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From the Division of Neurology (M.A. Nolan, and Drs. Bye and Andrews) and Division of Intensive Care (Drs. Prager and Williams), Sydney Children’s Hospital; Department of Microbiology (Drs. Craig and Rawlinson, and M.M. Lahra), South Eastern Area Laboratory Services, Prince of Wales Hospital; and the School of Women’s and Children’s Health (M.A. Nolan, and Drs. Craig, Williams, Bye, and Andrews), School of Medical Sciences and School of Biotechnology and Biomolecular Sciences (Dr. Rawlinson), University of New South Wales, Sydney, Australia.
Address correspondence and reprint requests to Dr. P. Ian Andrews, Division of Neurology, Sydney Children’s Hospital, High St., Randwick, Sydney, NSW 2031, Australia; e-mail: I.Andrews{at}unsw.edu.au
Background: A distinctive pattern of enterovirus 71 (EV71) infection, characterized by fever, exanthem, acute pulmonary edema (PE), brainstem encephalitis, and flaccid paresis, affects infants and young children. Most die rapidly owing to respiratory failure and fulminant PE.
Method: The authors report short- and long-term outcome of six survivors of the acute illness.
Results: In the context of acute PE and widespread weakness, recognition of the underlying neurologic disorder was facilitated by the distinctive pattern of MRI signal abnormalities in posterior pons and medulla. EV71-specific PCR of clinical samples helped confirm the diagnosis. Acute PE was managed with mechanical ventilation, afterload reduction, and inotrope support, and resolved completely over days. One patient with minimal neurologic recovery died 9 weeks after disease onset. The other patients have residual neurologic dysfunction, varying from subtle monoparesis to severe bulbar dysfunction, central and peripheral respiratory failure, and flaccid quadriparesis. Faster neurologic recovery was associated with less long-term deficit. Long-term outcome was similar in patients treated with and without pleconaril or IV immunoglobulin. Three long-term survivors treated with IV corticosteroids had less severe long-term neurologic disability than two not treated with steroids.
Conclusion: Acute pulmonary edema and encephalomyelitis occurs with EV71 infection in infants. Long-term neurologic outcome varied from minor, focal weakness to profound, global motor dysfunction with respiratory failure.
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