Reports of a puzzling polio-like syndrome affecting children in northern California have launched something of media frenzy.
Emmanuelle Waubant, MD, PhD, professor, clinical neurology and pediatrics, University of California at San Francisco (UCSF), said she was surprised by all the attention to this case series, and believes it's because the cases are linked to "children" and "polio," 2 words that set off alarms when used together.
To date, Dr. Waubant and her colleagues have identified 5 children, aged 2 to 16 years, who presented with acute onset of flaccid paralysis affecting 1 or more limbs that reached peak severity within 48 hours of onset. MRI showed non enhancing T2 hyperintensities of the central gray matter; cerebral spinal fluid was normal, albeit mildly pleocytotic.
The cases will be presented at the upcoming 66th Annual Meeting of the American Academy of Neurology (AAN) in Philadelphia, April 26 to May 3.
Doctors initially suspected transverse myelitis, an inflammation of the spinal cord that may cause paralysis, but lack of sensory changes and other telltale neurologic symptoms, plus a negative workup, ruled out this diagnosis, said Dr. Waubant. They also excluded Guillain-Barré syndrome and botulism.
Three of the youngsters had prodromal respiratory
illness before their paralysis, which, said Dr. Waubant, "is probably
significant."
Poliovirus has been eradicated from most of the globe, but
other viruses can also injure the lower motor neurons of the spine,
leading to a polio-like syndrome.
So far, 2 cases have tested positive for the enterovirus-68, a rare virus that in the past has been associated with polio-like syndrome.
"In the case of an enterovirus, you can have a cold-like presentation," said Dr. Waubant. "We think that if it's a virus [responsible for the symptoms presented], it's probably a virus that is not responsible for paralysis in every single patient that is affected, only in specific patients that have the right biological makeup."
Children Vaccinated
Although the disorder "looks clinically like polio," polio itself was also ruled out because all the children had been vaccinated against the polio virus. "All the cases had a good level of antibodies so they could not be infected with the polio virus," said Dr. Waubant.
There's nothing really to tie these children together except for their clinical presentation. "They had nothing in common," said Dr. Waubant. "It's not a cluster because they came from different areas," although they were from within a 100-mile radius of each other in northern California.
Treatment with steroids, intravenous immunoglobulin, and/or plasma exchange did not appear to have a clinical benefit, with motor function recovery at 6 months described as being poor. "The children have remained quite disabled," she said. "Some have recovered a little bit but the improvement has been from zero to marginal."
Emmanuelle Waubant, MD, PhD, professor, clinical neurology and pediatrics, University of California at San Francisco (UCSF), said she was surprised by all the attention to this case series, and believes it's because the cases are linked to "children" and "polio," 2 words that set off alarms when used together.
To date, Dr. Waubant and her colleagues have identified 5 children, aged 2 to 16 years, who presented with acute onset of flaccid paralysis affecting 1 or more limbs that reached peak severity within 48 hours of onset. MRI showed non enhancing T2 hyperintensities of the central gray matter; cerebral spinal fluid was normal, albeit mildly pleocytotic.
The cases will be presented at the upcoming 66th Annual Meeting of the American Academy of Neurology (AAN) in Philadelphia, April 26 to May 3.
Doctors initially suspected transverse myelitis, an inflammation of the spinal cord that may cause paralysis, but lack of sensory changes and other telltale neurologic symptoms, plus a negative workup, ruled out this diagnosis, said Dr. Waubant. They also excluded Guillain-Barré syndrome and botulism.
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So far, 2 cases have tested positive for the enterovirus-68, a rare virus that in the past has been associated with polio-like syndrome.
"In the case of an enterovirus, you can have a cold-like presentation," said Dr. Waubant. "We think that if it's a virus [responsible for the symptoms presented], it's probably a virus that is not responsible for paralysis in every single patient that is affected, only in specific patients that have the right biological makeup."
Children Vaccinated
Although the disorder "looks clinically like polio," polio itself was also ruled out because all the children had been vaccinated against the polio virus. "All the cases had a good level of antibodies so they could not be infected with the polio virus," said Dr. Waubant.
There's nothing really to tie these children together except for their clinical presentation. "They had nothing in common," said Dr. Waubant. "It's not a cluster because they came from different areas," although they were from within a 100-mile radius of each other in northern California.
Treatment with steroids, intravenous immunoglobulin, and/or plasma exchange did not appear to have a clinical benefit, with motor function recovery at 6 months described as being poor. "The children have remained quite disabled," she said. "Some have recovered a little bit but the improvement has been from zero to marginal."
