Cause of rare crippling disease in children discovered thanks to 14-year-old autopsy samples

The cause of a mysterious polio-like disease found primarily in children may have finally been identified thanks to an 11-year-old case study. New research – published last week on New England Journal of Medicine – provides the most substantial evidence to date that an enterovirus causes the condition, which is called acute flaccid myelitis (AFM) and has bewildered scientists For years.

New analysis of a 2011 study the death of a 5-year-old boy may have finally silenced the debate. Traces of enterovirus D68 (EV-D68) – a usually harmless respiratory virus – have been found in the spinal cord of the child who probably died of AFM in 2008.

“[Our] the findings support the view that EV-D68 infection is a cause of AFM, “the authors write in their paper.

“The pathogenesis of AFM may involve a combination of the direct effects of viral infection of motor neurons in the spinal cord and damage resulting from local inflammation.”

AFM it is a rare but serious condition that affects the nervous system. It causes inflammation of the spinal cord, which can lead to weakening of muscles and reflexes in the body. Sometimes, this can cause permanent paralysis and, in severe cases, can compromise the respiratory muscles and be fatal. The vast majority – 90 percent – of cases involve very young children.

There have been spikes in cases every two years in the United States since 2014, mostly between August and October. In 2018 the Centers for Disease Control and Prevention (CDC) reported 238 cases. Another peak was expected for 2020, but thanks to social distancing measures introduced as part of the response to the COVID-19 pandemic, cases were limited to just 33. So far in 2022, there have only been three confirmed cases.

In recent years, EV-D68 has emerged as the most likely cause of AFM, but so far there is a lack of evidence. EV-D68 is a cousin of the poliovirus and “has [previously] it has been detected in respiratory samples obtained from patients with AFM, but has rarely been detected in cerebrospinal fluid, “the authors of the new study write.

And there have been very few autopsy studies, they add. Children, thankfully, rarely die from the condition – there have been only two deaths out of 682 cases in the United States since 2014 – although that means there are fewer opportunities to study it as spinal cord biopsies are too dangerous to be. performed on living patients.

Fortunately, the team was able to find the 2011 case study and re-examine the autopsy tissues. By analyzing spinal cord cells, the team found RNA and EV-D68 proteins in motor neurons, as well as inflammation in infected cells.

“This is exciting. It is the best proof [that EV-D68 causes AFM] we had in humans, “said Dr. Megan Culler Freeman, an infectious disease specialist at Children’s Hospital of Pittsburgh Health Day.

Hopefully, the discovery will help inform future research on the condition and potential treatment options.

“Unfortunately, we currently have no antiviral or antibody drugs to treat EV-D68 infection in humans,” said Dr Matthew Vogt of the UNC School of Medicine and lead author of the study in a declaration. Even if he and his colleagues are experimenting therapies with monoclonal antibodieslike those used to treat SARS-CoV-2.

He remains optimistic: “If we can understand how this disease works, then we can work to figure out how to prevent it from happening.”

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