Parsonage Turner Syndrome
Parsonage Turner Syndrome (PTS), or neuralgic amyotrophy, is a rare disorder that may affect the nerves controlling the shoulder, arm, or hand. The onset of this disorder is usually characterized by the sudden onset of severe pain in the shoulder and upper arm. This is usually followed in a day or more by weakness. While the pain may begin to resolve over the next few weeks, the weakness may take much longer to recover. In some cases, the weakness is so severe that complete recovery is not possible.
PTS affects the brachial plexus — the network of nerves that sends signals from your spine to your shoulder, arm, and hand. PTS occurs when those nerves become inflamed. Typically, the inflammation causes sudden pain, followed by weakness and often numbness as well, though usually less dramatic. The severity of an attack can range from mild to severe. Some people have symptoms only in the shoulder, while other cases involve the arm and hand. In fact, some cases just the forearm and hand, though this is less common.
Causes of Parsonage Turner Syndrome
In most cases, it is not possible to identify the cause of Parsonage Turner Syndrome. Attacks can be triggered by an any systemic stressor. In fact, surgery as well as labor and delivery in women are known triggers. Influenza, high levels of stress, or extreme exertion may also may lead to an attack of Parsonage Turner syndrome.
A speedy and accurate diagnosis of this disorder is critical. Because they often appear after surgery, symptoms of PTS have been mistakenly blamed on a surgical injury. But while a surgical injury is usually immediately apparent when a person wakes up from surgery, PTS symptoms typically develop hours or days later.
Symptoms Of Parsonage Turner Syndrome
The pain of PTS is often described as sharp, aching, burning, stabbing, or throbbing. In some cases, the pain may extend from the shoulder to the neck, lower arm, and/or hand on the affected side. Rarely, both sides of the body are involved. Affected people typically experience constant pain that may become worse with movement. Intense pain can last from a few days to several months, at which point the pain usually begins to subside.
Diagnosis And Treatment Of Parsonage Turner Syndrome
Diagnosing PTS involves an evaluation to establish the presence and location of a patchy process consistent with nerve inflammation. This may include nerve tests such as nerve conduction studies (NCS) and electromyography (EMG), along with imaging exams, such as magnetic resonance imaging (MRI). Occasionally blood tests for alternative causes of inflammation and nerve biopsies are employed. Because PTS is rare and complex, if a primary care physician suspects it, the best course of action is usually to get a referral to a Paralysis Specialist experienced in diagnosing and managing the disorder.
If symptoms are still active when the diagnosis is made, steroid treatment may be recommended to reduce the inflammation. PTS symptoms usually fade over time, but physical therapy may be necessary to regain complete range of motion. Recovery time depends on the severity of the attack, the areas of the body affected, and how quickly treatment began.
For example, in a case with mild symptoms only affecting the shoulder that was quickly diagnosed and treated, recovery could take six months. For a more severe case that involves the shoulder, arm, and hand, full recovery may never transpire, In that care, a paralysis expert should follow you to determine whether recover will occur and, if not, to provide the reconstructive surgery necessary to restore the missing function.
Conditions/Weakness From Spinal Disorders (Spondylotic Amyotrophy)
Pinched nerves are a common occurrence and thousands of people undergo surgery annually due to pain from pinched nerves due to narrowing of the spine or a herniated disk. Rarely these same spinal disorders can result in progressive weakness with or without pain.
Sometimes it is in a single nerve root (C5 nerve root with shoulder weakness or L5 nerve root with foot drop) whereas at other times the entire region of the spine has developed slowly progressive narrowing resulting in progressive weakness of the entire limb. When this begins it may have typical pain which then resolves or it may have no pain at all. Sensory loss is also variable. Because this condition is typically slowly progressive, it may be difficult to tell when it actually began.
It is common to hear someone describe that the hand has been weak for a whole but over the past 6 months it has really progressed. It is important to distinguish this from a number of other sources of weakness including motor neuron disease, Parsonage Turner Syndrome, or other polyneuropathies.
Workup typically includes an MRI to look at the spinal nerve roots, a CT scan to look at the bony foramen at the levels of interest, and an EMG to deternine how much of the weakness is recent (with associated spontaneous activity) and how severely denervated the weak muscles are.
We will also try to determine if there is an associated neurapraxia where the muscle responds well to stimulation but the patient cannot activate it well. This finding is very promising that surgery will result in significant gains in strength.
When it is determined that the spine is the source, the first step is to halt the progression by stabilizing and decompressing the spine. This can also lead to recovery if caught early and if (as discussed above) a neurapraxia accounts for a significant portion of the weakness.
Once the progression is halted and we have achieved the gains that can be attained through that operation, it is time to develop the reconstructive plan. Because these are typically lower motor neuron disorders and, most often, they have progressed slowly over years, nerve transfers may not be an option.
Instead tendon transfers may be more useful in many of these cases. Free muscle transplants are also of value in some cases. When we do catch it early, decompression may lead a significant recovery. If it does not rapidly recover and we are still within the window for nerve transfers, that option can be pursued as well.
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Before your visit, write down questions you want answered.
Bring someone with you to help you ask questions and remember what your Specialist tells you.
At the visit, write down the name of your diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your specialist gives you.
Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.
Ask if your condition can be treated in other ways.
Know why a test or procedure is recommended and what the results could mean.
Know what to expect if you do not take the medicine or have the test or procedure.
If you have a follow-up appointment, write down the date, time, and purpose for that visit.
Know how you can contact your Paralysis Specialist if you have questions.