title: Survival after pulmonary edema due to enterovirus 71 encephalitis.
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Neurology, Sydney Children's Hospital, NSW, Australia.
authors.forename: M A
authors.initials: MA
authors.lastname: Nolan
authors.forename: M E
authors.initials: ME
authors.lastname: Craig
authors.forename: M M
authors.initials: MM
authors.lastname: Lahra
authors.forename: W D
authors.initials: WD
authors.lastname: Rawlinson
authors.forename: P C
authors.initials: PC
authors.lastname: Prager
authors.forename: G D
authors.initials: GD
authors.lastname: Williams
authors.forename: A M E
authors.initials: AM
authors.lastname: Bye
authors.forename: P I
authors.initials: PI
authors.lastname: Andrews
mesh_terms: D000208:Acute Disease; D000998:Antiviral Agents / Q000627:therapeutic use; D002675:Child, Preschool; D003131:Combined Modality Therapy; D004196:Disease Outbreaks; D018792:Encephalitis, Viral / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology; D004770:Enterovirus / Q000302:isolation & purification*; D004769:Enterovirus Infections / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009517:New South Wales / Q000453:epidemiology; D010069:Oxadiazoles / Q000627:therapeutic use; D010080:Oxazoles; D011654:Pulmonary Edema / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology* / Q000401:mortality / Q000628:therapy / Q000821:virology; D016019:Survival Analysis; D017741:Survivors
chemical_list: D000998:Antiviral Agents; D016756:Immunoglobulins, Intravenous; D010069:Oxadiazoles; D010080:Oxazoles; C115201:pleconaril