Uncovering Parsonage-Turner Syndrome in a Patient Diagnosed with Tendinopathy

Hepatitis E-associated neuralgic amyotrophy: a rare respiratory presentation.

Neuralgic amyotrophy is not the most common neurologic disorder of the shoulder: a 78-month prospective study of 60 neurologic shoulder patients in a specialist shoulder clinic

Background: Neuralgic amyotrophy (NA) has been described as a possible extrahepatic manifestation of hepatitis E virus (HEV) infection. Usually, HEV-associated NA occurs bilaterally. The clinical characteristics determining the course of HEV-associated NA have still not been defined. Methods: In this retrospective multicentric case series, 16 patients with HEV-associated NA were studied and compared to 176 HEV patients without NA in terms of their age, sex, and ALT levels. Results: Neither gender distribution (75% vs. 67% male) nor age (47 vs. 48 years median) differed significantly between the NA patients and controls. Eight NA patients (50%) presented with bilateral involvement—seven of these had right-side dominance and one had left-side dominance. Thirteen cases (81%) were hospitalized. Eight of these patients stayed in hospital for five to seven days, and five patients stayed for up to two weeks. The time from the onset of NA to the HEV diagnosis, as well as the diagnostic and therapeutic proceedings, showed a large variability. In total, 13 (81%) patients received treatment: 1/13 (8%) received intravenous immunoglobulins, 8/13 (62%) received glucocorticoids, 3/13 (23%) received ribavirin, and 6/13 (46%) received pregabalin/gabapentin. Patients with ages above the median (47 years) were more likely to be treated (p = 0.001). Conclusion: HEV-associated NA causes a relevant morbidity. In our case series neither the type of treatment nor the time of initiation of therapy had a significant effect on the duration of hospitalization or the course of the disease. The clinical presentation, the common diagnostic and therapeutic procedures, and the patients’ characteristics showed large variability, demonstrating the necessity of standardized protocols for this rare but relevant disease.

BackgroundParsonage Turner syndrome is an uncommon condition characterized by acute onset shoulder pain, followed by neurologic deficits such as weakness and paresthesia. It is a condition that is thought to be immune-mediated, and triggered by several recognized factors such as trauma, surgery, infections, and immunizations. Upper extremity Parsonage Turner syndrome may affect any distribution of the brachial plexus and most commonly presents unilaterally. Clinical history and examination are the basis of diagnosis, although electrodiagnostic studies may be important for confirmation. Magnetic resonance and ultrasonographic studies have also been effectively used in the diagnosis of Parsonage Turner syndrome. The case herein presents a patient with multiple possible triggers of Parsonage Turner syndrome.Case DescriptionWe present a case of 62-year-old Caucasian male with bilateral radicular pain and weakness in the upper extremities after cervical spine surgery for a fracture in a patient that was infected with COVID-19. The patient underwent electrodiagnostic testing, as well as ultrasonographic studies that demonstrated Parsonage Turner syndrome. A literature review on Parsonage Turner syndrome associated with trauma, surgery and COVID-19 was also performed.ConclusionsMost cases of Parsonage Turner syndrome have a known associated risk factor. The patient in this report is unique in that they had several known risk factors for Parsonage Turner syndrome simultaneously. For timely and accurate diagnosis, it is important to consider the potential triggers of Parsonage Turner syndrome including trauma, surgery and viral illnesses such as COVID-19.

To determine whether there is an association between an acute preceding hepatitis E virus (HEV) infection and neuralgic amyotrophy (NA), and if so, whether patients with HEV-related NA differ from patients without an associated HEV infection. HEV testing was conducted in a retrospective cohort of 28 Cornish patients with NA (2011-2013) and a prospective cohort of 38 consecutive Dutch patients with NA (2004-2007). Acute-phase serum samples were analyzed for the presence of anti-HEV immunoglobulin (Ig) M and IgG and HEV RNA (quantitative real-time PCR). Five cases (10.6%) of acute hepatitis E infection were identified in a total group of 47 patients with NA of whom serum samples were available. In 4 patients, HEV RNA was detected in serum samples taken at presentation. All patients with HEV-associated NA had clinical and electrophysiologic evidence of bilateral brachial plexus involvement. Anti-HEV IgM positivity was not related to age, sex, disease severity, disease course, or outcome. Acute hepatitis E is found in 10% of patients with NA from the United Kingdom and the Netherlands. Further research is required to investigate the role of HEV in NA in other geographical locations and to determine pathophysiologic mechanisms.

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.

Neuralgic amyotrophy (NA), or Parsonage-Turner syndrome, describes a spectrum of relatively rare peripheral neuropathies characterized by acute pain followed by patchy, multifocal sensory disturbances, weakness, and atrophy, predominantly in the upper extremity. While typically affecting the upper brachial plexus, NA can also present with selective involvement of distal nerve branches. This case report details a unique presentation of NA in a 39-year-old male bodybuilder who developed isolated weakness and atrophy of the left pectoralis major and a single head of the left triceps following a painful prodrome without preceding trauma. Electrodiagnostic studies confirmed active denervation in these muscles, while imaging revealed no cervical pathology or brachial plexus abnormalities. The patient was diagnosed with NA, specifically affecting the lateral pectoral nerve, and a branch of the radial nerve. Our case is compared to existing literature, including isolated reports of pectoralis major and branch-specific radial nerve involvement in NA. While these less common deficits have been described as separate entities, our case uniquely demonstrates them co-occurring. It highlights the potential for NA to affect less commonnerves (lateral pectoral and motor branches of the radial nerve) and specific nerve branches. Differential diagnoses included mononeuritis multiplex, compressive neuropathy, and infectious or inflammatory neuropathies. Mononeuritis multiplex was less likely due to the absence of sensory deficits and systemic symptoms. Infectious and inflammatory neuropathies were ruled out based on clinical presentation and lack of relevant markers. Compressive neuropathy was considered, however the acute painful prodrome, electrodiagnostic findings, and natural history favored NA. This case contributes to understanding the variable clinical spectrum of NA. It also underscores the importance of clinical history and comprehensive work-up in atypical presentations for prompt diagnosis and effective management. While the prognosis for NA is generally favorable, the long-term outcome for highly selective presentations requires further investigation.

BackgroundWest Nile Virus is a single-stranded Ribonucleic Acid arbovirus of the Flaviviridae family that is transmitted to humans by Culex species mosquitoes. West Nile Virus infection is asymptomatic in the majority of affected people. Of those who develop symptoms, the usual manifestation is a febrile syndrome, while only 1% develop neurological symptoms due to a neuroinvasive form of infection, including encephalitis, meningitis, asymmetrical flaccid paralysis, or a combination of all these features. Parsonage–Turner syndrome is a rare disorder characterized by sudden painful symptoms and subsequent paralysis, involving a shoulder or one of the upper limbs due to post-infective brachial plexopathy. The etiology is unknown, although it can be considered a multifactorial process: a predisposing factor, such as viral infection or strenuous upper-extremity exercise, can trigger an immune-mediated process localized in the brachial plexus.Clinical presentationIn late summer, a 79-year-old male Italian patient was admitted to the emergency department for acute right upper limb weakness and high fever, without any mental status impairment, pain, sensory alterations, or signs of meningeal irritation. Laboratory tests confirmed acute West Nile Virus infection, expressed as a unilateral upper limb flaccid paralysis. After a few days, the patient reported an acute pain in the right upper limb scarcely responsive to nonsteroidal anti-inflammatory drug therapy and a subsequent wider distribution of flaccid paralysis. After multiple examinations, Parsonage–Turner syndrome could be suspected. Patient was treated with steroids and reported an improvement of clinical condition after 2 months, with complete pain remission but partial strength recovery in the affected limb.ConclusionsWest Nile Virus disease has a broad spectrum of neurological manifestations, among which the most common are signs of meningeal irritation or cognitive impairment. We report an unusual presentation of neuroinvasive West Nile Virus infection with arm weakness as expression of unilateral viral neuritis, followed by a post-infective brachial plexopathy consistent with Parsonage–Turner syndrome diagnosis. We diagnosed Parsonage–Turner syndrome after excluding the most common causes of atraumatic acute upper limb pain, through a challenging differential diagnosis in a patient with several comorbidities.

Parsonage–Turner syndrome revealing Lyme borreliosis

Neuralgic amyotrophy is a common peripheral nerve disorder caused by autoimmune inflammation of the brachial plexus, clinically characterized by acute pain and weakness of the shoulder muscles, followed by motor impairment. Despite recovery of the peripheral nerves, patients often have residual motor dysfunction of the upper extremity, leading to persistent pain related to altered biomechanics of the shoulder region. Building on clinical signs that suggest a role for cerebral mechanisms in these residual complaints, here we show and characterize cerebral alterations following neuralgic amyotrophy. Neuralgic amyotrophy patients often develop alternative motor strategies, which suggests that (mal)adaptations may occur in somatomotor and/or visuomotor brain areas. Here, we tested where changes in cerebral sensorimotor representations occur in neuralgic amyotrophy, while controlling for altered motor execution due to peripheral neuropathy. We additionally explore the relation between potential cerebral alterations in neuralgic amyotrophy and clinical symptoms. During functional MRI scanning, 39 neuralgic amyotrophy patients with persistent, lateralized symptoms in the right upper extremity and 23 matched healthy participants solved a hand laterality judgement task that can activate sensorimotor representations of the upper extremity, across somatomotor and visuomotor brain areas. Behavioural and cerebral responses confirmed the involvement of embodied, sensorimotor processes across groups. Compared with healthy participants, neuralgic amyotrophy patients were slower in hand laterality judgement and had decreased cerebral activity specific to their affected limb in two higher-order visual brain regions: the right extrastriate cortex and the parieto-occipital sulcus. Exploratory analyses revealed that across patients, extrastriate activity specific to the affected limb decreased as persistent pain increased, and affected limb-related parieto-occipital activity decreased as imagery performance of the affected limb became slower. These findings suggest that maladaptive cerebral plasticity in visuomotor areas involved in sensorimotor integration plays a role in residual motor dysfunction and subsequent persistent pain in neuralgic amyotrophy. Rehabilitation interventions that apply visuomotor strategies to improve sensorimotor integration may help to treat neuralgic amyotrophy patients.

The neuralgic amyotrophy may be of difficult diagnosis, due to phenotypic variability, with different initial presentations (upper plexus multiple mononeuropathy, lumbosacral involvement, distal reached, phrenic involvement). To date, there is little guidance on these patients' therapeutic management, especially those for which neuralgic amyotrophy is triggered by hepatitis E virus (HEV-NA). The study aims to identify specific features that characterize patients bearing the neuralgic amyotrophy triggered by HEV. We first describe a new case report of HEV-neuralgic amyotrophy, with delayed diaphragmatic reach. Then, the literature was searched for reports of HEV-NA (n=39), and neuralgic amyotrophy with phrenic paresis (n=42) from 1999 to June 2016. Relevant data were retrieved, analyzed and compared with the parameters of idiopathic neuralgic amyotrophy (n=199) of the largest cohort, described by Van Alfen and Van Engelen in 2006. Compared to the published cohort, HEV-NA patients were more likely to be men (M/F 34/5 vs. 136/63, p=0.017), with more frequent bilateral symptoms (86.8% cases vs. 28.5%, p<0.0001) as well as phrenic paresis (18.0 vs. 6.6%, p=0.028). The clinical improvement is poor, with 15.6% of cases with remission only. A particular phenotype characteristic of the HEV-induced neuralgic amyotrophy has arisen. Our findings call for action in validating the above-mentioned features that illustrate the HEV-NA cases as an early diagnosis would prevent complications, especially the phrenic damage often associated with a worse functional outcome.

ObjectiveNeuralgic amyotrophy is considered a rare peripheral nervous system disorder but in practice seems grossly under recognized, which negatively affects care for these patients. In this study we prospectively counted the one-year incidence rate of classic neuralgic amyotrophy in a primary care setting.MethodsIn a prospective cohort study during the year 2012 we registered all new cases of neck, shoulder or arm complaints from two large primary care centers serving a population of 14,118. Prior to study, general practitioners received a short training on how to diagnose classic neuralgic amyotrophy. Neuralgic amyotrophy was defined according to published criteria irrespective of family history. Only patients with a classic phenotype were counted as definite cases. After inclusion, patients with suspected neuralgic amyotrophy who had not yet seen a neurologist were offered neurologic evaluation for diagnostic confirmation.ResultsOf the 492 patients identified with new onset neck, shoulder or arm complaints, 34 were suspected of having neuralgic amyotrophy. After neurologic evaluation the diagnosis was confirmed in 14 patients. This amounts to a one-year incidence rate for classic neuralgic amyotrophy of 1 per 1000.ConclusionsOur findings suggest that neuralgic amyotrophy is 30-50 times more common than previously thought. Unawareness of the disorder and its clinical presentation seems the most likely explanation for this difference. An incidence rate of 1 per 1000 and the long-term sequelae many patients suffer warrant more vigilance in diagnosing the disorder, to pave the way for timely treatment and prevent complications.

To describe the clinical phenotype and recovery of diaphragm dysfunction caused by neuralgic amyotrophy in a large cohort of patients, to improve accurate awareness of this entity, and to encourage adoption of a standardized approach for diagnosis and treatment. This observational cohort study recruited adult patients with neuralgic amyotrophy and symptoms of idiopathic phrenic neuropathy from the database of the Dutch expert center for neuralgic amyotrophy and the Dutch centers for home mechanical ventilation. Demographic and clinical information on diagnosis, symptoms, and recovery was obtained from chart review. We attempted to contact all patients for a follow-up interview. Phrenic neuropathy occurs in 7.6% of patients with neuralgic amyotrophy. Unilateral diaphragmatic dysfunction and bilateral diaphragmatic dysfunction are frequently symptomatic, causing exertional dyspnea, orthopnea, disturbed sleep, and excessive fatigue. Diagnostic practices varied widely and were often not optimally targeted. The majority of patients experienced at least moderate recovery within 2 years. We recommend screening every patient with neuralgic amyotrophy for diaphragm dysfunction by asking about orthopnea and by performing upright and supine vital capacity screening and diaphragm ultrasound in cases of suspected phrenic neuropathy to optimize diagnosis and care.

Role of electromyography for the diagnosis of Parsonage-Turner syndrome

Background:Neuralgic amyotrophy (NA) or Parsonage and Turner syndrome is triggered at least in 25% by a viral infection: parvovirus B19, CMV, HSV, etc... Recently, few cases of Hepatitis E Virus (HEV) related NA were reported. This particular association remains little known and is overlooked by most physicians. Besides, clinical, electrodiagnostic (EDX) and MRI characteristics, as well as evolution of HEV-related NA have not been fully described yet.Objectives:To describe 6 cases of HEV-related NA and to perform a review of the literature.Methods:We describe longitudinally clinical examination, electrodiagnostic (EDX), biological and MRI results of 6 cases of HEV-associated NA, diagnosed in our center.Results:The 6 cases were aged between 33 and 57 years old (mean 44.5), sex ratio was 5M/1F. All patients had positive IgM anti-HEV (serology) and a cervical MRI that could not explain clinical presentation. Overall, the 6 patients totalize 26 mononeuropathies (range 1 to 8 per patient), 5/6 patients had a severe presentation of NA, with bilateral and asymmetric symptoms (3 cases). HEV-related NA involved classical nerves such as supra-scapular (6 cases, twice bilaterally) and long thoracic nerves (5 cases), some less classical nerves like anterior interosseous nerve (3 cases, twice bilaterally), and some very unusual ones such as the lateral antebrachial cutaneous nerve (1 case) and the sensory fibers of median nerve (1 case). NA also involved accessory spinal (2 cases, once bilaterally) and phrenic nerves (1 case bilaterally), both originating from cervical plexus. The EDX pattern of these nerve lesions consisted of unique or multiple extensive asymmetric inflammatory mononeuropathies with severe axonal loss and numerous denervation signs damage involving mostly the supra-scapular. On scapular MRI (available for 5/6 patients), amyotrophy in at least one muscle was observed in all patients. Out of 26 nerves involved, after 12 months all had well recovered (above 3/5 MRC scale).Conclusion:HEV should be systematically screened when NA is suspected, whatever the severity, if the onset is less than 3 or 4 months (before IgMs anti-HEV disappear). HEV-related NA appears to be frequently associated with a severe pattern, without modifying the recovery usually observed.Disclosure of Interests:Romain Garofoli: None declared, Paul Seror: None declared, Jennifer Zauderer: None declared, Christelle Nguyen: None declared, François Rannou Grant/research support from: Pierre Fabre, Fidia, MSD, Pfizer, Bone Therapeutics, Expanscience, Grunenthal, Thuasne, Genévrier, Fondation Arthritis, Consultant of: Pierre Fabre, Fidia, MSD, Pfizer, Bone Therapeutics, Expanscience, Grunenthal, Thuasne, Genévrier, Speakers bureau: Pierre Fabre, Fidia, MSD, Pfizer, Bone Therapeutics, Expanscience, Grunenthal, Thuasne, Jean-Luc Drapé: None declared, Alexandra Roren: None declared, Marie-Martine Lefevre Colau: None declared