Parsonage-Turner Syndrome and Hereditary Brachial Plexus Neuropathy.

Parsonage-Turner syndrome and hereditary brachial plexus neuropathy (HBPN) present with indistinguishable attacks of rapid-onset severe shoulder and arm pain, disabling weakness, and early muscle atrophy. Their combined incidence ranges from 3 to 100 in 100,000 persons per year. Dominant mutations of SEPT9 are the only known mutations responsible for HBPN. Parsonage and Turner termed the disorder "brachial neuralgic amyotrophy," highlighting neuropathic pain and muscle atrophy. Modern electrodiagnostic and imaging testing assists the diagnosis in distinction from mimicking disorders. Shoulder and upper limb nerves outside the brachial plexus are commonly affected including the phrenic nerve where diaphragm ultrasound improves diagnosis. Magnetic resonance imaging can show multifocal T2 nerve and muscle hyperintensities with nerve hourglass swellings and constrictions identifiable also by ultrasound. An inflammatory immune component is suggested by nerve biopsies and associated infectious, immunization, trauma, surgery, and childbirth triggers. High-dose pulsed steroids assist initial pain control; however, weakness and subsequent pain are not clearly responsive to steroids and instead benefit from time, physical therapy, and non-narcotic pain medications. Recurrent attacks in HBPN are common and prophylactic steroids or intravenous immunoglobulin may reduce surgical- or childbirth-induced attacks. Rehabilitation focusing on restoring functional scapular mechanics, energy conservation, contracture prevention, and pain management are critical. Lifetime residual pain and weakness are rare with most making dramatic functional recovery. Tendon transfers can be used when recovery does not occur after 18 months. Early neurolysis and nerve grafts are controversial. This review provides an update including new diagnostic tools, new associations, and new interventions crossing multiple medical disciplines.

[Association between hematopoietic stem cell transplantation and Parsonage Turner syndrome: coincidence or cause-effect relationship?] / Asociación de trasplante de progenitores hematopoyéticos y síndrome de Parsonage-Turner: ¿coincidencia o relación causa-efecto?

Parsonage-Turner syndrome or idiopathic brachial neuritis is a total or partial inflammation of the brachial plexus, with a typical presentation as a sudden and very intense pain in the shoulder, followed by weakness and early amyotrophy. The etiology is still unknown, although an immune mediated mechanism is thought to be involved. Hematopoietic stem cell transplantation is a well-established treatment for hematological malignancies, but with a growing implication in the treatment of autoimmune diseases. The neurological side effects are probably underdiagnosed. The association of the Parsonage-Turner syndrome and the hematopoietic stem cell transplantation is scarce. We describe two clinical cases of idiopathic brachial plexopathy after hematopoietic stem cell transplantation. The reconstruction of the immune system after a transplant may be the trigger of a brachial plexopathy, but more studies are necessary for the etiology of this disease to be understood and to establish a cause-effect relation with the transplant.

[Diagnostics and treatment of hourglass-like nerve constrictions and torsions in neuralgic amyotrophy]. / Diagnostik und Therapie sanduhrförmiger Nervenstrikturen und -torsionen bei neuralgischer Amyotrophie.

Neuralgic amyotrophy is a disease of the peripheral nervous system characterized by severe neuropathic pain followed by peripheral paralysis. A distinction is made between a hereditary and an idiopathic form, which is assumed to have an autoimmunological origin. Conservative medicinal treatment mainly consists of nonsteroidal anti-inflammatory drugs (NSAID), opioids and glucocorticoids; however, despite treatment, symptoms in the form of pain or paralysis persist in over 50% of cases. Inflammation can lead to strictures and torsions of peripheral nerves, which can be visualized by imaging using nerve sonography or magnetic resonance (MR) neurography and confirmed intraoperatively during surgical exploration. Based on the currently available data, patients with strictures and torsions of peripheral nerves can benefit from neurosurgical treatment.

[Neuralgic amyotrophy: a common cause of unilateral and bilateral diaphragmatic pareses]. / Die neuralgische Amyotrophie: eine häufige Ursache uni- oder bilateraler Zwerchfellparesen.

There are several causes for unilateral or bilateral diaphragmatic paresis. The most common cause is an (intraoperative) injury to the phrenic nerve.However, in up to 20% of cases, no explanation can be found despite extensive workup. Neuralgic amyotrophy (NA, also known as Parsonage-Turner syndrome) is a common underdiagnosed multifocal autoimmune-inflammatory disease that predominantly affects proximal nerve segments of the upper extremities. Classic symptoms include acute onset of severe pain in the shoulder girdle with delayed onset of paresis of the shoulder and arm muscles. In at least 7% of cases, the phrenic nerve is also affected. Based on the annual incidence of NA of 1:1000, the entity as a cause of diaphragmatic dysfunction is probably not as uncommon as previously thought. However, clinical experience shows that this diagnosis is often not considered, and diaphragmatic paresis gets wrongly classified as idiopathic.This is particularly disastrous because in the early stage of NA, medical therapy with corticosteroids is mostly not considered and the possibility that surgical repair of the diaphragm may be performed prematurely, given that the condition may resolve spontaneously many months after symptom onset.The aim of the present article is to raise awareness of the entity of NA as a cause of diaphragmatic paresis and to establish a standardized approach to diagnosis and treatment.

Shoulder Pain and Dysfunction After Vaccination: A Systematic Review.

BACKGROUND: Persistent shoulder pain and dysfunction after vaccination are relatively rare but well-known complications after inoculations into the deltoid muscle. The term SIRVA (shoulder injury related to vaccine administration) is frequently used to encompass many of these occurrences; however, multiple distinct pathologies with similar presentations have been reported after vaccination. We performed a systematic review of the literature on vaccine-related shoulder injuries to help guide practitioners in appropriate workup and treatment based on specific diagnoses. METHODS: PubMed was used to search for combinations of multiple keywords (including vaccine, immunization, SIRVA, injury, inflammation, bursitis, Parsonage-Turner syndrome, and neuritis), and all references of each potential article were reviewed. A total of 56 articles were included. Patient demographics, vaccine information, presentation, diagnostic studies, treatment, and outcomes were recorded. RESULTS: Diagnoses were divided into 3 categories: (1) local inflammatory reaction (SIRVA), (2) brachial neuritis, and (3) direct nerve injury. The included articles reported on 57 cases of SIRVA, 18 of brachial neuritis, and 4 of direct nerve injury. The diagnoses reported for the SIRVA cases included frozen shoulder, pseudoseptic arthritis, subacromial bursitis, rotator cuff injury, and lytic lesions of the humeral head. Various treatments were used, and most patients had resolution of symptoms with conservative treatment including physical therapy, analgesics, and/or corticosteroid injections. Advanced imaging rarely provided information that affected treatment. The brachial neuritis and direct nerve injury cases were typically confirmed with electromyography/nerve conduction studies. Treatment of these 2 categories was nonoperative in all cases, typically with analgesics and/or corticosteroids, and most patients had symptomatic improvement after a few months, with most patients regaining strength. However, some (1 of 3 patients with brachial neuritis and >1 year of follow-up and 2 of 4 patients with direct injury) had residual weakness. CONCLUSIONS: Medical professionals should be aware of the various pathologies that can lead to prolonged shoulder pain after vaccination. Fortunately, most of these conditions can be treated successfully with nonoperative modalities, although differentiating among the diagnoses can help guide treatment, as some likely benefit from systemic corticosteroids or localized corticosteroid injections. Outcomes for most patients have been good, with the majority recovering without residual pain or deficits. LEVEL OF EVIDENCE: Prognostic Level IV.

Ultrasound Diagnostic and Physiotherapy Approach for a Patient with Parsonage-Turner Syndrome-A Case Report.

Parsonage-Turner syndrome (PTS) is a rare neurological disorder that causes major diagnostic problems. This paper presents a case report of a patient with PTS and proposes a new physiotherapy program. CASE DESCRIPTION: a 23-year-old man presents a sudden severe pain of his right arm. The man is consulted by several doctors and physiotherapists. Three magnetic resonance imagings (MRI), a nerve conduction study (NCS), and needle electromyography (EMG) are performed. After 6 months, based on medical history, physical examination and ultrasound imaging (UI), the physiotherapist suggests PTS, which is confirmed by a neurologist. INTERVENTION: due to the lack of physiotherapy treatment standards in PTS, we apply neurodynamic techniques. OUTCOMES: neurodynamic techniques are effective in reducing pain and paraesthesia, improving sensation, and reducing nerve swelling (assessed by UI), as well as improving manual dexterity and overall health status. CONCLUSIONS: the patient with PTS is challenging for making an accurate diagnosis. This study shows an important role for UI, which shows changes in the musculocutaneous nerve, despite the lack of abnormalities in the MRI, NCS, and EMG, and helps in making an accurate diagnosis. This report also confirms that physiotherapy based on neurodynamic techniques may have beneficial effects in PTS.

Parsonage-Turner Syndrome mimicking musculoskeletal shoulder pain: A case report during the SARS-CoV-2 pandemic era.

Parsonage-Turner Syndrome or neuralgic amyotrophy is a peripheral neuropathy typically characterized by an abrupt onset of pain, followed by progressive neurological deficits (e.g. weakness, atrophy, occasionally sensory abnormalities) that involve the upper limb, mainly the shoulder, encompassing an extensive spectrum of clinical manifestations, somehow difficult to recognize. This case report describes the proper management of a 35-year-old, bank employee and sports amateur who reported subtle and progressive upper limb disorder with previous history of neck pain. SARS-CoV-2 pandemic era made patient's access to the healthcare system more complicated. Nevertheless, proper management of knowledge, relevant aspects of telerehabilitation-based consultation for musculoskeletal pain, advanced skills, tools and technologies led the physiotherapist to suspect an atypical presentation of Parsonage-Turner Syndrome. Further, neurologist consultation and electromyography suggested signs of denervation in the serratus anterior and supraspinatus muscle. Therefore, an appropriate physiotherapist's screening for referral is conducted to correct diagnosis and thorough treatment.

COVID-19 atypical Parsonage-Turner syndrome: a case report.

BACKGROUND: Neurological manifestations of Sars-CoV-2 infection have been described since March 2020 and include both central and peripheral nervous system manifestations. Neurological symptoms, such as headache or persistent loss of smell and taste, have also been documented in COVID-19 long-haulers. Moreover, long lasting fatigue, mild cognitive impairment and sleep disorders appear to be frequent long term neurological manifestations after hospitalization due to COVID-19. Less is known in relation to peripheral nerve injury related to Sars-CoV-2 infection. CASE PRESENTATION: We report the case of a 47-year-old female presenting with a unilateral chest pain radiating to the left arm lasting for more than two months after recovery from Sars-CoV-2 infection. After referral to our post-acute outpatient service for COVID-19 long haulers, she was diagnosed with a unilateral, atypical, pure sensory brachial plexus neuritis potentially related to COVID-19, which occurred during the acute phase of a mild Sars-CoV-2 infection and persisted for months after resolution of the infection. CONCLUSIONS: We presented a case of atypical Parsonage-Turner syndrome potentially triggered by Sars-CoV-2 infection, with symptoms and repercussion lasting after viral clearance. A direct involvement of the virus remains uncertain, and the physiopathology is unclear. The treatment of COVID-19 and its long-term consequences represents a relatively new challenge for clinicians and health care providers. A multidisciplinary approach to following-up COVID-19 survivors is strongly advised.

[Neuralgic amyotrophy]. / Nevralgicheskaya amiotrofiya.

A review of the literature covering the issues of etiology, pathogenesis, variety of atypical forms, diagnosis and management of patients with neuralgic amyotrophy is presented.

Applying a clinical decision-making model to a patient with severe shoulder pain ultimately diagnosed as neuralgic amyotrophy.

Objective and Purpose Shoulder symptoms are often encountered in physical therapy and a myriad of etiologies can cause these symptoms, either locally or remotely. The purpose of this case report is to describe the physical therapist's differential diagnostic process for a patient with acute and severe onset of shoulder pain. Case Description: The patient was a 37-year-old female with sudden onset of right shoulder pain that awakened her at night. Pain was associated with decreased range of motion and shoulder weakness. Faced with an uncertain diagnosis, the physical therapist followed a systematic approach to clinical decision-making. Outcomes: Neuralgic amyotrophy was the primary diagnostic hypothesis but other causes of shoulder pain could not be ruled out. Conclusion: The clinical decision-making process helped the physical therapist narrow down the differential diagnosis list and make a decision to send the patient for further testing. Magnetic resonance imaging and electromyogram confirmed the diagnosis of neuralgic amyotrophy.

Neuralgic amyotrophy.

PURPOSE OF REVIEW: This review focuses on the current insights and developments in neuralgic amyotrophy (NA), an auto-immune multifocal peripheral nervous system disorder that leaves many patients permanently impaired if not recognized and treated properly. RECENT FINDINGS: NA is not as rare as previously thought. The phenotype is broad, and recent nerve imaging developments suggest that NA is the most common cause of acute anterior or posterior interosseous nerve palsy. Phrenic nerve involvement occurs in 8% of all NA patients, often with debilitating consequences. Acute phase treatment of NA with steroids or i.v. immunoglobulin may benefit patients. Long-term consequences are the rule, and persisting symptoms are mainly caused by a combination of decreased endurance in the affected nerves and an altered posture and movement pattern, not by the axonal damage itself. Patients benefit from specific rehabilitation treatment. For nerves that do not recover, surgery may be an option. SUMMARY: NA is not uncommon, and has a long-term impact on patients' well-being. Early immunomodulating treatment, and identifying phrenic neuropathy or complete nerve paralysis is important for optimal recovery. For persistent symptoms a specific treatment strategy aiming at regaining an energy balance and well-coordinated scapular movement are paramount.

Extended Neuralgic Amyotrophy Syndrome in a Confirmed COVID-19 Patient After Intensive Care Unit and Inpatient Rehabilitation Stay.

ABSTRACT: The cause of neuralgic amyotrophy is often unknown but is commonly associated with a recent upper respiratory viral tract infection. Since the beginning of the COVID-19 pandemic, there has been a tireless effort to understand the sequelae of the virus. A 46-yr-old woman who presented after a COVID-19 hospitalization complicated by hypoxic respiratory failure requiring intubation and mechanical ventilation for 23 days was subsequently found to have lower limb sensorium changes as well as upper limb weakness. Left shoulder abduction and extension were both 3/5 in motor strength, and left hip flexion strength was 4/5 with diminished sensation to crude touch in the left lateral thigh. Nerve conduction studies and electromyography findings included a mild left median neuropathy at the wrist and motor unit recruitment pattern consistent with a chronic left upper trunk plexopathy with reinnervation. The case presented describes an extended neuralgic amyotrophy syndrome from an atraumatic mechanism in a previously diagnosed COVID-19 patient. An extended neuralgic amyotrophy syndrome has at least three immune mediated etiologies postulated (1) direct neuropathogenicity, (2) molecular mimicry, and (3) direct cytotoxic effects on peripheral nerves. As COVID-19 survivors continue to be seen in outpatient settings, practitioners should remain aware of diffuse neurological complications as sequelae of the virus persist.

Outcomes of Microneurolysis of Hourglass Constrictions in Chronic Neuralgic Amyotrophy.

PURPOSE: Wide variability in the recovery of patients affected by neuralgic amyotrophy (NA) is recognized, with up to 30% experiencing residual motor deficits. Using magnetic resonance imaging and ultrasound (US), we identified hourglass constrictions (HGCs) in all affected nerves of patients with chronic motor paralysis from NA. We hypothesized that chronic NA patients undergoing microsurgical epineurolysis and perineurolysis of constrictions would experience greater recovery compared with patients managed nonsurgically. METHODS: We treated 24 patients with chronic motor palsy from NA and HGCs identified on magnetic resonance imaging and US either with microsurgical epineurolysis and perineurolysis of HGCs (11 of 24) or nonsurgically (13 of 24). Muscle strength (both groups) and electrodiagnostic testing (EDX) (operative group) was performed before and after surgery. Preoperative EDX confirmed muscle denervation in the distribution of affected nerve(s). All patients met criteria for microneurolysis: 12 months without improvement since onset or failure of clinical and EDX improvement after 6 months documented by 3 successive examinations, each at least 6 weeks apart. RESULTS: Mean time from onset to surgery was 12.5 ± 4.0 months. Average time to most recent post-onset follow-up occurred at 27.3 months (range, 18-42 months; 15 nerves). Average time to latest follow-up among nonsurgical patients was 33.6 months (range, 18-108 months; 16 nerves). Constrictions involved individual fascicular groups (FCs) of the median nerve and the suprascapular, axillary and radial nerves proper (HGCs). Nine of 11 operative patients experienced clinical recovery compared with 3 of 13 nonsurgical patients. EMG revealed significant motor unit recovery from axonal regeneration in the operative group. CONCLUSIONS: Microsurgical epineurolysis and perineurolysis of FCs and HGCs was associated with significantly improved clinical and nerve regeneration at an average follow-up of 14.8 months compared with nonsurgical management. We recommend microneurolysis of HGCs and FCs as a treatment option for patients with chronic NA who have failed to improve with nonsurgical treatment. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

The Role of Positional Release Therapy in Treating Recalcitrant Brachial Plexus Neuritis: A Case Report.

A 17-year-old female soccer player presented with severe right shoulder pain and scapular winging due to brachial plexus neuritis. The patient was diagnosed with Parsonage-Turner syndrome, a rare condition often resistant to traditional physical therapy, which typically persists for 6 months to years, at times requiring surgical intervention. Over the course of 6 weeks, the patient received positional release therapy once a week coupled with electrical modalities, massage, and a daily home exercise program. This case report is unique because we believe we were the first to use positional release therapy for treatment and the patient's condition resolved more quickly than is typically reported.

Phrenic Nerve Involvement in Neuralgic Amyotrophy (Parsonage-Turner Syndrome).

Neuralgic amyotrophy is a poorly understood neuromuscular disorder affecting peripheral nerves mostly within the brachial plexus distribution but can also involve other sites including the phrenic nerve. In the classic form of the syndrome it causes proximal upper limb and neck pain on the affected side with subsequent muscle weakness that can be highly heterogeneous. Nocturnal noninvasive ventilation support is a first-line treatment after phrenic mononeuropathy. The regular monitoring of diaphragm function with spirometry and diaphragm ultrasound can help determine prognosis and inform decision-making.