Neuralgic Amyotrophy Research Articles

Idiopathic Neuralgic Amyotrophy in a Child Treated with Integrated Rehabilitation Program: A Case Report

Idiopathic neuralgic amyotrophy (INA) in children is a rare peripheral neuropathy showing distinctive clinical features. An 8-year-old boy presented with left shoulder pain and weakness lasting 4 weeks. He had learned Taekwondo and swimming before the onset of pain. A fever had developed 10 weeks before onset but had completely resolved after medication. Initially, he was diagnosed with shoulder subluxation and underwent successful reduction. However, he continued to complain of persistent pain and weakness. All the studies indicated normal findings, except for the electrodiagnostic assessment, which revealed partial brachial plexopathy. A rehabilitation program was performed. It consisted of physical and occupational therapy twice a week. After 5-month rehabilitation, the patient remarkably improved in both neurologic and functional status. INA should be considered and evaluated in cases with abrupt onset of shoulder pain and weakness in children. Also, an integrated rehabilitation program during the subacute phase can be helpful to facilitate neurologic and functional recovery and reduce sequelae.

Unilateral Diaphragmatic Paralysis Secondary to Neuralgic Amyotrophy—a Case Report

Unilateral Diaphragmatic Paralysis Secondary to Neuralgic Amyotrophy—a Case Report

Neuromuscular diseases associated with COVID-19 vaccines: a systematic review and pooled analysis of 258 patients

BackgroundNeuromuscular diseases (NMD) emerged as one of the main side effects of the COVID-19 vaccination. We pooled and summarized the evidence on the clinical features and outcomes of NMD associated with COVID-19 vaccination.MethodsWe comprehensively searched three databases, Medline, Embase, and Scopus, using the key terms covering “Neuromuscular disease” AND “COVID-19 vaccine”, and pooled the individual patient data extracted from the included studies.ResultsA total of 258 NMD cases following COVID-19 have been reported globally, of which 171 cases were Guillain-Barré syndrome (GBS), 40 Parsonage-Turner syndrome (PTS), 22 Myasthenia Gravis (MG), 19 facial nerve palsy (FNP), 5 single fiber neuropathy, and 1 Tolosa-Hunt syndrome. All (100%) SFN patients and 58% of FNP patients were female; in the remaining NMDs, patients were predominantly male, including MG (82%), GBS (63%), and PTS (62.5%).The median time from vaccine to symptom was less than 2 weeks in all groups. Symptoms mainly appeared following the first dose of vector vaccine, but there was no specific pattern for mRNA-based.ConclusionCOVID-19 vaccines might induce some NMDs, mainly in adults. The age distribution and gender characteristics of affected patients may differ based on the NMD type. About two-thirds of the cases probably occur less than 2 weeks after vaccination.

Inborn errors of type I interferon immunity in patients with symptomatic acute hepatitis E.

The clinical spectrum of human infection by HEV ranges from asymptomatic to severe acute hepatitis. Furthermore, HEV can cause diverse neurological manifestations, especially Parsonage-Turner syndrome. Here, we used a large-scale human genomic approach to search for genetic determinants of severe clinical presentations of HEV infection. We performed whole genome sequencing in 3 groups of study participants with PCR-proven acute HEV infection: (1) 24 patients with symptomatic acute hepatitis E; (2) 12 patients with HEV-associated Parsonage-Turner syndrome; and (3) 16 asymptomatic blood donors (controls). For variant calling and annotation, we used GATK4 best practices followed by Variant Effect Predictor (VEP) and Annovar. For variant classification, we implemented the American College of Medical Genetics and Genomics/Association for Molecular Pathology Bayesian classification framework in R. Variants with a probability of pathogenicity >0.9 were considered damaging. We used all genes with at least 1 damaging variant as input for pathway enrichment analyses.We observed a significant enrichment of type I interferon response pathways in the symptomatic hepatitis group: 10 out of 24 patients carried a damaging variant in one of 9 genes encoding either intracellular viral sensors ( IFIH1 , DDX58 , TLR3 , POLR3B , POLR3C ) or other molecules involved in type I interferon response [interferon regulatory factor 7 ( IRF7 ), MYD88 , OAS3 , GAPDH ]. We did not find any enriched pathway in the Parsonage-Turner syndrome group or in the controls. Our results highlight the essential role of type I interferon in preventing symptomatic acute hepatitis E.

Parsonage-Turner syndrome after COVID-19 vaccination: A case report

Parsonage-Turner syndrome (PTS) is one of the rare peripheral neuropathies that causes sudden and severe pain followed by sensory defect, loss of muscle strength and atrophy in the upper extremities. If a patient experiences persistent shoulder pain after COVID-19 vaccination, followed by weakness and sensory changes in the extremity, Parsonage-Turner syndrome should be considered in the differential diagnosis. We report a rare case of PTS that developed after COVID-19 vaccination.

Obstetric Management of a Novel Superimposition of Facial Onset Sensory and Motor Neuronopathy on Parsonage Turner Syndrome

Obstetric Management of a Novel Superimposition of Facial Onset Sensory and Motor Neuronopathy on Parsonage Turner Syndrome

Parsonage-turner syndrome after COVID-19 vaccination in a 25-year-old woman: A case report

Parsonage-turner syndrome after COVID-19 vaccination in a 25-year-old woman: A case report

Post-Transplant Pain and Paralysis: Neurologic Amyotrophy As an Atypical Cause of Shoulder Dysfunction Following Hematopoietic Stem Cell Transplant

Introduction: Neuralgic amyotrophy (NA), also known as Parsonage-Turner Syndrome, brachial neuritis, or idiopathic brachial plexopathy, is a rare inflammatory peripheral nervous system disorder affecting the brachial plexus, typically presenting as sudden and abrupt unilateral shoulder pain followed by progressive painless weakness and occasionally reflex and sensory abnormalities. Diagnosis is clinical - based on history and detailed neuromuscular physical examination. Electrodiagnostic studies (EDX) and advanced imaging are supportive to confirm diagnosis and exclude alternative etiologies. While the pathogenesis is not entirely understood, the idiopathic form is thought to be secondary to immune or vascular mediated effects with only a few case reports after hematopoietic stem cell transplantation (HCT). Given under recognition and misdiagnosis, a high degree of suspicion is necessary in patients developing acute onset of shoulder pain after HCT. While no curative treatment is available, early diagnosis will result in appropriate management, patient education, pain control, and timely focused rehabilitation. Methods: We retrospectively collected demographics, HCT details, clinical characteristics, imaging studies, and EDX on patients with acute shoulder pain post-HCT at a single institution. Descriptive statistics were utilized to summarize the collected data. Results: Between 8/2020 -7/2022, nine patients (44% male, median age 60 years) were diagnosed with NA following autologous (n=4, for multiple myeloma (n=2), non-Hodgkin lymphoma (n=1), or germ cell tumor (n=1)) or allogeneic HCT (n=5, for myelodysplastic syndrome (n=3) and acute myelogenous leukemia (n=2)). Each patient acutely developed severe shoulder pain at a median of 9 days (range 1-21) mean. Shoulder pain was bilateral in 3 patients. Neurological weakness involving the shoulder (s) was observed on average 5.1 days after pain onset (range 1-15 days). All but one patient developed sensory deficits in the affected upper extremity. Shoulder pain subsided after a median of 23 days (range 8-40 days). MRI of the shoulder and/or brachial plexus was performed in all patients and showed periscapular muscle edema, atrophy and enhancement in six patients. These signal abnormalities were observed in varying distributions including supraspinatus, infraspinatus, deltoid, teres minor, serratus anterior and intercostals (Figure 1). Of the 6 patients who had EDX, findings were consistent with NA in 5 patients (axillary neuropathy in 2 patients, upper trunk plexopathy in 1 patient, bilateral posterior cord plexopathy in 1 patient and diffuse bilateral brachial plexopathy in 1 patient). All patients were treated with pain medications and received optimal rehabilitative care including consult with physical medicine and rehabilitation physicians and physical and occupational therapy. Upon long term follow up (>1 year), three patients experienced full or near-full resolution of symptoms, four patients partially recovered, and two patients had little to no improvement of their neurological symptoms. Conclusion: Patients undergoing HCT are susceptible to neurological insults, potentially driven by immune-mediated mechanisms. In individuals experiencing acute shoulder pain accompanied by neurological symptoms early post-HCT, it is important to maintain a high level of suspicion for the diagnosis of NA. A timely diagnosis is essential to facilitate early intervention and ensure optimal functional recovery. MRI of the shoulder and/or brachial plexus and EDX are valuable diagnostic tools for assessing the presence and distribution of neuromuscular involvement. While recovery is possible, comprehensive long-term follow-up is necessary to ascertain the extent of symptom resolution and functional restoration.

Diagnostics and treatment of hourglass-like nerve constrictions and torsions in neuralgic amyotrophy

Neuralgic amyotrophy is adisease of the peripheral nervous system characterized by severe neuropathic pain followed by peripheral paralysis. Adistinction is made between ahereditary 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: sensitivity and specificity of magnetic resonance neurography in diagnosis: A retrospective study.

Neuralgic amyotrophy (NA) is a clinically acute or subacute disease. To study the characteristics of brachial plexus magnetic resonance neurography (MRN) in patients with NA, and to explore the clinical application value of MRN combined with electromyography (EMG) in the diagnosis of NA. Brachial plexus MRN images of 32 patients with NA were retrospectively analyzed, and their characteristics were investigated. The accuracy, sensitivity and specificity of MRN, EMG, and the combination of the 2 methods for NA diagnosis were compared. Among the 32 patients with NA, 28 (87.5%) cases of unilateral brachial plexus involvement, 18 (56.3%) cases of multiple nerve roots involvement. In 10 cases, C5 nerve roots were involved alone, and in 9 cases, C5 to C6 nerve roots were involved together. The T2 signal intensity of the affected nerve increased, and 19 cases showed thickened and smooth nerve root edges. Twelve cases showed uneven thickening and segmental stenosis of the involved nerve roots. The diagnostic accuracy, sensitivity, and specificity of MRN for NA were higher than those of EMG. Combining MRN and EMG could improve the sensitivity and specificity of diagnosis. The main feature of MRN in patients with NA was that it was unilateral brachial plexus asymmetric involvement. The diagnostic effect of MRN was better than that of EMG. The combined diagnosis of MRN and EMG can help clinicians diagnose NA accurately.

Double trouble-unveiling the hidden pain: bilateral paediatric parsonage-turner syndrome: an unprecedented encounter

Parsonage-turner syndrome (PTS), also known as idiopathic brachial neuritis, is a rare disorder characterized by sudden-onset severe shoulder pain followed by weakness and atrophy of the shoulder and arm muscles. This case report presents the clinical scenario of an 11-year-old girl diagnosed with PTS, highlighting the unique challenges and considerations in the paediatric population. In our case, a 11-year-old girl presented with sudden-onset severe bilateral shoulder pain, followed by weakness and atrophy of the shoulder and upper arm muscles. The clinical examination revealed muscle wasting and limited range of motion. The diagnosis of PTS was confirmed through MRI and electromyography (EMG) showing denervation in the affected shoulder muscles. Patient was managed with symptomatic supportive treatment in the form of analgesics and physical therapy. Patient is on the road to recovery and on regular follow up. We concluded that prompt recognition and appropriate management of PTS are crucial to optimize outcomes and prevent long-term disability, particularly in the paediatric population.

Course of Rehabilitation Treatment of Neuralgic Amyotrophy Presenting with Collet-Sicard Syndrome

Course of Rehabilitation Treatment of Neuralgic Amyotrophy Presenting with Collet-Sicard Syndrome

High-resolution MR neurography follow-up of SARS-CoV-2 vaccination-associated neuralgic amyotrophy.

Little is known about the value of high-resolution follow-up imaging in patients with neuralgic amyotrophy (NA) and the question of the best treatment algorithm remains unclear. Three patients (one female, two male) with the clinical presentation of SARS-CoV-2-vaccination-associated NA underwent initial magnetic resonance neurography (MRN) imaging and follow-up examinations. All patients showed a marked clinical improvement, independent of treatment, including an almost full recovery of motor function over the course of 8-12 months which was accurately mirrored by imaging findings on MRN. MRN imaging is a valuable tool for monitoring the further clinical course of patients suffering from vaccination-associated NA.

Application of a nerve stereoscopic reconstruction technique based on ultrasonic images in the diagnosis of neuralgic amyotrophy.

Objective: To propose a nerve stereoscopic reconstruction technique based on ultrasound imaging for site diagnosis, intuitive reflection of disease severity, and classification of neuralgic amyotrophy (NA). Methods: We enrolled 44 patients with NA who underwent high-frequency ultrasonography examination. Multiple sites on the normal side and the affected side were scanned to calculate the ratio of the cross-section area (CSA) of the affected side to the normal side at each location measured, i.e., the cross-section area swelling ratio (CSASR). The CSASR of 44 patients and 30 normal controls was analyzed to determine their threshold value for the diagnosis of NA. Then, ultrasound images of the cross-section were used to reconstruct the stereoscopic model of the nerve on the affected side and the normal side. Using the CSASR values in each measurement location, a CSASR stereoscopic model was developed. Results: The threshold value of CSASR for ultrasound diagnosis of NA was 1.55. The average diseased segments per patient was 2.49 ± 1.97, with an average overall length of 10.03 ± 7.95cm. Nerve stereoscopic reconstruction could be conducted for swelling, torsion, incomplete constriction, and complete constriction. Conclusion: The ultrasound image reconstruction method proposed in this study can accurately determine the site, range, and type of neuropathies in patients with NA, and simultaneously provide complete and accurate data information and intuitive morphological information.

MR Neurography and Quantitative Muscle MRI of Parsonage Turner Syndrome Involving the Long Thoracic Nerve.

Parsonage-Turner syndrome (PTS) is characterized by severe, acute upper extremity pain and subsequent paresis and most commonly involves the long thoracic nerve (LTN). While MR neurography (MRN) can detect LTN hourglass-like constrictions (HGCs), quantitative muscle MRI (qMRI) can quantify serratus anterior muscle (SAM) neurogenic changes. 1) To characterize qMRI findings in LTN-involved PTS. 2) To investigate associations between qMRI and clinical assessments of HGCs/electromyography (EMG). Prospective. 30 PTS subjects (25 M/5 F, mean/range age = 39/15-67 years) with LTN involvement who underwent bilateral chest wall qMRI and unilateral brachial plexus MRN. 3.0 Tesla/multiecho spin-echo T2-mapping, diffusion-weighted echo-planar-imaging, multiecho gradient echo. qMRI was performed to obtain T2, muscle diameter fat fraction (FF), and cross-sectional area of the SAM. Clinical reports of MRN and EMG were obtained; from MRN, the number of HGCs; from EMG, SAM measurements of motor unit recruitment levels, fibrillations, and positive sharp waves. qMRI/MRN were performed within 90 days of EMG. EMG was performed on average 185 days from symptom onset (all ≥2 weeks from symptom onset) and 5 days preceding MRI. Paired t-tests were used to compare qMRI measures in the affected SAM versus the contralateral, unaffected side (P < 0.05 deemed statistically significant). Kendall's tau was used to determine associations between qMRI against HGCs and EMG. Relative to the unaffected SAM, the affected SAM had increased T2 (50.42 ± 6.62 vs. 39.09 ± 4.23 msec) and FF (8.45 ± 9.69 vs. 4.03% ± 1.97%), and decreased muscle diameter (74.26 ± 21.54 vs. 88.73 ± 17.61 μm) and cross-sectional area (9.21 ± 3.75 vs. 16.77 ± 6.40 mm2). There were weak to negligible associations (tau = -0.229 to <0.001, P = 0.054-1.00) between individual qMRI biomarkers and clinical assessments of HGCs and EMG. qMRI changes in the SAM were observed in subjects with PTS involving the LTN. 2 TECHNICAL EFFICACY: Stage 1.

Síndrome de Parsonage Turner em Nadadora de Elite

Parsonage-Turner syndrome (PTS) is a rare disorder characterized by sudden shoulder pain followed by neurologic deficits. The exact etiology remains unclear, although a genetic predisposition is suspected in association with a trigger. The diagnosis is made clinically, supported by electrodiagnostic and imaging studies. There is no specific treatment, and the prognosis is generally favorable. This case-report relates to an 18-year-old competitive swimmer who developed left shoulder pain after a flu-like illness, resulting in a deficit of muscle strength and sensitivity. She was diagnosed at PTS and treated with corticosteroids and an individualized rehabilitation program, resulting in resolution of the condition within 6 months. Early diagnosis and treatment are important to prevent long-term sequelae and allow a quicker return to sports activities.

Neuralgic amyotrophy associated with hepatitis E as a rare cause of dyspnoea

Neuralgic amyotrophy associated with hepatitis E as a rare cause of dyspnoea

703 Parsonage Turner Syndrome After COVID-19 Vaccination – a Systematic Review

Abstract Aim Parsonage Turner Syndrome (PTS) is a rare acute neuritis usually reported after an antecedent event like surgery, infection, trauma, medication or immunisation. PTS presents as sudden, often unilateral, severe shoulder pain with progressive weakness and altered neurology. The global mass COVID vaccine program has resulted in increased reporting of post-vaccine PTS. The aim of this review is to report the outcomes of PTS after COVID-19 vaccination. Method A systematic review was conducted according to the PRISMA statement. An electronic search was performed on Medline, PubMed, Embase and CINAHL databases using the keywords ‘COVID-19’, ‘Coronavirus’, ‘Parsonage Turner Syndrome’, ‘Brachial neuritis’, ‘Neuralgic Amyotrophy’, ‘Vaccine’, ‘Immunisation’ and ‘Shoulder’. Results 16 articles with a total of 35 patients, 24 males and 11 females, were reviewed. The mean age was 52 years. The median time to develop symptoms was 7 days, with 69% patients reporting ipsilateral symptoms to the vaccinated arm. 91% patients presented with sensory and motor symptoms. MRI scan was normal in 26% while denervation muscle oedema was present in 20%. 37% patients were treated with physiotherapy and rest, while 63% received steroid therapy. 23% showed no improvement while 69% showed partial or complete improvement. The mean follow-up time was 3 months (range one week to six months). Conclusions PTS is a rare complication described with vaccines like influenza, tetanus and hepatitis. PTS is also associated with the COVID vaccine. The limited reports suggest the treatment and recovery are consistent with that for other aetiology. This complication is not a reason to avoid the COVID vaccine.

Neuralgic amyotrophy: a common cause of unilateral and bilateral diaphragmatic pareses

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.

Hourglass-like constrictions on MRI are common in electromyography-confirmed cases of neuralgic amyotrophy (Parsonage-Turner syndrome): A tertiary referral center experience.

Hourglass-like constrictions (HGCs) of involved nerves in neuralgic amyotrophy (NA) (Parsonage-Turner syndrome) have been increasingly recognized with magnetic resonance neurography (MRN). This study sought to determine the sensitivity of HGCs, detected by MRN, among electromyography (EMG)-confirmed NA cases. This study retrospectively reviewed records of patients with the clinical diagnosis of NA, and with EMG confirmation, who underwent 3-Tesla MRN within 90 days of EMG at a single tertiary referral center between 2011 and 2021. "Severe NA" positive cases were defined by a clinical diagnosis and specific EMG criteria: fibrillation potentials or positive sharp waves, along with motor unit recruitment (MUR) grades of "discrete" or "none." On MRN, one or more HGCs, defined as focally decreased nerve caliber or diffusely beaded appearance, was considered "imaging-positive." Post hoc inter-rater reliability for HGCs was measured by comparing the original MRN report against subsequent blinded interpretation by a second radiologist. A total of 123 NA patients with 3-Tesla MRN performed within 90 days of EMG were identified. HGCs were observed in 90.2% of all NA patients. In "severe NA" cases, based on the above EMG criteria, HGC detection resulted in a sensitivity of 91.9%. Nerve-by-nerve analysis (183 nerve-muscle pairs, nerves assessed by MRN, muscles assessed by EMG) showed a sensitivity of 91.0%. The second radiologist largely agreed with the original HGC evaluation, (94.3% by subjects, 91.8% by nerves), with no significant difference between evaluations (subjects: χ2 = 2.27, P = .132, nerves: χ2 = 0.98, P = .323). MRN detection of HGCs is common in NA.