Outbreak of brainstem encephalitis associated with enterovirus-A71 in Catalonia, Spain (2016): a clinical observational study in a children's reference centre in Catalonia.
OBJECTIVE
To describe the characteristics of an outbreak of brainstem encephalitis and encephalomyelitis related to enterovirus (EV) infection in Catalonia (Spain), a setting in which these manifestations were uncommon.


METHODS
Clinical and microbiological data were analysed from patients with neurological symptoms associated with EV detection admitted to a reference paediatric hospital between April and June 2016.


RESULTS
Fifty-seven patients were included. Median age was 27.7 months (p25-p75 17.1-37.6). Forty-one (72%) were diagnosed with brainstem encephalitis, seven (12%) with aseptic meningitis, six (11%) with encephalitis, and three (5%) with encephalomyelitis (two out of three with cardiopulmonary failure). Fever, lethargy, and myoclonic jerks were the most common symptoms. Age younger than 12 months, higher white-blood-cell count, and higher procalcitonin levels were associated with cardiopulmonary failure. Using a PAN-EV real-time PCR, EV was detected in faeces and/or nasopharyngeal aspirate in all the patients, but it was found in cerebrospinal fluid only in patients with aseptic meningitis. EV was genotyped in 47 out of 57 and EV-A71 was identified in 40 out of 47, being the only EV type found in patients with brainstem symptoms. Most of the detected EV-A71 strains were subgenogroup C1. Intravenous immunoglobulins were used in 34 patients. Eight cases (14%) were admitted to the intensive care unit. All the patients but three, those with encephalomyelitis, showed a good clinical course and had no significant sequelae. No deaths occurred.


CONCLUSIONS
The 2016 outbreak of brainstem encephalitis in Catalonia was associated with EV-A71 subgenogroup C1. Despite the clinical manifestations of serious disease, a favourable outcome was observed in the majority of patients.
Department of Paediatrics, Hospital Sant Joan de Deu (University of Barcelona), Spain.
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Casas-Alba
Department of Paediatrics, Hospital Sant Joan de Deu (University of Barcelona), Spain; Paediatric Infectious Diseases Research Group, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, Spain; CIBER en Epidemiología y Salud Pública, CIBERESP, Spain.
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de Sevilla
Paediatric Infectious Diseases Research Group, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, Spain; Department of Molecular Microbiology, Hospital Sant Joan de Deu, Spain.
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Valero-Rello
Department of Paediatrics, Hospital Sant Joan de Deu (University of Barcelona), Spain; Paediatric Infectious Diseases Research Group, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, Spain; CIBER en Epidemiología y Salud Pública, CIBERESP, Spain.
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Fortuny
Department of Paediatrics, Hospital Sant Joan de Deu (University of Barcelona), Spain; Paediatric Infectious Diseases Research Group, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, Spain; CIBER en Epidemiología y Salud Pública, CIBERESP, Spain.
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García-García
Department of Paediatric Neurology, Hospital Sant Joan de Deu (University of Barcelona), Spain.
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Ortez
Department of Diagnostic Imaging, Hospital Sant Joan de Deu (University of Barcelona), Spain.
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Muchart
Department of Paediatric Neurology, Hospital Sant Joan de Deu (University of Barcelona), Spain.
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Armangué
Paediatric Infectious Diseases Research Group, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, Spain; CIBER en Epidemiología y Salud Pública, CIBERESP, Spain; Paediatric Intensive Care Unit, Hospital Sant Joan de Deu (University of Barcelona), Spain.
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Jordan
Emergency Department, Hospital Sant Joan de Deu (University of Barcelona), Spain.
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Luaces
Epidemiological Surveillance Unit of Health Region, Barcelona-South, Public Health Agency of Catalonia, Hospitalet de Llobregat, Spain.
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Barrabeig
Enterovirus Unit, National Centre for Microbiology, Institute of Public Health "Carlos III", Madrid, Spain.
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González-Sanz
Enterovirus Unit, National Centre for Microbiology, Institute of Public Health "Carlos III", Madrid, Spain.
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Cabrerizo
Paediatric Infectious Diseases Research Group, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, Spain; CIBER en Epidemiología y Salud Pública, CIBERESP, Spain; Emergency Department, Hospital Sant Joan de Deu (University of Barcelona), Spain; School of Medicine, Universitat Internacional de Catalunya, Barcelona, Spain.
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Muñoz-Almagro
Department of Paediatrics, Hospital Sant Joan de Deu (University of Barcelona), Spain; Paediatric Infectious Diseases Research Group, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, Spain; CIBER en Epidemiología y Salud Pública, CIBERESP, Spain. Electronic address: claunes@sjdhospitalbarcelona.org.
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Launes
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001933:Brain Stem / Q000821:virology*; D002675:Child, Preschool; D004196:Disease Outbreaks / Q000706:statistics & numerical data*; D018792:Encephalitis, Viral / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy / Q000821:virology; D029821:Enterovirus A, Human / Q000235:genetics*; D004769:Enterovirus Infections / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy / Q000821:virology; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D017720:Molecular Epidemiology; D013030:Spain / Q000453:epidemiology
D000893:Anti-Inflammatory Agents
Brainstem; EV-A71; Encephalitis; Enterovirus; Molecular epidemiology; Types