N.J. child dies, tests positive for enterovirus, while CHOP reports three cases of muscle weakness

Children's Hospital of Philadelphia on Friday reported that it was treating three patients with a combination of muscle weakness and spinal abnormality, joining a growing number of institutions across the country that are seeking to understand clusters of cases with the same symptoms.

While the symptoms have been seen before, the combination is uncommon and similar cases would normally be seen over a much longer time period. This type of neurological disorder is often caused by an infection, and investigators hope to determine whether these symptoms are linked to an enterovirus, D68, that suddenly showed up in increasing numbers less than a month ago and has been causing respiratory illness in at least 40 states.

Late Friday night, the U.S. Centers for Disease Control and Prevention notified the New Jersey Department of Health that a 4-year-old boy from Mercer County who died last week had been tested positive for EV-D68, although it was not known if the virus caused the death.

For the neurological cases, among the distinguishing symptoms is a weakness, not paralysis, typically in one limb. Maybe the patient is "not able to lift an arm fully over the head but can move the shoulder," said Brenda Banwell, chief of neurology at Children's Hospital, or perhaps the child can "move fingers but is not able to hold or grip."

The other key finding is a lesion on a part of the spinal cord that controls motor strength that can be seen on an MRI largely restricted to what is known as gray matter.

The CDC on Sept. 26 asked clinicians to review their records and report cases with those two findings since Aug. 1 in patients up to age 21. The three patients at Children's Hospital, which normally would see between three and eight cases a year with some similarities, were the only ones that met the CDC criteria.

In the two weeks before the children were admitted, Banwell said, two of them had experienced a mild fever and rash, while one had cough- or cold-like symptoms but no fever and no rash.

"They are not getting worse," she said, declining to give details. "This is a type of illness where the recovery can take some time."

About 20 similar cases have been reported around the country, although that number is expected to grow rapidly in response to the CDC's request for a review.

At the same time, hundreds of cases of the enterovirus D68 have been reported, including six in Pennsylvania and nine in New Jersey. Some have caused severe respiratory illness around the country. Specimens from several children who died, including the Mercer County child, were positive for EV-D68, although tests to determine the cause of death are continuing; hundreds of viruses circulate in the fall, and it would not be unusual to find some in a patient who died from any number of causes.

Similarly, a link between EV-D68 and the muscle weakness is possible but not confirmed. At least four Colorado children with the neurological symptoms also tested positive for the enterovirus, the CDC said Friday, but the meaning of that finding was not yet known.

Banwell said tests for EV-D68 in the three patients here were not expected back until next week.

The treatment - mainly to manage swelling and enhance immune response - would not necessarily change; there are no antiviral drugs to treat EV-D68.

Should parents worry?

"Any abnormality of the spinal cord is a serious neurological illness," Banwell said.

She had the usual advice: practice good hand hygiene, don't visit people who are visibly ill. If a child has borderline weakness of a limb, ask a pediatrician. If it is severe or builds quickly, go to the emergency room. (Ditto for breathing problems that could be caused by an enterovirus.)

"If a child has any weakness of arms or legs, or facial weakness, bring then in for a consultation" at Children's Hospital, she said, which has a formal program to deal with this type of neurological illness.

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@DonSapatkin