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Neurologic complications in children with enterovirus 71 infection
C C Huang1, C C Liu, Y C Chang
1Department of Pediatrics, College of Medicine, National Cheng Kung University, Tainan, Taiwan. huangped@mail.ncku.edu.tw
Insights
Enterovirus 71 caused severe neurologic issues, primarily rhombencephalitis, during Taiwan's 1998 epidemic. This brainstem inflammation led to significant morbidity and a 14% fatality rate in children.
Area of Science:
- Neurology
- Infectious Diseases
- Pediatrics
Background:
- Enterovirus 71 (EV71) causes hand-foot-and-mouth disease (HFMD) in children, presenting with fever, oral lesions, and limb vesicles.
- EV71 infection can lead to severe neurological complications, including aseptic meningitis, encephalomyelitis, and acute flaccid paralysis resembling polio.
Purpose of the Study:
- To describe the spectrum of neurologic complications of EV71 infection during the 1998 epidemic in Taiwan.
- To analyze the clinical features, neuroimaging findings, and outcomes of children with EV71-associated neurological syndromes.
Main Methods:
- Retrospective analysis of 41 children with culture-confirmed EV71 infection and acute neurological manifestations.
- Clinical data collection and magnetic resonance imaging (MRI) performed on patients with acute flaccid paralysis and rhombencephalitis.
Main Results:
- The predominant neurologic syndrome was rhombencephalitis (brain-stem encephalitis) in 37 patients (90%).
- Rhombencephalitis presented with varying severity, including myoclonic jerks, cranial nerve palsies, respiratory distress, shock, and coma. Grade III rhombencephalitis had a 71% mortality rate (5/7 deaths).
- MRI revealed brainstem lesions, particularly in the pontine tegmentum, in 17/24 patients. Acute flaccid paralysis occurred in 4 patients, with residual weakness in 2.
Conclusions:
- Rhombencephalitis was the primary neurologic complication of the 1998 EV71 epidemic in Taiwan, with a 14% overall fatality rate.
- Myoclonic jerks were common initial symptoms, and MRI was crucial for diagnosing brainstem involvement.
- Neurological sequelae, including limb weakness and persistent deficits, were observed in survivors.
Background:
Enterovirus 71 infection causes hand-foot-and-mouth disease in young children, which is characterized by several days of fever and vomiting, ulcerative lesions in the oral mucosa, and vesicles on the backs of the hands and feet. The initial illness resolves but is sometimes followed by aseptic meningitis, encephalomyelitis, or even acute flaccid paralysis similar to paralytic poliomyelitis.
Methods:
We describe the neurologic complications associated with the enterovirus 71 epidemic that occurred in Taiwan in 1998. At three major hospitals we identified 41 children with culture-confirmed enterovirus 71 infection and acute neurologic manifestations. Magnetic resonance imaging (MRI) was performed in 4 patients with acute flaccid paralysis and 24 with rhombencephalitis.
Results:
The mean age of the patients was 2.5 years (range, 3 months to 8.2 years). Twenty-eight patients had hand-foot-and-mouth disease (68 percent), and 6 had herpangina (15 percent). The other seven patients had no skin or mucosal lesions. Three neurologic syndromes were identified: aseptic meningitis (in 3 patients); brain-stem encephalitis, or rhombencephalitis (in 37); and acute flaccid paralysis (in 4), which followed rhombencephalitis in 3 patients. In 20 patients with rhombencephalitis, the syndrome was characterized by myoclonic jerks and tremor, ataxia, or both (grade I disease). Ten patients had myoclonus and cranial-nerve involvement (grade II disease). In seven patients the brain-stem infection produced transient myoclonus followed by the rapid onset of respiratory distress, cyanosis, poor peripheral perfusion, shock, coma, loss of the doll's eye reflex, and apnea (grade III disease); five of these patients died within 12 hours after admission. In 17 of the 24 patients with rhombencephalitis who underwent MRI, T2-weighted scans showed high-intensity lesions in the brain stem, most commonly in the pontine tegmentum. At follow-up, two of the patients with acute flaccid paralysis had residual limb weakness, and five of the patients with rhombencephalitis had persistent neurologic deficits, including myoclonus (in one child), cranial-nerve deficits (in two), and ventilator-dependent apnea (in two).
Conclusions:
In the 1998 enterovirus 71 epidemic in Taiwan, the chief neurologic complication was rhombencephalitis, which had a fatality rate of 14 percent. The most common initial symptoms were myoclonic jerks, and MRI usually showed evidence of brainstem involvement.