Group, Institute of Neuroscience and Muscle Research at the Kids Research Institute, Children's Hospital at Westmead, University of Sydney, Australia;
5
Infectious Diseases and Microbiology, and.
6
Statistics, the Children's Hospital at Westmead, Sydney, Australia; National Health Medical Research Council Clinical Trials Centre, University of Sydney, Camperdown, New South Wales, Australia;
7
TY Nelson Department of Neurology and Neurosurgery and.
8
Centre Hospitalier Territorial Magenta, Service Pediatric, Nouméa, New Caledonia;
9
Chelsea & Westminster Hospital, Department of Neurology, Imperial College Healthcare National Health Service Trust, London, England; and.
10
Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, England; Evelina Children's Hospital, London, England.
11
Neuroimmunology Group, Institute of Neuroscience and Muscle Research at the Kids Research Institute, Children's Hospital at Westmead, University of Sydney, Australia; TY Nelson Department of Neurology and Neurosurgery and russell.dale@health.nsw.gov.au.

Abstract

BACKGROUND AND OBJECTIVES:

Pediatric encephalitis has a wide range of etiologies, clinical presentations, and outcomes. This study seeks to classify and characterize infectious, immune-mediated/autoantibody-associated and unknown forms of encephalitis, including relative frequencies, clinical and radiologic phenotypes, and long-term outcome.

METHODS:

By using consensus definitions and a retrospective single-center cohort of 164 Australian children, we performed clinical and radiologic phenotyping blinded to etiology and outcomes, and we tested archived acute sera for autoantibodies to N-methyl-D-aspartate receptor, voltage-gated potassium channel complex, and other neuronal antigens. Through telephone interviews, we defined outcomes by using the Liverpool Outcome Score (for encephalitis).

RESULTS:

An infectious encephalitis occurred in 30%, infection-associated encephalopathy in 8%, immune-mediated/autoantibody-associated encephalitis in 34%, and unknown encephalitis in 28%. In descending order of frequency, the larger subgroups were acute disseminated encephalomyelitis (21%), enterovirus (12%), Mycoplasma pneumoniae (7%), N-methyl-D-aspartate receptor antibody (6%), herpes simplex virus (5%), and voltage-gated potassium channel complex antibody (4%). Movement disorders, psychiatric symptoms, agitation, speech dysfunction, cerebrospinal fluid oligoclonal bands, MRI limbic encephalitis, and clinical relapse were more common in patients with autoantibodies. An abnormal outcome occurred in 49% of patients after a median follow-up of 5.8 years. Herpes simplex virus and unknown forms had the worst outcomes. According to our multivariate analysis, an abnormal outcome was more common in patients with status epilepticus, magnetic resonance diffusion restriction, and ICU admission.

CONCLUSIONS:

We have defined clinical and radiologic phenotypes of infectious and immune-mediated/autoantibody-associated encephalitis. In this resource-rich cohort, immune-mediated/autoantibody-associated etiologies are common, and the recognition and treatment of these entities should be a clinical priority.

KEYWORDS:

MRI; autoantibody; encephalitis; infection; outcome

PMID:
25802349
DOI:
10.1542/peds.2014-2702
[Indexed for MEDLINE]
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