Personage Turner syndrome is a lesion of nerve trunks and branches of the brachial plexus of unclear etiology, characterized by attacks of neuropathic pain, rapid multi—focal paresis and atrophy of the muscles of the upper extremities with a long recovery period. Diagnostics is carried out taking into account the data of neurological status, laboratory, electrophysiological, radiological, tomographic studies. Conservative treatment is carried out with glucocorticoids, nonsteroidal anti-inflammatory drugs, intravenous immunoglobulins. If drug therapy is ineffective, surgical methods may be used.
ICD 10
G54.5 Neuralgic amyotrophy
General information
Personage Turner syndrome belongs to the group of neural amyotrophy. The first mention of pathology dates back to 1887 .. A detailed description was made in 1948 by the British Maurice Personage and John Turner based on the results of observation of 136 clinical cases. Synonymous names of the disease are “idiopathic brachial neuritis”, “neuralgic amyotrophy”, “scapular girdle syndrome”. The annual incidence rate is 1 case per 1000 people. Men dominate among the sick, according to various data, they get sick 2-10 times more often than women. Interest in the disease has increased due to the appearance of cases induced by the coronavirus and the COVID-19 vaccine.
Causes
Etiological factors continue to be studied. Hereditary forms of pathology and spontaneously emerging idiopathic variants have been identified. Among the main etiofactors that can lead to the emergence of Personage Turner syndrome, there are:
- Genetic disorders. A family history testifies in favor of the hereditary nature of the disease. In 50% of patients, point mutations of the SEPT9 gene located on chromosome 17q25 were detected.
- Immunological factors. More than 50% of patients have a history of an event that activates the immune system, such as infection, vaccination, surgery, pregnancy, stress. Infectious causes include smallpox, coxsackie viruses, cytomegalovirus, coronavirus, parvovirus, HIV.
- Mechanical loads. Sports activities, work associated with heavy physical activity can provoke the development of the syndrome. In studies, about 10% of patients reported previous intense mechanical stress on the shoulder girdle and upper limb.
Pathogenesis
Hereditary genetic mutations cause disturbances in the synthesis of guanosine 5-triphosphate-binding protein involved in the formation of the cytoskeleton of glial cells forming the myelin sheath of peripheral nerve trunks. The pathological structure of the latter makes the nerve susceptible to inflammatory changes under the influence of external triggers, causing a recurrence of clinical symptoms.
Among the pathogenetic theories of spontaneous occurrence of Personage-Turner amyotrophy, the immunological one is the most recognized. According to this theory, variable immunological triggers cause immune hyperactivation, leading to inflammatory processes. There is evidence of an increase in the level of antibodies to peripheral nerve myelin.
The immunological theory was confirmed in a number of studies that revealed epineural perivascular T-cell infiltrates in biopsies of affected nerves. CD8+ T lymphocytes, CD68+ macrophages and CD20+ B lymphocytes were also found in the endothelium of the vessels surrounding the involved nerve trunks. At the same time, mechanical factors have a provoking effect, trigger the mechanism of immune inflammation.
Classification
According to modern ideas about the etiology and pathogenesis of Personage Turner syndrome, there are 2 main variants of the disease. Their clinical picture does not differ much, understanding the differences in the mechanism of occurrence is necessary for successful treatment and determining the prognosis:
- Idiopathic form. Represents the majority of cases of neuralgic amyotrophy. It is characterized by a more benign course, the absence of relapses.
- Hereditary form. It occurs about 10 times less often than idiopathic. Inherited autosomal dominant. It is characterized by the occurrence of relapses of acute or subacute inflammation.
Symptoms
Personage Turner syndrome more often affects one limb, 30-35% of patients have bilateral asymmetric lesion. In 96% of cases, the disease debuts with acute pain in the shoulder girdle, radiating into the neck, shoulder, anterior surface of the chest, forearm. A small proportion of patients experience pain only in a limited area, for example, in the neck, shoulder blade or shoulder.
In about 60% of cases, seizures begin at night. Patients wake up early in the morning with severe pain, which then reaches maximum intensity over the next few hours. The pain is usually “stabbing”, “throbbing”, “aching” in nature, increases with movements of the shoulder, hands. The duration of an attack of brachyalgia varies from several hours to 2 months, the average duration of the pain syndrome is 4 weeks.
In about 5% of patients, the pain goes away within 24 hours, in 10% of patients, the pain persists for more than 2 months. A few days or weeks after the manifestation of symptoms, muscle weakness occurs, progressing as the pain subsides. Typically, the defeat of the biceps and triceps of the shoulder, the anterior dentate, supraspinatus, deltoid, subacute muscles.
Most patients have sensory disorders in the form of hypesthesia and / or paresthesia of the lateral surface of the shoulder, the area above the deltoid muscle. However, in the first place in the clinical symptoms comes muscle weakness, which makes it difficult to move the upper limb. The defeat of the long thoracic nerve with paresis of the anterior dentate muscle leads to the separation of the lower angle of the scapula from the chest.
Complications
In general, Personage Turner syndrome has a benign course. However, severe paresis with muscle strength less than 3 points leads to its incomplete recovery after the main symptoms subside. The development of muscular atrophy is accompanied by a persistent loss of normal motor function. Another complication of the disease is the recurrence of the inflammatory process with the resumption of a painful episode, which is more typical for the hereditary variant of neuralgic amyotrophy.
Diagnostics
The diagnosis of Personage Turner syndrome is established on the basis of anamnesis and examination by a neurologist after excluding another possible pathology. In the anamnesis, attention is paid to the familial nature of the case, diseases and mechanical factors preceding the onset of symptoms. The general examination determines the restriction of movements in one or both hands, the position of the forced reduction of the shoulder and the bend in the elbow joint. In 20% of cases, a pterygoid scapula is detected. To confirm the diagnosis , the following examinations are performed:
- Neurological examination. Allows you to identify and determine the degree of paresis of the muscles of the shoulder girdle and upper extremities. Violations of surface sensitivity are diagnosed in the area of the deltoid muscle, the outer surface of the shoulder. Deep sensitivity is preserved.
- Laboratory tests. They include a general blood test, blood culture to exclude the infectious process. Rheum tests are necessary for differential diagnosis with connective tissue diseases.
- Electromyography. It allows you to localize the site of nerve damage, assess the degree of damage, the dynamics of the process, and the effectiveness of treatment. It reveals a decrease in the amplitude of the M-response, fibrillation in the muscles that are innervated by the affected nerve fibers.
- Electroneurography. There is a decrease in the amplitudes of the passage of action potentials in the trunks and branches of the affected motor nerves. Conduction disturbances along sensory nerves are observed in 30-45% of cases.
- Radiography. It is performed if necessary to differentiate the syndrome from the pathology of the spine and shoulder joint. In Personage Turner syndrome, only age-related changes are diagnosed.
- Magnetic resonance imaging. MRI of the spine is performed to exclude radiculopathy caused by a herniated disc. On soft tissue MRI with gadolinium amplification, areas of high signal intensity corresponding to inflammation in nerve fibers are visualized.
Differential diagnosis
Personage-Turner disease must be differentiated from diseases of the shoulder joint, plexitis, pathology of the cervical spine, which have similar symptoms. The main diseases requiring exclusion include:
- shoulder plexitis;
- subacromial bursitis;
- cervical radiculopathy;
- compression neuropathy;
- rupture of the rotator cuff of the shoulder.
A distinctive feature of the Personage Turner syndrome is an increase in paresis with a decrease in the intensity of brachyalgia, the absence of radiological and tomographic changes in the spine and shoulder joint.
Treatment
Conservative therapy
In the acute period, immobilization is recommended for patients to minimize pain. In order to stop inflammatory changes and pain syndrome, active pharmacotherapy is carried out. Subsequently, rehabilitation measures are needed to restore muscle strength, including massage, individually selected therapeutic gymnastics. In the course of therapy of Personage Turner disease in practical neurology, the following drugs are used:
- Nonsteroidal anti-inflammatory drugs. They are used as maintenance therapy. They allow to stop the inflammatory process, reduce the intensity of pain. Functional outcomes of the disease do not significantly affect.
- Corticosteroids. Glucocorticoid pulse therapy for 2 weeks accelerates pain relief, reduces the recovery time of the nerve, increases the chances of recovery. The use of corticosteroids does not prevent the occurrence of muscle atrophy.
- Immunoglobulins. They are prescribed intravenously in combination with corticosteroids. According to clinical studies, such a treatment regimen can reduce the duration of the acute period, improve the results of treatment.
Surgical treatment
Since many patients with Personage Turner syndrome have spontaneous resolution of the disease, surgical interventions are indicated in the absence of clinical signs of recovery within 3 months. Magnetic resonance neurography is performed beforehand. When a narrowing of the nerve trunk is detected, the question of the expediency of the operation is considered. Surgical treatment options include:
- Intrafascicular neurolysis. It is indicated for nerve thinning of less than 75% according to tomography. The operation is aimed at excision of pathologically altered tissues and creation of optimal conditions for nerve regeneration.
- Neuroraphy. It is performed when the diameter of the nerve decreases by more than 75%. Surgical intervention consists in excision of the site of narrowing and stitching of the ends, which can be a stimulus for nerve restoration.
Prognosis and prevention
The outcome of the Personage Turner syndrome depends on the degree of development of collateral innervation. In most cases, spontaneous recovery occurs. As a rule, patients recover to 80-90% of their previous condition in 2-3 years, but more than 70% of patients have residual motor weakness.
If the amplitude of complex motor potentials during electromyography is reduced by more than 70%, or there is an initial paresis of ≤ 3 degrees on the scale of muscle strength, collateral reinnervation will be incomplete, and the prognosis for the restoration of motor function will be unfavorable. Since the etiology of the Personage-Turner disease is definitively unclear, specific preventive measures have not been developed.