In mice, the drug bortezomib preserved muscle growth and prevented loss of elbow and shoulder motion in conditions resembling cerebral palsy and newborn brachial plexus injury, the most common causes of childhood paralysis.
Over time, these muscle contractures disrupt skeletal growth, leading to pain, loss of mobility, and heavy reliance on costly health care and supportive services. Many affected children require surgeries and other treatments that can temporarily relieve symptoms but do not cure the contractures. This lack of effective treatments results from doctors not knowing how muscle contractures form in childhood paralysis. Until now.
Experts at Cincinnati Children’s report that, in mice, a drug called bortezomib helped re-balance disrupted muscle growth and prevented the contractures that untreated mice displayed. This early-stage success suggests that future generations of children facing these conditions may be treated in new and far more effective ways.
The study was published online Oct. 29, 2019, in JCI Insight.
«After four weeks of treatment shortly after birth, our study found that bortezomib significantly reduced shoulder and elbow contractures in a mouse model that mimics these common childhood conditions,» says Roger Cornwall, MD, Division of Pediatric Orthopaedics. «Future studies confirming the efficacy of this approach could ultimately render obsolete the destructive surgeries currently required to alleviate contractures in a variety of conditions.»
Conditions affect 1 in every 200 newborns
The brachial plexus is a bundle of intertwined nerves running from the neck that control movement and sensation in the arm and hand. In some cases, these nerves can be damaged during childbirth, leading to weakness or even complete paralysis of an arm. While about two thirds of such injuries heal on their own, the rest can require nerve reconstruction and other therapies.
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Materials provided by Cincinnati Children’s Hospital Medical Center. Note: Content may be edited for style and length.