Neuralgic Amyotrophy Research Articles

Hepatitis E virus-associated brachial neuritis presenting with orthopnoea as a result of bilateral diaphragmatic weakness

British Journal of Hospital MedicineVol. 84, No. 2 Case ReportHepatitis E virus-associated brachial neuritis presenting with orthopnoea as a result of bilateral diaphragmatic weaknessSamuel Mackrill, Prithwiraj Chakrabarti, Richard Butterworth, Veeresh PatilSamuel MackrillCorrespondence to: Samuel Mackrill; E-mail Address: [email protected]Department of Medicine, Milton Keynes University Hospital, Milton Keynes, UKSearch for more papers by this author, Prithwiraj ChakrabartiDepartment of Medicine, Milton Keynes University Hospital, Milton Keynes, UKSearch for more papers by this author, Richard ButterworthDepartment of Medicine, Milton Keynes University Hospital, Milton Keynes, UKSearch for more papers by this author, Veeresh PatilDepartment of Medicine, Milton Keynes University Hospital, Milton Keynes, UKSearch for more papers by this authorSamuel Mackrill; Prithwiraj Chakrabarti; Richard Butterworth; Veeresh PatilPublished Online:9 Feb 2023https://doi.org/10.12968/hmed.2022.0280AboutSectionsView articleView Full TextPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareShare onFacebookTwitterLinked InEmail View article References McLean BN, Gulliver J, Dalton HR. Hepatitis E virus and neurological disorders. Pract Neurol. 2017;17(4):282–288. https://doi.org/10.1136/PRACTNEUROL-2016-001588 Crossref, Medline, Google ScholarNoushad MA, Limnatitou D, Bhattacharjee S, Mohd Nor A. Diaphragmatic paralysis resulting in respiratory failure as a feature of hepatitis E virus-associated neuralgic amyotrophy. BMJ Case Rep. 2021;14(4):e242113. https://doi.org/10.1136/BCR-2021-242113 Crossref, Medline, Google ScholarShinder N, Polson A, Pringle E, O'Donnell DE. Neuralgic amyotrophy: a rare cause of bilateral diaphragmatic paralysis. Can Respir J. 1998;5(2):139–142. https://doi.org/10.1155/1998/926375 Crossref, Medline, Google Scholarvan Eijk JJJ, Dalton HR, Ripellino P et al.. Clinical phenotype and outcome of hepatitis E virus-associated neuralgic amyotrophy. Neurology. 2017;89(9):909–917. https://doi.org/10.1212/WNL.0000000000004297 Crossref, Medline, Google Scholar FiguresReferencesRelatedDetails 2 February 2023Volume 84Issue 2ISSN (print): 1750-8460ISSN (online): 1759-7390 Metrics History Published online 9 February 2023 Published in print 2 February 2023 Information© MA Healthcare LimitedPDF download

Effectiveness of an outpatient rehabilitation programme in patients with neuralgic amyotrophy and scapular dyskinesia: a randomised controlled trial

BackgroundNeuralgic amyotrophy (NA) is an acute inflammation of nerves within the brachial plexus territory leading to severe pain and multifocal paresis resulting in >60% of patients having residual complaints and...

Diagnosis and treatment of phrenic nerve hourglass constriction in patients with Parsonage-Turner syndrome.

Phrenic nerve injury can occur anywhere along its course and clinically results in diaphragm paralysis. Although most patients with Parsonage-Turner syndrome and phrenic nerve dysfunction improve without treatment, some patients do not recover spontaneously. In these cases, an initial autoimmune response produces scarring along the affected nerve(s). This scar, known as an hourglass constriction, causes focal compression of the nerve at the site of the scar, which prevents the nerve from spontaneously recovering. Thus, the authors present a unique case of phrenic nerve injury secondary to Parsonage-Turner syndrome that improved with internal neurolysis. The video can be found here: https://stream.cadmore.media/r10.3171/2022.10.FOCVID22105.

Distal peripheral nerves may be involved in Parsonage Turner syndrome

Distal peripheral nerves may be involved in Parsonage Turner syndrome

Needle electromyography abnormalities in the upper trapezius muscle in neuromuscular disorders.

: Needle electromyography (EMG) abnormalities in the trapezius muscle (TM) can be seen in neuromuscular disorders. The aim was to determine the characteristics of needle EMG abnormalities observed in the TM in neuromuscular disorders. The data of patients who applied to the Clinical Neurophysiology Laboratory of University of Health Sciences Adana City Training and Research Hospital between December 2018 and October 2021 were reviewed. Polio survivors, amyotrophic lateral sclerosis (ALS) patients, patients with sensorimotor polyneuropathy, patients with spinal cord lesions involving C2/C3/C4 segments, patients with spinal accessory nerve (SAN) lesions, neuralgic amyotrophy (NA) patients, and patients with myopathy were included. Needle EMG findings of the upper TM of the patients were analyzed. Positive sharp waves, fibrillation potentials, fasciculation potentials, myotonic discharges, and motor unit action potential (MUAP) changes were considered needle EMG abnormalities. Eighty-one polio survivors, 23 ALS patients, 39 patients with sensorimotor polyneuropathy, 10 patients with cervical spinal lesions, eight NA patients, seven patients with SAN lesions, and three patients with myopathy were included in the study. Fifteen (65.2%) ALS patients, 18 (22.2%) polio survivors, three (30%) patients with cervical spinal lesions, two (5.1%) patients with sensorimotor neuropathy, one (12.5%) NA patient, seven (100%) patients with SAN lesions, and two (66.7%) patients with myopathies had at least one needle EMG abnormality in the TM. Fasciculation potentials in the TM were seen in 10 (43.5%) ALS patients. In four patients with SAN lesions and one polio survivor, MUAP could not be obtained from the TM. There may be more frequent needle EMG abnormalities, particularly in ALS patients and patients with SAN lesions. Since the number of patients with myopathy included in this study was low, it is difficult to comment on the needle EMG features of the TM for these patients. In addition, this study indicated that fasciculation potentials in the TM are typical in ALS patients and that MUAP may not be obtained from the TM in patients with SAN lesions.

Chapter 24 - Autoimmune polyneuropathies

The autoimmune peripheral neuropathies with prominent motor manifestations are a diverse collection of unusual peripheral neuropathies that are appreciated in vast clinical settings. This chapter highlights the most common immune-mediated, motor predominant neuropathies excluding acute, and chronic inflammatory demyelinating polyradiculoneuropathy (AIDP and CIDP, respectively). Other acquired demyelinating neuropathies such as distal CIDP and multifocal motor neuropathy will be covered. Additionally, the radiculoplexus neuropathies, resulting from microvasculitis-induced injury to nerve roots, plexuses, and nerves, including diabetic and nondiabetic lumbosacral radiculoplexus neuropathy and neuralgic amyotrophy (i.e., Parsonage-Turner syndrome), will be included. Finally, the motor predominant peripheral neuropathies encountered in association with rheumatological disease, particularly Sjögren's syndrome and rheumatoid arthritis, are covered. Early recognition of these distinct motor predominant autoimmune neuropathies and initiation of immunomodulatory and immunosuppressant treatment likely result in improved outcomes.

Parsonage-Turner Syndrome and COVID-19 Vaccine: Correspondence.

Parsonage-Turner Syndrome and COVID-19 Vaccine: Correspondence.

A case of neuralgic amyotrophy after vaccination against COVID-19

A case of neuralgic amyotrophy after vaccination against COVID-19

Cerebral Adaptation Associated with Peripheral Nerve Recovery in Neuralgic Amyotrophy: A Randomized Controlled Trial

Background: Neuralgic amyotrophy (NA) is a common peripheral nerve disorder caused by auto-immune inflammation of nerves in the brachial plexus territory, characterized by acute pain and weakness of the shoulder muscles, followed by motor impairment. Recent work has confirmed that NA patients with residual motor dysfunction have abnormal cerebral sensorimotor representations of their affected upper extremity. Objective: To determine whether abnormal cerebral sensorimotor representations associated with NA can be altered by specialized, multidisciplinary outpatient rehabilitation focused on relearning motor control. Methods: 27 NA patients with residual lateralized symptoms in the right upper extremity participated in a randomized controlled trial, comparing 17 weeks of multidisciplinary rehabilitation (n = 16) to usual care (n = 11). We used task-based functional MRI and a hand laterality judgment task, which involves motor imagery and is sensitive to altered cerebral sensorimotor representations of the upper extremity. Results: Change in task performance and related brain activity did not differ significantly between the multidisciplinary rehabilitation and usual care groups, whereas the multidisciplinary rehabilitation group showed significantly greater clinical improvement on the Shoulder Rating Questionnaire. Both groups, however, showed a significant improvement in task performance from baseline to follow-up, and significantly increased activity in visuomotor occipito-parietal brain areas, both specific to their affected upper extremity. Conclusions: Abnormal cerebral sensorimotor representations of the upper extremity after peripheral nerve damage in NA can recover toward normality. As adaptations occurred in visuomotor brain areas, multidisciplinary rehabilitation after peripheral nerve damage may be further optimized by applying visuomotor strategies. This study is registered at ClinicalTrials.gov (NCT03441347).

Evaluation Of Antibody Response To Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) Vaccination In Patients With Lymphoid And Solid Organ Malignancies

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is responsible for the current COVID-19 pandemic. There is emerging evidence regarding suboptimal response to vaccination against COVID-19 in patients with hematologic and solid organ malignancies. We conducted a single-center prospective study assessing seroconversion in response to vaccination against COVID-19 in 53 patients with chronic lymphocytic leukemia (CLL), non-Hodgkin’s lymphoma (NHL), multiple myeloma (MM), and solid organ malignancies. A quantitative immunoassay of IgG antibodies to SARS-CoV-2 Spike (S) protein was measured prior to vaccination and at 2 weeks after completion of two-dose vaccination series. A fourfold increase in antibody titers was considered positive seroconversion. Through a predesigned survey, patients also self-reported side effects from each dose of vaccination. Seroconversion on vaccination was seen in 6/12 (50%) patients with CLL, 7/11 (63.6%) patients with NHL, 9/10 (90%) patients with MM, and 17/20 (85%) patients with solid organ malignancy. Only 6 of the 14 (42.8%) patients currently on or with previous history of rituximab use seroconverted. Injection site soreness was the most reported side effect. The only severe side effect occurred in a patient with solid organ malignancy who developed Parsonage-Turner syndrome. Patients with CLL and NHL appear less likely to respond to vaccination against COVID-19 in contrast to patients with MM or solid organ malignancies. Previous treatment with rituximab is a possible risk factor for suboptimal response to vaccination. These data highlight the importance of continuing risk mitigation strategies against COVID-19 in individuals with hematologic malignancy, particularly those with CLL or on treatment with rituximab.

Bilateral Parsonage-Turner syndrome as a complication ofan unknown hepatitis E infection.

Bilateral Parsonage-Turner syndrome as a complication ofan unknown hepatitis E infection.

The role of ultrasonography in diagnosis of neuralgic amyotrophy.

To investigate the value of high frequency ultrasound in diagnosis of neuralgic amyotrophy. From January 2010 to December 2020, the ultrasonographic images of 117 patients with neuralgic amyotrophy diagnosed by the Department of Neurology and hand & foot surgery of Shandong Provincial Hospital Affiliated to Shandong First Medical University were retrospectively analyzed. The ultrasonographic features were summarized. High frequency ultrasound could clearly show the degree of the affected nerves: No ultrasonic findings were found in 12 cases (10%). The affected nerves were thickening and hypoechogenicity with loss of normal fascicular definition in 28 cases (24%). The affected nerves showed hourglass-like changes, including constriction and torsion in 77 cases (66%). In addition, ultrasound can determine the extent of the lesion, and microvascular imaging can display small blood flow signal within the nerve. There was a significant statistical difference between the diameter of the thickened nerve fascicle and the diameter of the nerve fascicle at the corresponding site of the contralateral normal limb. High frequency ultrasound is a valuable imaging method for diagnosis of neuralgic amyotrophy.

Neuralgic Amyotrophy After COVID-19 Vaccination in an Adolescent: Successful Intravenous Immunoglobulin Treatment

Neuralgic amyotrophy, or Parsonage-Turner syndrome, is a neurological condition characterized by sudden pain attacks followed by patchy muscle paresis in the upper extremities. It is uncommon in children, and symptoms are generally preceded by triggering events, such as infection, surgery, and vaccination. [1] Neuralgic amyotrophy after coronavirus disease-2019 (COVID-19) vaccination has been reported in adults, but not (0/3,449) in children <18 years old. [2]

COVID-19 vaccine-related neuralgic amyotrophy

COVID-19 vaccine-related neuralgic amyotrophy

Neuralgic amyotrophy detected by magnetic resonance neurography: subclinical, bilateral, and multifocal brachial plexus involvement

ABSTRACT Introduction Neuralgic amyotrophy (NA) is a painful non-traumatic peripheral nervous system condition affecting the brachial plexus. Signal abnormalities in nerves and muscles have been detected in these patients using magnetic resonance neurography (MRN). Methods Electronic medical records and MRN images obtained in a 3 T scanner, in 14 adult patients diagnosed with NA at our Neurological institution (Neuromuscular Disorders Section), between December 2015 and December 2019 were retrospectively reviewed. The study was first approved by our Institutional Ethics Committee. Results Subclinical, multifocal, and bilateral nerve signal anomalies were recorded in the brachial plexus of these patients. We identified four different types of nerve constriction without entrapment, which we categorized as follows: incomplete focal (type I), complete focal or hourglass (type II), multifocal or string of pearls (type III) and segmental (type IV). Conclusions Given that MRN is an accurate diagnostic tool to detect nerve damage, we believe abnormal findings could improve early detection of NA patients.

Associations of neuralgic amyotrophy with COVID-19 vaccination: Disproportionality analysis using the World Health Organization pharmacovigilance database.

There are limited studies on the association of COVID-19 vaccination with neuralgic amyotrophy (NA). Therefore, we evaluated the association between COVID-19 vaccination and the occurrence of NA. We explored unexpected safety signals for NA related to COVID-19 vaccination through disproportionality analysis using VigiBase, the World Health Organization's pharmacovigilance database. On October 15, 2021, 335 cases of NA were identified in the database. The median time to onset of NA after vaccination was around 2 weeks. A significant signal of disproportionality of NA was observed for the ChAdOx1 nCoV-19 vaccine (AstraZeneca) (information component [IC]025 =0.33, reporting odds ratio [ROR]025 =1.30) and two mRNA-based COVID-19 vaccines (BNT162b2 [Pfizer and BioNTech] and mRNA-1273 [Moderna]) (IC025 =1.74, ROR025 =3.82) compared with the entire database. However, when compared with influenza vaccines, we did not detect any signal of disproportionality of NA for both the ChAdOx1 nCoV-19 vaccine (IC025 =-2.71, ROR025 =0.05) and mRNA-based COVID-19 vaccines (IC025 =-1.38, ROR025 =0.13). A weak association was observed between NA and COVID-19 vaccines. However, the risk did not surpass that of influenza vaccines.

Imaging of neuralgic amyotrophy in the acute phase.

Hourglass-like constrictions (HGCs) occur in neuralgic amyotrophy (NA), but the earliest time at which they can be recognized by imaging is poorly understood. We aimed to determine the prevalence of abnormal imaging findings in the acute phase of NA. Magnetic resonance neurography (MRN) and high-resolution ultrasound (US) examinations were performed at five sites. The investigation included 39 patients with acute NA who underwent imaging within 31 days of symptom onset. Correlation between imaging and electromyography (EMG) findings was measured. US was performed in 29 patients and MRN in 23; 16 patients underwent US only, 10 MRN only, and 13 had both. US and MRN showed nerve abnormalities within 1mo from NA onset in 90% of patients. HGCs were found in 74% (29/39) of the patients: 4 within 1wk, 8 within 2wk, 5 within 3wk, and 12 within 4wk. The earliest HGC on US was found within 12 h, and on MRN within 3 days from symptom onset. MRN demonstrated a denervation edema pattern of affected muscles in 91% of the patients. The shortest time to observe an edema pattern on MRN was 8 days. EMG was performed in 30 patients and revealed fibrillation potentials in affected muscles in 22 (73%). A denervation edema pattern on MRN was significantly associated with the presence of HGCs both on MRN and US, and with fibrillation potentials on EMG. In the early phase of NA, US and MRN are useful diagnostic techniques for demonstrating nerve abnormalities.

Double Fascicular Nerve Transfer to Restore Elbow Flexion Following Neuralgic Amyotrophy: A Case Report

Double fascicular nerve transfer (DFNT) involves transferring fascicles from the median and ulnar nerves to the musculocutaneous nerve to restore elbow flexion. We performed this procedure on a patient who was diagnosed with neuralgic amyotrophy (NA) 2 years previously. We aimed to investigate the short-term outcomes and feasibility of DFNT applied to non-traumatic neuropathy. A 63-year-old, right-handed man presented to us with persistent right elbow flexion weakness 24 months after receiving the diagnosis of NA. On examination, biceps strength was 0/5, and shoulder abduction strength was 3/5. Magnetic resonance neurography demonstrated muscle atrophy in the upper trunk. DFNT was considered to restore elbow flexion. The donor and recipient fascicles were then coapted (flexor digitorum superficialis to biceps brachii, flexor carpi ulnaris to brachialis). Postoperatively, paresthesia was reported in the area of ulnar innervation, and the patient complained of hand clumsiness. From postoperative day 10 onward, biceps muscle contracture was identified and on the 14th day, grade 3 elbow flexion strength was observed. We suggest that DFNT may be a safe and effective method to restore elbow flexion in patients with NA.

Diagnostic difficulties in a paediatric patient with shoulder pain: Parsonage-Turner syndrome – Case Report

Diagnostic difficulties in a paediatric patient with shoulder pain: Parsonage-Turner syndrome – Case Report

Surgical Treatment of Parsonage Turner Syndrome With Primary Nerve Transfers: A Case Series and Cadaver Dissection.

Parsonage Turner syndrome (PTS) is the development of severe, spontaneous pain with subsequent nerve palsy. Unfortunately, many patients never achieve full functional recovery, and many have chronic pain. The use of nerve transfers in PTS has not been reported in the literature. We present 4 cases of PTS treated surgically with primary nerve transfer and neurolysis of the affected nerve following the absence of clinical and electrodiagnostic recovery at 5 months from onset. In addition, we present a cadaver dissection demonstrating an interfascicular dissection of the anterior interosseous nerve (AIN) into its components to enable a fascicular transfer in partial AIN neuropathy. Two patients with complete axillary neuropathy underwent a neurorrhaphy between the nerve branch to the lateral head of the triceps and the anterior/middle deltoid nerve branch of the axillary nerve. Two patients with partial AIN neuropathy involving the FDP to the index finger (FDP2) underwent a neurorrhaphy between an extensor carpi radialis brevis nerve branch and the FDP2 nerve branch. All patients had neurolysis of the affected nerves. All subjects recovered at least M4 motor strength. The cadaver dissection demonstrates 3 separate nerve fascicles of the AIN into FPL, FDP2, and pronator quadratus that can be individually selected for reinnervation with a fascicular nerve transfer. Functional recovery for patients with PTS with neurolysis alone is variable. Surgical treatment with neurolysis and a nerve transfer to improve functional recovery when no recovery is seen by 5 months is an option.