Neuralgic Amyotrophy Research Articles

Hepatitis E and diaphragmatic dysfunction: Case series and review of the literature.

The causes of diaphragmatic paresis are manifold. An association between neuralgic amyotrophy (NA) and hepatitis E virus (HEV) infection has been reported. We wondered about the prevalence of diaphragmatic disfunction and hepatitis E infection in our clinic. From July 1st, 2020 to August 31st, 2023, patients presenting with diaphragmatic dysfunction and simultaneous clinical symptoms of an acute NA, or a history of NA, as well as patients with previously unexplained diaphragmatic dysfunction were examined for HEV infection. By August 31st, 2023, 13 patients with diaphragmatic dysfunction and HEV infection were diagnosed (4 women, 9 men). Mean age was 59 ± 10 years. Liver values were normal in all patients. The median latency to diagnosis was five months (range: 1-48 months); nine patients, 4 of them with typical symptoms of NA, presented with acute onset three patients showed bilateral diaphragmatic dysfunction. All patients had a positive IgG immunoblot. Seven patients, three with NA, had an elevated hepatitis E IgM titer and six of them also a positive IgM immunoblot. In all cases, O2C hepatitis genotype 3 was identified. In eight cases, all those with a high IgG titer >125, the O2 genotype 1 was also detected. NA that shows involvement of the phrenic nerve resulting in diaphragmatic dysfunction and dyspnoea, may be associated with HEV infection. The observation of 13 patients with diaphragmatic dysfunctions and HEV infection within a period of three years indicates a high number of undetected HEV-associated diaphragmatic dysfunction in the population, especially in the absence of NA symptoms. Therefore, even in diaphragmatic dysfunction without NA symptoms and causative damaging event, HEV infection should be considered, as it may represent a subform of NA with only phrenic nerve involvement. Therapy of HEV-associated diaphragmatic dysfunction in the acute phase is an open question. In view of the poor prognosis for recovery, antiviral therapy should be discussed. However, no relevant data are currently available.

Bilateral Phrenic Nerve Palsy Associated With Neuralgic Amyotrophy

Bilateral Phrenic Nerve Palsy Associated With Neuralgic Amyotrophy

138 Inflammatory Neuropathy in the Military: Effects on Readiness and Retention

INTRODUCTION: Neuralgic amyotrophy (NA), or Parsonage-Turner Syndrome, is a complex, multifocal presumed inflammatory disorder of the peripheral nervous system. Presentation varies widely though it classically presents following a precipitating event and begins with a period of sudden onset pain followed by functional loss and atrophy. Prognosis is generally favorable with recovery rates near 80% by 2-3 years. Currently, no data exists on the natural history or surgical management of NA in the active duty (AD) military population, who are subject to uniquely physical work demands. As such, the impact of NA on military readiness and retention remains poorly understood. METHODS: AD service-members with a clinic ICD-10 diagnosis of NA were identified from the MHS medical record between the years 2011-2022 and data were obtained retrospectively through individual chart review. RESULTS: Over an eleven-year period, forty-one active duty patients were identified (27 males, 12 females, average age 39.6) with an ICD-10 diagnosis of NA. Average time to diagnosis from symptom presentation was 152 days (SD 177). Workup included EMG/NCS in nearly all patients, but advanced imaging in only 9/41 (22%). Ten patients underwent surgical intervention (7 nerve transfers, 1 neurolysis only, 1 tendon transfer, 1 cervical spine decompression). Full recovery was observed for 13/41 (32%) patients while 26/41 (63%) gained only partial improvement (two with unknown recovery/lost in follow-up). Only 1/10 (10%) surgical patients had full recovery. Eight patients underwent a medical board evaluation and were discharged from military service (20%). CONCLUSIONS: Neuralgic amyotrophy is a complex disease with a prolonged recovery course that can impact military readiness and active duty retainment.

An unusual cause of unilateral shoulder weakness: Parsonage–Turner syndrome—A case report

An unusual cause of unilateral shoulder weakness: Parsonage–Turner syndrome—A case report

Parsonage-Turner Syndrome following COVID-19 Vaccination: A Systematic Review.

Parsonage-Turner syndrome (PTS) is an inflammatory condition of the brachial plexus, with more than half of patients presenting a trigger, such as infection or vaccination. Our objective was to synthesize the clinical and paraclinical features, therapeutic responses, and outcomes of PTS post-COVID-19 vaccination. We systematically reviewed two databases (LitCOVID and the WHO database on COVID-19) up to January 2024 following a published protocol (OSF registries). We included 59 cases. PTS occurred more frequently in males (61.1% mRNA group, 83.3% viral vector group). Patients in the mRNA group were younger (41.7% between 41 and 50 years vs. 38.9% between 61 and 70 years). Most cases had sudden pain within two weeks. Unilateral PTS was present in 94.4% of mRNA and all viral vector-vaccinated cases. Symptoms included pain (97.1% and 92.3%, respectively), usually followed within two weeks by motor deficits (97.2% and 94.1%, respectively), amyotrophy (30% and 81.8%, respectively), paresthesia (50% and 27.3%, respectively), and sensory loss (33.3% and 38.5%, respectively). Viral vector vaccine recipients had nerve involvement outside the brachial plexus. Ancillary investigations revealed CSF albuminocytological dissociation (33.3% and 100%, respectively) and ipsilateral axillary lymphadenopathy. Two PTS cases worsened after the second mRNA dose, and another recurred after influenza vaccination. One patient well tolerated the second dose of the viral vector vaccine, but symptoms reemerged in another. Current evidence suggests PTS may occur after all COVID-19 vaccine types, with some subgroup differences. Also, PTS might recur with subsequent similar or unrelated vaccines.

Electrodiagnostic studies and new diagnostic modalities for evaluation of peripheral nerve disorders.

Electrodiagnostic studies (EDx) are frequently performed in the diagnostic evaluation of peripheral nerve disorders. There is increasing interest in the use of newer, alternative diagnostic modalities, in particular imaging, either to complement or replace established EDx protocols. However, the evidence to support this approach has not been expansively reviewed. In this paper, diagnostic performance data from studies of EDx and other diagnostic modalities in common peripheral nerve disorders have been analyzed and described, with a focus on radiculopathy, plexopathy, compressive neuropathies, and the important neuropathy subtypes of Guillain-Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), vasculitic neuropathy and diabetic neuropathy. Overall EDx retains its place as a primary diagnostic modality in the evaluated peripheral nerve disorders. Magnetic resonance imaging and ultrasound have developed important complementary diagnostic roles in compressive and traumatic neuropathies and atypical CIDP, but their value is more limited in other neuropathy subtypes. Identification of hourglass constriction in nerves of patients with neuralgic amyotrophy may have therapeutic implications. Investigation of radiculopathy is confounded by poor correlation between clinical features and imaging findings and the lack of a diagnostic gold standard. There is a need to enhance the literature on the utility of these newer diagnostic modalities.

Brachial neuralgic amyotrophy, a rare disease with serious diagnostic problems - case report

Neuralgic amyotrophy(NA), is a rare and highly variable neurological disorder of unknown aetiology that causes serious diagnostic problems. We described a case of NA with atrophy of the brachialis muscle in order to enrich clinicians’ knowledge about this disorder and to help them in the proper diagnosis of NA. A 21-year-old man presented with sudden, severe pain in the right biceps muscle. The man had consultations with several doctors and physiotherapists. Three magnetic resonance imaging (MRI) examinations, a nerve conduction study (NCS) and needle electromyography (EMG) were performed. Only after 6 months, based on medical history, physical examination and ultrasound imaging (USI), the physiotherapist suggested NA, which was later on confirmed by a neurologist. This case description of NA showed a variety of symptoms that made it difficult to establish an accurate diagnosis for a longer period of time. The diagnostic procedure, as well as the prolonged time between the onset of symptoms and diagnosis, indicates the need for further education of clinicians to better understand the symptoms of NA.

PAINLESS NEURALGIC AMYOTROPHY (PARSONAGE-TURNER SYNDROME) – A CASE REPORT

Background: Neuralgic amyotrophy (NA) is a rare disorder typically characterized by an abrupt onset of upper extremity pain followed by progressive muscle weakness, atrophy and occasional sensory loss. Although NA has been hypothesized to be an autoimmune-mediated disorder. It is considered a primarily clinical diagnosis, electrodiagnostic evaluation is essential for the diagnosis confirmation and can exclude other etiologies. Electrodiagnostic findings can reveal patchy damage to any nerve within the brachial plexus. Case Description: In the current case report, we are presenting a 43-years-old man admitted to the Neurology Department of “UMHAT Dr. Georgi Stranski” in Pleven, Bulgaria, with decreased muscle strength and limited active movements in the left upper shoulder for approximately 3 weeks. The patient denied feeling any pain during the onset and afterwards. He had no previous infections, vaccinations and history of other diseases. The detailed neurological examination showed the left upper extremity decreased antigravity strength in the deltoid and infraspinatus muscles with marked atrophy of the same. Hyporeflexia of the left biceps and brachioradialis deep‐tendon reflexes was present. Electromyography findings showed denervation of the deltoid and infraspinatus muscles. Initial reinnervation of supraspinatus and cervical paraspinal muscles was present. The diagnosis of NA was confirmed by both the neurological examination and the electrophysiological findings. Conclusion: We are presenting a clinical case of idiopathic neuralgic amyotrophy with atypical painless presentation and discussing the most significant aspects of the disorder with regards to the difficulties in approaching the correct diagnosis. A better understanding of the NA clinical symptoms and signs variability improves the diagnostic and therapeutic approach.

Neuralgic Amyotrophy As an Atypical Cause of Acute Shoulder Pain and Paralysis Following Hematopoietic Stem Cell Transplant

Neuralgic Amyotrophy As an Atypical Cause of Acute Shoulder Pain and Paralysis Following Hematopoietic Stem Cell Transplant

Neuralgic amyotrophy: diagnosis and treatment

Neuralgic amyotrophy: diagnosis and treatment

A patient with bilateral neuralgic amyotrophy and hepatitis E virus infection: an educational communication.

Neuralgic Amyotrophy, a peripheral nervous system disorder, is characterized by severe pain and muscle weakness, which can have a significant impact on patients' quality of life. The exact cause of neuralgic amyotrophy is unknown, but it may be linked to immunopathological mechanisms. Recent research has found an association between neuralgic amyotrophy and hepatitis E virus infection. This communication aims to expand knowledge on the clinical phenotype of patients with neuralgic amyotrophy and hepatitis E virus infection, presenting the case of a 55-year-old man diagnosed with bilateral neuralgic amyotrophy and hepatitis E virus infection.

Orthopnea secondary to brachial plexitis with bilateral diaphragmatic paralysis

BackgroundDiaphragmatic paralysis can present with orthopnea. We report a unique presentation of bilateral diaphragmatic paralysis, an uncommon diagnosis secondary to an unusual cause, brachial plexitis. This report thoroughly describes the patient’s presentation, workup, management, and outcome. It also reviews the literature on diaphragmatic paralysis and Parsonage-Turner syndrome.Case presentationA 50-year-old male patient developed insidious orthopnea associated with left shoulder and neck pain over three months with no associated symptoms. On examination, marked dyspnea was observed when the patient was asked to lie down; breath sounds were present and symmetrical, and the neurological examination was normal. The chest radiograph showed an elevated right hemidiaphragm. Echocardiogram was normal. There was a 63% positional reduction in Forced Vital Capacity and maximal inspiratory and expiratory pressures on pulmonary function testing. The electromyogram was consistent with neuromuscular weakness involving both brachial plexus and diaphragmatic muscle (Parsonage and Turner syndrome).ConclusionsCompared to unilateral, bilateral diaphragmatic paralysis may be more challenging to diagnose. On PFT, reduced maximal respiratory pressures, especially the maximal inspiratory pressure, are suggestive. Parsonage-Turner syndrome is rare, usually with unilateral diaphragmatic paralysis, but bilateral cases have been reported.

Neuralgic Amyotrophy and Hourglass Nerve Constriction/Nerve Torsion: Two Sides of the Same Coin? A Clinical Review.

Neuralgic amyotrophy, also called Parsonage-Turner syndrome, in its classic presentation is a brachial plexopathy or a multifocal neuropathy, involving mainly motor nerves of the upper limb with a monophasic course. Recently, a new radiological entity was described, the hourglass constriction, which is characterized by a very focal constriction of a nerve, or part of it, usually associated with nerve thickening proximally and distally to the constriction. Another condition, which is similar from a radiological point of view to hourglass constriction, is nerve torsion. The pathophysiology of neuralgic amyotrophy, hourglass constriction and nerve torsion is still poorly understood, and a generic role of inflammation is proposed for all these conditions. It is now widely accepted that nerve imaging is necessary in identifying hourglass constrictions/nerve torsion pre-surgically in patients with an acute mononeuropathy/plexopathy. Ultrasound and MRI are useful tools for diagnosis, and they are consistent with intraoperative findings. The prognosis is generally favorable after surgery, with a high rate of good motor recovery.

Bell Palsy and Parsonage-Turner Syndrome Following Pneumococcal Vaccination: A Case Report

Parsonage-Turner syndrome (PTS), also known as brachial neuritis, is a rare self-limiting, idiopathic condition characterized by sudden onset upper extremity pain with associated weakness and muscle atrophy. There exists a growing association between the development of PTS and infection/vaccinations in recent years. No case of PTS has been documented following pneumococcal vaccination to date. Bell palsy (BP) is an idiopathic disease process involving acute-onset unilateral acute facial paralysis resulting from edema and inflammation of the seventh cranial nerve. The etiology of BP is also largely unknown, but infectious processes, including viral reactivation and adverse vaccination reactions, have recently been documented. Although both PTS and BP are uncommon on their own, it is extremely rare for the 2 conditions to occur simultaneously. This case report presents a unique case of PTS and BP developing following the administration of the pneumococcal 20-valent conjugate vaccine.

Claw Hand in Parsonage Turner syndrome

An elderly patient with an extensive smoking history presented with acute severe right-hand pain and evolving weakness with patchy sensory deficit. He had minimal relief from multiple analgesics, gabapentin, and acupuncture. Motor deficits in his right-hand progressed. A nerve conduction study confirmed motor-predominant polyneuropathy and magnetic resonance imaging (MRI) of his C-spine and brachial plexus ruled out apical lung mass or other structural lesions. A positive GalNAc-GD1a ganglioside antibody (IgG) was noted. A diagnosis of immune mediated neuralgic amyotrophy or Parsonage-Turner Syndrome was made. Intravenous immune globulin (IVIG) was started with partial improvement in motor function, six months later. Although this patient did not have SARS-CoV-2 infection nor was he recently vaccinated, this case is of interest to hospital-based internists as the Covid pandemic has seen an increase in the in reported cases of neuralgic amyotrophy worldwide due to the virus itself as well as the associated COVID-19 vaccines.

Chapter 2 - The role of imaging in focal neuropathies

Electrodiagnostic testing (EDX) has been the diagnostic tool of choice in peripheral nerve disease for many years, but in recent years, peripheral nerve imaging has been used ever more frequently in daily clinical practice. Nerve ultrasound and magnetic resonance (MR) neurography are able to visualize nerve structures reliably. These techniques can aid in localizing nerve pathology and can reveal significant anatomical abnormalities underlying nerve pathology that may have been otherwise undetected by EDX. As such, nerve ultrasound and MR neurography can significantly improve diagnostic accuracy and can have a significant effect on treatment strategy. In this chapter, the basic principles and recent developments of these techniques will be discussed, as well as their potential application in several types of peripheral nerve disease, such as carpal tunnel syndrome (CTS), ulnar neuropathy at the elbow (UNE), radial neuropathy, brachial and lumbosacral plexopathy, neuralgic amyotrophy (NA), fibular, tibial, sciatic, femoral neuropathy, meralgia paresthetica, peripheral nerve trauma, tumors, and inflammatory neuropathies.

Chapter 16 - Focal inflammatory neuropathies

This chapter focuses on neuropathies that present with focal involvement of nerve roots, plexus, and/or peripheral nerves associated with autoimmune and inflammatory mechanisms that present with focal involvement of nerve roots, plexus and/or peripheral nerves. The clinical presentation, diagnosis, and treatment of focal autoimmune demyelinating neuropathies, focal nonsystemic vasculitic disorders (diabetic and nondiabetic radiculoplexus neuropathies, postsurgical inflammatory neuropathy, and neuralgic amyotrophy), and focal neuropathies associated with sarcoidosis and bacterial and viral infections are reviewed.

Chapter 8 - Axillary and musculocutaneous neuropathies

This chapter covers axillary and musculocutaneous neuropathies, with a focus on clinically relevant anatomy, electrodiagnostic approaches, etiologic considerations, and management principles. Disorders of the lateral antebrachial cutaneous nerve, a derivative of the musculocutaneous nerve, are also reviewed. We emphasize the importance of objective findings, including the physical examination and electrodiagnostic evaluation in confirming the isolated involvement of each nerve which, along with the clinical history, informs etiologic considerations. Axillary and musculocutaneous neuropathies are both rare in isolation and most frequently occur in the setting of trauma. Less commonly encountered etiologies include external compression or entrapment, neoplastic involvement, or immune-mediated disorders including neuralgic amyotrophy, postsurgical inflammatory neuropathy, multifocal motor neuropathy, vasculitic neuropathy, and multifocal chronic inflammatory demyelinating polyradiculoneuropathy.

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