Neuralgic shoulder amyotrophy (neuritis brachialis or Parsonage-Turner syndrome) is a rare neurologic disorder. It was first mentioned in the 1940s. The disease is caused by an acute inflammation of the brachial plexus. A cause for the development of that inflammation has not yet been found.
What is neuralgic shoulder amyotrophy?
Vaccinations, viral infections, surgery, injury, or vigorous exercise usually precede the condition. In most patients, however, no tangible cause can be found. Hallmarks of neuralgic shoulder amyotrophy are severe pain in the shoulder and arm muscles, which can culminate in paralysis. These symptoms have similarities to those of cervical disc herniation. Appropriate testing can rule out a differential diagnosis. Neuralgic shoulder amyotrophy predominantly affects men and most commonly occurs between the ages of 20 and 60. It is rarely hereditary and in this case usually begins in the first or second decade of life. The incidence is estimated to be 1.64 per 100,000 persons annually. The majority of cases involve the right arm, although it can occur equally on both sides.
Causes
The cause of neuralgic shoulder amyotrophy is unknown. However, an association with Coxsackie virus and cytomegalovirus has been found. Furthermore, vaccinations, physical exertion and heroin addiction are suspected to favor the development of the disease. As a result of the factors mentioned, inflammation of the sheaths of the brachial plexus occurs. This leads to faulty impulse transmission, which in turn causes severe pain and paralysis. The nerve is damaged. Muscle fibers can therefore no longer be controlled properly and become narrower. If the inflammation subsides, the sheaths are formed again. Their regeneration is a lengthy process that can take up to a year. This is due to the slow renewal of damaged nerve components. Other scientists consider circulating immune complexes to be the cause of plexus damage. These are protein compounds found in the blood. Genetic variants are rare, although they should be considered when investigated.
Symptoms, complaints, and signs
Neuralgic shoulder amyotrophy begins with severe pain in the upper arm and shoulder area. This is difficult for many sufferers to tolerate and lasts for several days to weeks. It is a pain that is equally present during movement and at rest. When it subsides, the affected arm becomes weak. Mild paralysis of the shoulder and upper arm muscles is the result. However, these can lead to complete arm plexus paresis (paralysis). Due to the inflammation, the patient finds it difficult or impossible to lift the inflamed arm. Affected muscles include deltoid (deltoid muscle), supraspinatus (superior spinae muscle), infraspinatus (inferior spinae muscle), serratus anterior (anterior saw muscle), and trapezius (trapezius muscle). The cervical plexus is usually not affected by inflammation. Inflammation of the diaphragm, biceps brachii muscle (biceps), individual nerves and nerve branches rarely occurs. Sensory disturbances in the arm and hand are also seen in a few patients. In most cases, atrophy (tissue loss) of the denervated muscles can be observed. A scapula alata that protrudes during movements is typical of neuralgic shoulder amyotrophy.
Diagnosis and course of the disease
The physician will first perform a history and neurologic examinations. Blood tests are atypical in this clinical picture. However, elevated antibodies to Coxsackie virus may be detected in the blood. A positive finding may indicate neuralgic shoulder amyotrophy. Nerve water studies are used to detect possible elevation of total protein. If a nerve fluid examination is positive, it may reflect cell damage or an increase in inflammatory cells.Because the symptoms of neuralgic shoulder amyotrophy are similar to those of cervical nerve root irritation (due to disc herniation or wear), misdiagnosis occasionally occurs. In this case, an electromyographic examination can lead to a definite finding. In this method, muscle activity is measured using concentric needle electrodes. If there is still doubt, a magnetic resonance imaging (MRI) scan is useful for detecting cervical disc herniation and cervical spine wear.
Complications
In cica 25 percent of all cases, neuralgic shoulder amyotrophy does not heal completely. Then the shoulder joint remains permanently paralyzed. The healing process depends on the development of the disease in the first two months. However, even if the healing process is ideal, it usually takes two years to become symptom-free. In neuralgic shoulder amyotrophy, capsular shrinkage occurs in the shoulder joint. Due to this, complications can occur, which initially manifest as paralysis. If the rotator cuff is paralyzed, there is then a constant risk of dislocation or subluxation. Luxation means complete dislocation of the shoulder joint, while subluxation means incomplete dislocation. To reduce the risk of dislocation, a bandage should be applied around the shoulder joint. Capsular shrinkage can be reduced with passive shoulder motion. However, even then there is no guarantee of healing without complications. So far, there is no proper treatment concept for this condition, so the patient needs a lot of patience. In addition to painkillers, cortisone is often used. However, this therapy is controversial because permanent cortisone treatment usually leads to severe side effects. The full picture of a Cushing’s syndrome can develop. In addition to truncal obesity with a full moon face and water retention in the body, there are then also psychological changes. But psychological problems can occur even without drug treatment because the healing process takes so long.
When should you go to the doctor?
Pain in the shoulder or upper arm is the first characteristic feature of neuralgic shoulder amyotrophy. If they do not arise from current overuse of the body, they must be monitored further. If the pain increases in intensity or persists unabated for several days, a visit to the doctor is necessary. Taking an analgesic medication should only be done in consultation with a doctor. Otherwise, further inconsistencies may occur. If there are sleep disturbances, an inner restlessness or if the general resilience decreases, a doctor is needed. If daily obligations can no longer be fulfilled or if the usual sporting activities can no longer be performed, consultation with a physician is advisable. If there are signs of paralysis, limitations of the musculoskeletal system, and poor posture of the upper body, the affected person needs medical assistance. Discomfort with twisting movements or lifting the arm is unusual and must be presented to a physician. Clarification of the cause is necessary to prevent further impairment or permanent damage to the organism. Sensitivity disorders, numbness of the skin or hypersensitivity to pressure as well as touching the affected areas of the body, should be examined by a physician. If the general quality of life decreases, or if behavioral abnormalities or mood swings occur due to the symptoms, the affected person should seek medical care.
Treatment and therapy
Treatment usually begins with the use of so-called opioids. These are centrally acting agents that reduce pain. After receiving a diagnosis, the abduction position promotes recovery. In this position, the arm is raised away from the body. In this way, the onset of stiffening of the shoulder joint capsule (more precisely, the rotator cuff) can be counteracted. Otherwise, this leads to luxation (dislocation) or subluxation (dislocation) of the shoulder joint. Bandaging becomes unavoidable. In most patients (75%), complete healing occurs within two years of the onset of the condition. Patience is essential. Physiotherapy is required to maintain mobility and strength of the arm and shoulder joint.It achieves good results in patients with neuralgic shoulder amyotrophy. Since the inflammation of the affected muscles must be brought to a halt, cortisone is often used. The use of this drug is controversial in this and numerous other conditions. When taken regularly, cortisone can cause psychological changes. Furthermore, it can cause stomach bleeding, muscle wasting, and other symptoms and illnesses. Rest and irradiation with red light, on the other hand, have no side effects. These measures are recommended by doctors and physiotherapists.
Outlook and prognosis
The prognosis is good for neuralgic shoulder amyotrophy. However, the course is extremely protracted, and the paralysis regresses only gradually within a few years. Thus, about half of those affected complain of pain in the arm area over a long period of time. In 80 to 90 percent of those affected, the disease heals almost completely within two years. Although minor residual symptoms may persist, overall bodily functions are usually normal and do not impair quality of life. The probability of a complete regression of the paralysis symptoms depends decisively on the healing process in the first months of treatment. If there is no improvement, future paralysis cannot be ruled out. In this case, physiotherapeutic exercises as well as targeted movement exercises under low stress are particularly important for the prognosis. These are particularly important in cases where muscle strength is permanently reduced by the disease. In some cases, certain movement sequences have to be relearned in order to ensure adequate strength development of the arms. In rare cases (less than 10 percent), recurrences – a reappearance of paralysis – can also occur.
Prevention
Currently, there are no known measures for prophylaxis.
Follow-up
Despite the limitations imposed by the clinical picture, affected individuals should pay close attention to aftercare. Sensible pain management combined with rehabilitation therapy provide the best conditions for the best possible recovery of the musculoskeletal system. Visits to a physiotherapist help sufferers learn to cope with the symptoms of the disease. Excessive strain on the shoulder/neck area, such as heavy lifting and prolonged monotonous performance of the arms, should be avoided. Targeted as well as controlled exercises, which are learned in physiotherapy and also followed at home, are an optimal supplement. If the arm and shoulder do not experience any movement in the long term, there is a risk of paralysis and muscle loss. Active and passive physiotherapy can be used to counteract this. Patients should consciously listen to themselves during follow-up care and clarify with the accompanying physician and physiotherapist what promises relief for the affected area. While some patients prefer cold therapy, others prefer local heat applications and radiation treatments. Also supportive is a change in diet with low-acid and low-fat foods, as well as abstaining from alcohol and drugs. Patience is the biggest challenge for sufferers of neuralgic shoulder amyotrophy, which can last up to two years in the worst cases. The more coordinated the daily, but not excessive, exercise to restore movement, the more successful the follow-up.
Here’s what you can do yourself
Sufferers of neuralgic shoulder amyotrophy should pay close attention to their body’s signals. Stressful situations or overexertion should be avoided. If an inflammatory disease is present, it should always be cured. The affected person harms himself if he exposes his body to too much strain before a complete recovery. In order for inflammations in the organism to heal better, a stable immune system is required. This can be supported by a healthy lifestyle as well as a balanced diet. Consumption of harmful substances such as nicotine and alcohol should be refrained from. Food should be rich in vitamins. The daily calorie requirement is to be determined according to the existing weight. Existing excess weight should be reduced as far as possible, as this puts additional strain on the muscles, bones and tendons. The disease is accompanied by severe pain in the area of the shoulders or arm. However, the patient should not take painkillers on his own responsibility.Rather, relaxation techniques or mental training help to support the mind. In this way, the sensation of pain can be influenced. In addition, incorrect postures or one-sided stresses on the body should be avoided. These trigger new complaints and lead to an overall deterioration in well-being. If permanent paralysis of the shoulder occurs, the affected person should seek emotional support. Otherwise, the risk of psychological sequelae increases.
