A student athlete in excruciating shoulder pain couldn’t pinpoint why. A Washington University pediatric neurologist at St. Louis Children’s Hospital diagnosed his rare neurological disorder, Parsonage-Turner Syndrome.
Parsonage-Turner Syndrome (PTS) is a neurological disorder characterized by sudden onset of severe pain in the shoulder or arm followed by weakness in the affected area. It is rare — thought to occur in about 1 to 3 people out of 100,000 each year — and especially uncommon in children. When a pediatric patient was referred to the Children’s Specialty Care Center-West County in St. Louis, Missouri, with pain in his left shoulder, providers initially thought it was a rotator cuff injury, but imaging did not show anything out of the ordinary. After the same patient presented with extreme weakness in the same arm, a Washington University pediatric neurologist was able to diagnose the child with PTS by using a nerve conduction study and electromyogram to identify nerve inflammation in multiple areas of the brachial plexus. The neurologist was able to quickly refer the patient to physical therapy at the Washington University and St. Louis Children’s Young Athlete Center, which aided in his nearly full recovery.
When an elementary-aged patient was referred to the Children’s Specialty Care Center-West County in St. Louis, Missouri, with onset of left shoulder pain, providers initially suspected that he had torn his rotator cuff. The patient was an active child, a competitive hockey player — but the family couldn’t pinpoint what might have caused an injury or pain. An X-ray revealed nothing out of the ordinary. An MRI was also normal. But the pain became unbearable.
When, a month later, the same patient was experiencing severe weakness in his left shoulder, tests confirmed it was something more rare: severe denervation of the suprascapular and axillary nerves in the left shoulder, suggestive of severe neuropathy of both nerves and consistent with an uncommon neurological condition called Parsonage-Turner Syndrome (PTS) — almost unheard of in pediatric patients.
PTS, also referred to as idiopathic brachial plexopathy or neuralgic amyotrophy, is estimated to occur in 1 to 3 people out of every 100,000 individuals each year, but because it is often not diagnosed or misdiagnosed, the incidence is difficult to determine.
The condition presents with:
Abrupt onset of shoulder pain, usually unilaterally
Progressive motor weakness
Dysesthesia
Numbness
The cause of PTS is not well understood, but it has been observed in the following scenarios:
Postoperatively
Post infection
Post traumatic event
Postvaccination
Although information on PTS in pediatrics is scarce and the condition is difficult to diagnose in its acute stage, recovery can be favorable, additional testing limited and surgical exploration avoided if the diagnosis is made early. Doing so, however, requires a combination of expertise as well as care that straddles neurology, orthopedics, and physical therapy.
Three things tipped off Sheel Pathak, MD, a Washington University pediatric neurologist at St. Louis Children’s Hospital, that the patient might be experiencing a neurological problem with his brachial plexus rather than just an orthopedic issue. The first two were the profound weakness and asymmetry of his scapula. The third was a detail from the patient’s history: He hadn’t experienced a hockey injury, but he had reported pulling a heavy object — a large bag — out of a car, stretching his shoulder and neck, before the pain's onset.
Pathak suspected that the patient was experiencing nerve damage and ordered both a nerve conduction study and an electromyogram. The results of the testing showed nerve inflammation in multiple areas of the brachial plexus. Knowing the patient’s story, Pathak pointed to PTS as the cause.
“Trauma is one of the causes of that, and he had yanked on that bag and hurt himself, so we thought that was the mechanism,” he says. “He had a story that made sense and electrodiagnostic studies that supported an inflammatory process to the brachial plexus.”
Pathak knew the next step in treatment was physical therapy and referred the patient to the Washington University and St. Louis Children’s Young Athlete Center, conveniently located within the Children’s Specialty Care Center.
When the patient began physical therapy for PTS at the Young Athlete Center, he was only able to actively raise his left arm to 70 degrees of shoulder flexion and 40 degrees of shoulder abduction; normal ranges are 180 degrees for both motions. The patient only had trace contraction of all rotator cuff muscles and was severely limited in his ability to perform any overhead activity.
Although PTS is rare in pediatric patients, Gabrielle Griffin, PT, DPT, the physical therapist at the Young Athlete Center who treated the patient, was able to find a case study similar to the patient’s case that outlined neuromuscular electric stimulation (NMES) protocol to activate and strengthen the patient’s muscle. She paired the protocol with progressive rotator cuff strengthening and saw the patient for seven months, first twice a week before moving to once a week.
When the patient started physical therapy, he had barely any activation of the rotator cuff muscles and had trace muscle function in some areas. Attempting to move through a full range of motion, then, was not an option if he could not activate his muscles in a gravity-lessened position. Instead, Griffin focused on early strengthening isometrics, such as pressing and holding a shoulder into a wall, working on activating muscle fibers. From there, therapy was a gradual progression of moving those muscles against gravity and adding resistance.
One challenging aspect of the patient’s care was that there was no indication of how long the weakness would last. During therapy, the patient was unable to play hockey or even sports at school during recess. Unsure of how long his symptoms would last, if he would make a full recovery or if he would play sports again, he struggled emotionally with his condition.
“His mom did pull me aside and tell me that she was noticing some signs of depression because he couldn’t play hockey,” Griffin says.
Griffin addressed the patient’s morale by implementing positive psychological coaching strategies to motivate the patient. Each week, she would point out progress, be it gaining even 2 to 3 degrees of motion.
“In our sessions, we kept it as fun as possible,” Griffin says. “He got to pick out music to play and however I could incorporate hockey into the sessions, we did that.” The Young Athlete Center had space to accommodate a hockey goal, and the patient was able to practice taking shots, a taste of his former activity that spurred him on. On his last day of therapy, he had a nearly full range of motion and very close to manual muscle testing strength in all planes. The patient not only returned to playing competitive hockey, but he also moved up to a more advanced team.
A year after the patient entered care within St. Louis Children’s Hospital, the Washington University neurology team found he only had mild weakness of shoulder rotation on his left side, an excellent outcome thanks in part to a speedy diagnosis and access to high-quality physical therapy. “Identifying a good pattern of this, being able to say, ‘Yes, this is Parsonage-Turner Syndrome’ based on what we see on the electrodiagnostic studies helps prevent some diagnostic odyssey, more tests and unnecessary treatments,” Pathak says. “We were able to identify it and treat him so he could get back to doing what he loves.”
https://www.doximity.com/doc_news/v2/sponsored_entries/aHR0cHM6Ly9jYW1wYWlnbnMuZG94aW1pdHkuY29tL2FwaS92Mi9kZWxpdmVyaWVzLzg4M2Q5M2EyLTVhNDAtNDQ2NS1hOTA2LWRiMTQyYzU1Y2QyZj9jaGFubmVsPW5ld3NmZWVk?channel=newsfeed
See: https://issuu.com/st.louischildrenshospital/docs/slch_foundationmagazine_fall2022_web
https://www.stlouischildrens.org/health-resources/pulse/solving-medical-mystery-takes-teamwork
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