Challenging case: Enterovirus

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On Oct. 3, 2014, Elisa Holt was nursing her six-month-old son Noah when she realized he wasn’t moving his feet, legs or toes. Panicked, she called her pediatrician at Garden City Pediatrics and was directed to Beverly Hospital.

“We are so thankful for the emergency room doctor [Dr. Munirah Qualls] who told us, ‘I don’t know. I’m going to send you to Boston Children’s Hospital.’”

Within 15 minutes, an ambulance arrived to rush Noah to Boston Children’s where the emergency department was on high alert for EV D-68.

A bedside spinal tap and initial lab results narrowed Noah’s diagnosis to either Guillain Barre Syndrome or a type of myelitis possibly caused by EV-D68. Both conditions can be treated with immunotherapy, so Noah’s doctors moved forward with treatment.

Then an MRI confirmed Acute Flaccid Myelitis (AFM), a disorder caused by inflammation of the spinal cord. “We don’t know if EV D68 is the cause of AFM. There’s epidemiologic evidence that suggests it is, but we don’t have hard data to suggest that’s the case,” explains Mark Gorman, MD, from the Boston Children’s Hospital Department of Neurology.

The unproven link between enterovirus and AFM is not the only mystery associated with the virus.

“Assuming EV D68 causes AFM, experts are unsure of the mechanism of disease,” says Gorman.

There are three possibilities:

  • EV D68 may directly infect the spinal cord tissue and cause an infection.
  • EV D68 may infect the respiratory tract and provoke an autoimmune response against the spinal cord.
  • The mechanism may be a hybrid of these two possibilities.

Because researchers and physicians haven’t identified the mechanism at play, there are unknowns in terms of the best treatment.

holt_noah5_cropTreating AFM

The Centers for Disease Control and Prevention has published treatment guidelines that indicate no treatments are effective for AFM. However, doctors at Boston Children’s and other hospitals have used immunotherapy, including corticosteroids, intravenous immunoglobulin and/or plasma exchange, on a case-by-case basis with some anecdotal suggestion of benefit in some patients.

“We’ve tended to treat pretty aggressively with anti-inflammatory agents. Based on our observations, this treatment seems to be temporally associated with improvements,” says Gorman.

Other factors, including age and initial severity of presentation, also may correlate with a child’s prognosis. Younger children seem to have a more guarded prognosis, says Gorman. Patients with more severe initial presentations also seem to take longer to recover.

Noah’s recovery

After eight days at Boston Children’s, Noah was discharged and referred for twice-weekly physical therapy, which has expanded to include music therapy, hippotherapy and pool therapy.

More than 18 months after diagnosis, Noah is walking with a gait trainer and standing with assistance.

His mother and Gorman credit Noah’s fast-acting pediatrician and emergency room physician with this progress.

“If a child come into a primary care provider’s office with significant weakness, she should be sent directly to the emergency room,” says Gorman. We need to do a thorough workup to look for the cause. If the child does have enterovirus, earlier respiratory tract testing may produce a positive result.”

Learn more about the Department of Neurology.