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NORD is very grateful to Nigel L. Ashworth, MD, Division Physical Medicine and Rehabilitation, University of Alberta, Canada, for assistance in the preparation of this report.
Parsonage-Turner syndrome (PTS) is an uncommon neurological disorder characterized by rapid onset of severe pain in the shoulder and arm. This acute phase may last for a few hours to a few weeks and is followed by wasting and weakness of the muscles (amyotrophy) in the affected areas. PTS involves the brachial plexus, the networks of nerves that extend from the spine through the neck, into each armpit and down the arms. These nerves control movements and sensations in the shoulders, arms, elbows, hands, and wrists. The exact cause of PTS is unknown, but it is believed to be caused by an abnormality of the immune system (immune-mediated disorder). The severity of the disorder can vary widely from one individual to another due, in part, to the specific nerves involved. Affected individuals may recover without treatment, meaning that strength returns to the affected muscles and pain goes away. However, individuals may experience recurrent episodes. Some affected individuals may experience residual pain and potentially significant disability.
The initial descriptions of this disorder in the medical literature date back to the late 1800s. In 1948, Drs. Parsonage and Turner were the first physicians to describe a large series of patients. They termed the disorder ‘amyotrophic neuralgia’. There is an extremely rare, inherited form known as hereditary neuralgic amyotrophy, on which NORD has a separate report. Sometimes PTS is referred to as idiopathic neuralgic amyotrophy to distinguish it from the genetic form and to denote that the cause is unknown. However, usually PTS is simply referred to as neuralgic amyotrophy. PTS can be broadly classified as a form of peripheral neuropathy or disorder of the peripheral nervous system, which encompasses any disorder that primarily affects the nerves outside the central nervous system (i.e. brain and spinal cord).
The hallmark finding of PTS is the abrupt onset of pain in one of the shoulders (unilateral pain). In rare cases, both shoulders can be affected (bilateral). Onset may be rapid in some cases, while in others pain onset is gradual and subtle (insidious), followed by a rapid increase in both intensity and severity. Pain has been described as sharp, aching, burning or stabbing. Pain may also affect the neck and the arm and hand on the same side as the affected shoulder.
Again, it is important to note that PTS is a highly variable disorder and one person’s individual case may not resemble another person’s experience. Some individuals (approximately 75%) will only experience one episode, while others (approximately 25%) will experience recurrent episodes. In addition, recurrent episodes may involve the same peripheral nerves that were originally affected, completely different peripheral nerves, or a mix of the same and different peripheral nerves. The intensity, duration and location of pain can also differ greatly from one person to another.
Pain is often continuous, severe, and worse during the evening or at night. Pain can potentially be excruciating and debilitating. This initial period may be known as the acute phase. Eventually, affected individuals enter a period where the continuous pain lessens and there may be no pain when the affected shoulder and/or arm are not being used (i.e. at rest). However, specific movements may aggravate the condition, causing sharp, stabbing intense pain that persists for a few hours before lessening. This occurs because previously damaged nerves remain abnormally sensitive (hypersensitive). Eventually, affected individuals develop a chronic, low grade pain that can persist for a year or longer, sometimes known as the chronic phase.
Eventually, anywhere from a few days to a few weeks after the onset of the disorder, pain is replaced by progressive weakness of muscles in the affected shoulder. The severity of muscle weakness can vary greatly, ranging from mild weakness that may be barely noticeable to, in rare cases, almost complete paralysis of the affected muscles. In individuals with PTS, muscle weakness results from damage to the nerves that serve the muscles in the shoulders and arms. The degree of weakness is related to the number of nerves affected. In addition to weakness, the affected muscles may progressively shrink and thin (atrophy) due to lack of use. Muscle weakness may go unnoticed at first, until atrophy and wasting of the affected muscles progresses and is easy to observe.
Additional symptoms include absent or reduced reflexes and sensory deficits in the affected areas such as the loss of sensation or numbness (hypoesthesia), a sensation of tickling, prickling, or burning on the skin of the affected areas (paresthesia), or an abnormally unpleasant or painful sensation to a light touch (dysesthesia).
Additional complications can develop in some cases. The position of the shoulders, arms, wrists, and hands can shift slightly because of atrophy and weakness of affected muscles. This can leave an affected individual at risk of secondary impingement or subluxation. Secondary impingement is a painful condition that occurs when the shoulder’s tendons are compressed or trapped during shoulder movements. Subluxation refers to partial dislocation of the shoulder joint. Affected individuals may also be at risk of developing contractures, in which abnormal shortening of muscles or tendons leads to deformity or rigidity of an affected joint. Contracture of the shoulder, also known as adhesive capsulitis, can result in pain and limitation of the normal range of movement of the joint.
In some cases, nerves outside of the brachial plexus may be involved such as the nerves of the lumbosacral plexus, the phrenic nerve, or the recurrent laryngeal nerve. Involvement of the nerves in the lower portion of the back (lumbosacral plexus) can cause pain, hypoesthesia, and paresthesia in the legs. The phrenic nerve sends signals between the brain and the diaphragm, which is the muscle that separates the lungs from the abdomen. Involvement of the phrenic nerve can result in a significant shortness of breath. Involvement of the recurrent laryngeal nerve can result in weakness and partial paralysis of the vocal cords and, consequently, hoarseness and soft speech (hypophonia). In extremely rare cases, facial or other cranial nerves may be affected.
Because nerve damage in PTS can affect blood vessels additional symptoms may develop including affected skin, particularly on the hands, becoming reddened, purplish or spotted. Swelling due to fluid retention (edema) may also occur. The skin, hair and nails may grow more quickly than normal. Certain areas of the body particularly the hands and forearms may no longer be able to respond properly to outside temperature. Excessive sweating may occur or affected individuals may feel abnormally cold in the affected areas.
Some individuals may recover full strength and functional levels of the shoulder or affected areas. Numerous reports in the medical literature state that most individuals will regain up to 70-90% of their original strength and functional levels within two years. However, recent studies indicate that this can take more than two years in some people, while other people will experience residual, chronic pain and complications such as impaired movement of the shoulder and/or affected joints. In severe cases, affected individuals can be left with significant disability that can impact their ability to work and perform common tasks.
The exact, underlying cause of PTS is not fully understood. Different factors, including immunologic, environmental, and genetic ones, are thought to play a role in the development of the disorder.
Researchers believe that most cases result from an immune-mediated inflammatory response to some infection or environmental trigger that damages the nerves of the brachial plexus. A recent viral illness is the most common ‘triggering’ factor associated with the development of the disorder. Additional triggers that have been linked to PTS are recent immunization, surgery on the brachial plexus, unaccustomed strenuous exercise, minor trauma, bacterial infection, parasitic infection, anesthesia, rheumatologic diseases such as connective tissue disorders, and autoimmune disorders such as lupus, temporal arteritis, or polyarteritis nodosa. In women, childbirth can trigger PTS. In some cases, no triggering event or underlying factor can be identified.
Some researchers suspect that certain affected individuals are genetically predisposed to developing PTS (i.e. injury to the brachial plexus) following such exposures described above. A person who is genetically predisposed to a disorder carries a gene (or genes) for the disease, but it may not be expressed unless it is triggered or "activated" under certain circumstances, such as due to particular environmental or immunologic factors.
PTS affects males more often than females. The incidence of the disorder is based upon available population studies and is estimated to be approximately 1.64 to 3.00 people per every 100,000 individuals in the general population per year. However, cases of PTS may go undiagnosed or misdiagnosed, making it difficult to determine the true frequency in the general population. The condition develops most often in young to middle-aged adults, but has been reported in young children and the elderly.
Symptoms of the following disorders can be similar to those of PTS. Comparisons may be useful for a differential diagnosis.
PTS is only one cause of acute peripheral neuropathy. Diagnosis requires the exclusion of other causes including neuropathy caused by alcohol, poisons, drugs, inflammation of the blood vessels (vasculitis), and cancer. Additional conditions or disorders that can cause symptoms similar to those seen in PTS include bursitis, rotator cuff injury or disease, calcific tendonitis, impingement syndromes, Guillain-Barre syndrome, cervical disc disease, cervical radiculopathy, mononeuritis monoplex, amyotrophic lateral sclerosis (Lou Gehrig’s disease), chronic inflammatory demyelinating polyneuropathy, polymyalgica rheumatica, thoracic outlet syndrome, and brachial plexus injury secondary to cancer (neoplastic brachial plexopathy). Adhesive capsulitis, which can develop as a complication of PTS, can develop for other reasons as well. NORD has individual reports on some of these disorders and a general report on peripheral neuropathy. (For more information, choose the specific disorder name as your search term in the Rare Disease Database.)
A diagnosis of PTS is based upon identification of characteristic symptoms, a detailed patient history, a thorough clinical evaluation and a variety of specialized tests.
Clinical Testing and Workup
Certain tests such as nerve conduction studies or electromyography can be used to assess the health of muscles and the nerves that control muscles. Nerve conduction studies determine the ability of specific nerves in the peripheral nervous system to relay nerve impulses to the brain. During a nerve conduction study, electrodes are placed over specific nerves such as those of the shoulders and arms. The electrodes stimulate the nerves and record the conduction of the signal. This test can help to pinpoint the site of disease or injury to the nerve.
During an electromyography, a needle electrode is inserted through the skin into an affected muscle. The electrode records the electrical activity of the muscle. This record shows how well a muscle responds to nerves and can determine whether muscle weakness is caused by the muscles themselves or by the nerves that control those muscles.
A specialized imaging technique known as magnetic resonance imaging (MRI) can help to obtain a diagnosis of PTS. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues. An MRI can help to exclude other potential cause of shoulder pain, demonstrate atrophy of affected muscles, and detect signal changes caused by lack of nerve supply (denervation).
A traditional x-ray (radiograph) of the shoulder may be ordered to rule out specific conditions that can damage the shoulder.
There is no specific treatment for PTS. Treatment is directed toward the specific symptoms that are apparent in each individual. Treatment may require the coordinated efforts of a team of specialists. Orthopedic surgeons, neurologists, neuromuscular disease specialists, pediatricians or general internists, and other healthcare professionals may need to systematically and comprehensively plan an affect individual’s treatment. Early diagnosis and prompt treatment are important to increase the chances of a full recovery.
In some cases, PTS may resolve on its own without treatment and only require support measures such as various pain management strategies including pain medications (analgesics). Specific pain medications used to treat PTS include opiates and non-steroidal anti-inflammatory drugs (NSAIDs), which are usually used in combination. Some physicians have recommended using oral corticosteroids such as prednisone, which has led to a decreased duration of pain and accelerated the healing process in some cases. However, corticosteroids have proven ineffective for many individuals and are potentially associated with adverse side effects. Consequently, other physicians do not recommend their use.
After the acute phase, different medications known as co-analgesics may be administered. Such medications include gabapentin, carbamazepine, and amitryptiline. These drugs specifically treat nerve pain.
Physical and rehabilitation therapy are also used to treat individuals with PTS in order to preserve muscle strength and range of motion of affected joints. Specific techniques include active and passive range of motion exercises to help to prevent muscle atrophy and contractures. In most cases, muscle strengthening exercises cannot be used during the acute phase because they worsen pain.
Other techniques used to treat individuals with PTS include the application of heat or cold or transcutaneous electrical nerve stimulation (TENS), a procedure during which electrical impulses are sent through the skin to help to control pain by altering or blocking nerve transmissions.
Surgery may be considered in individuals who, after two years, have not responded favorably to other, less aggressive treatments. Surgical techniques can include nerve grafting or tendon transfers. Nerve grafting involves taking a segment of nerve tissue from one part of the body and using it to repair damaged nerves in another part of the body. Tendon transfer surgery is indicated when muscle function is lost because of nerve injury. During this procedure, a healthy tendon is removed from one part of the body and used to replace damaged or diseased tendon in another part of the body. These surgeries restore movement and function to the shoulder muscles and joint.
The prognosis for PTS varies greatly. Some individuals only experience one episode and fully recover their strength and functional level in the shoulder and other affected areas. According to older medical literature, most affected individuals will recover up to 70-90% of their original strength and functional level. However, more recent medical articles suggest that residual complications are more common than previously believed. There is approximately a 5-26% risk that PTS will recur after initially resolving or being effectively treated. In approximately 10-20% of cases, affected individuals may be left with residual persistent pain and decreased endurance or exercise intolerance in the affected shoulder. There have been cases reported in which affected individuals experience significant disability that can impact quality of life by making basic household tasks or work extremely difficult. For example, some individuals may have difficulty reaching or lifting. Other people may have difficulty with repetitive tasks that involve the shoulder or arm. Some individuals may develop a winged scapula, a condition in which the shoulder blade (scapula) protrudes or sticks out abnormally from the back. Individuals who experience repeated episodes are more likely to develop long-term complications.
Researchers are studying the use of intravenous immune globulin (IVIG) therapy to treat individuals with PTS. Immune globulins are specialized proteins known as antibodies that are found in blood and other bodily fluids of animals. Antibodies are used by the immune system to fight foreign substances like bacteria and viruses. More research is necessary to determine the long-term safety and effectiveness of IVIG therapy for individuals with PTS.
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Report last updated: 2014/05/07 00:00:00 GMT+0