School of Medicine

Children with rare gene mutation gather in St. Louis as doctors, researchers try to build treatment guide

ST. LOUIS • When only 100 people in the world share your child’s specific gene mutation, it is hard to find anyone who understands, including doctors.

Three dozen families came here from Argentina, Italy and all over the U.S. this week to help doctors understand the medical challenges caused by a mutation of the GNAO1 gene. Symptoms including seizures, uncontrolled movements and developmental delays typically start in the child’s first year.

The gene mutation, discovered in 2013, probably has been misdiagnosed as cerebral palsy or other disorders in many more children and adults who have not had complete DNA testing, said Dr. Amy Viehoever, assistant professor of neurology at Washington University, which hosted the first-ever GNAO1 clinic Thursday and Friday.

Doctors and researchers at the clinic are trying to build a diagnostic and treatment guide for the rare disease. They plan to hold the clinic every year to track the children’s symptoms and provide a baseline for researchers to measure success or failure if a treatment becomes available.

“Unlike something like Alzheimer’s or stroke with thousands and thousands of patients, in rare diseases we can’t do these big clinical trials with placebo-controlled studies,” Viehoever said. “There are a limited number of patients, and obviously these families are desperate for treatments.”

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