COVID-19 Vaccine Related Cervical Radiculitis and Parsonage-Turner Syndrome: Case Report and Review of the Literature.

Parsonage-Turner Syndrome, or neuralgic amyotrophy, is an acute-onset upper limb and shoulder girdle palsy that can occur in a post-viral, post-surgical or idiopathic setting. There have also been some reported cases of the syndrome occurring following vaccinations. The pathophysiology of neuralgic amyotrophy is not completely understood and many of the commonly used diagnostic imaging modalities we use to try and diagnose this syndrome are inaccurate and misleading. We present the case of a 40-year-old gentleman who presented with acute onset burning pain and fasciculations in his right upper extremity following vaccination with the second dose of the Pfizer-BioNTech COVID-19 vaccine. His symptoms progressed to weakness in isolated muscle groups with electromyographic evidence of decreased nerve conduction. MRI of the cervical spine demonstrated multilevel central and foraminal stenosis, suggesting a diagnosis of cervical radiculopathy. The patient underwent a C4-5/C5-6 and C6-7 laminoforaminotomy and tolerated the procedure well. Post-operatively, the patient has experienced gradual symptom improvement with residual right triceps and pectoralis muscle weakness as well as paresthesias of the right elbow and forearm. Parsonage-Turner Syndrome is a brachial plexus palsy that can affect one or multiple branches of the brachial plexus. It causes acute-onset pain and weakness, and the diagnosis can be difficult to make with the commonly used diagnostic imaging methods. We reviewed other case reports about neuralgic amyotrophy following vaccinations as well as the current literature on more accurate diagnostic imaging modalities that may help our diagnosis and understanding of the pathophysiology of this condition.

Orthopnea secondary to brachial plexitis with bilateral diaphragmatic paralysis.

BACKGROUND: Diaphragmatic 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 PRESENTATION: A 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). CONCLUSIONS: Compared 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.

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.

[Neuralgic amyotrophy: an inflammatory neuropathy and its surgical treatment]. / Neuralgische Amyotrophie ­ eine entzündliche Neuropathie und ihre operative Behandlung.

BACKGROUND: Neuralgic amyotrophy (NA) is a monofocal or oligofocal inflammatory neuropathy whose incidence has been significantly underestimated. A connection between constrictions and torsions of peripheral nerves with this disease has been increasingly established in recent years. Modern imaging techniques such as high-resolution nerve ultrasound and MR neurography have contributed to a better understanding of the pathophysiology and a better assessment of the prognosis of the disease. This has led to the concept of treating patients with such focal changes surgically in order to improve the prognosis. This review presents current ideas on the pathophysiology, clinical presentation, diagnosis and treatment of the disease. PATIENTS AND METHODS: In a retrospective study, pre-, intra- and postoperative findings of 22 patients with 23 constrictions/torsions of peripheral nerves of the upper extremity were analysed. The patients underwent surgery at a nerve surgery centre over a period of 3.5 years (Dec. 2019-May 2023). The median nerve was most frequently affected (N=9), followed by the suprascapular nerve (N=6) and radial nerve (N=4). The axillary nerve (N=3) and the accessory nerve (N=1) were also involved. Surgical exploration revealed nerve torsions (N=9), nerve constrictions (N=5), fascicular torsions (N=12) and fascicular constrictions (N=9). Depending on the intraoperative findings, epineuriotomies (N=1), epi- and perineuriotomies (N=33), end-to-end sutures (N=2), and one epi- and one perineural suture were performed. RESULTS: After an average follow-up of 10 months (3-28 months), 17 patients were re-examined. All of them reported a clear subjective improvement in motor deficits. Clinically and electromyographically, a reinnervation and significant increase in strength from a pre-existing strength grade of M0 to at least M3 in the vast majority of affected muscles was demonstrated in these patients. SUMMARY: The incidence of NA continues to be underestimated and, in a significant proportion of patients, leads to permanent motor deficits, most likely due to constrictions and torsions of affected nerves. Surgical treatment is recommended as early as possible. Very good results can usually be achieved with epi- and perineuriotomy. In rare cases, end-to-end neurorrhaphy or nerve grafting is required.

[MR neurography reveals fascicular constriction of the median nerve in a patient with neuralgic amyotrophy].

Diagnosing neuralgic amyotrophy can be challenging in clinical practice. Here, we report the case of a 37-years old Japanese woman who suddenly developed neuropathic pain in the right upper limb after influenza vaccination. The pain, especially at night, was severe and unrelenting, which disturbed her sleep. However, X-ray and MRI did not reveal any fractures or muscle injuries, and brain MRI did not reveal any abnormalities. During neurological consultation, she was in a posture of flexion at the elbow and adduction at the shoulder. Manual muscle testing suggested weakness of the flexor pollicis longus, pronator quadratus, flexor carpi radialis (FCR), and pronator teres (PT), while the flexor digitorum profundus was intact. Medical history and neurological examination suggested neuralgic amyotrophy, particularly anterior interosseous nerve syndrome (AINS) with PT/FCR involvement. Innervation patterns on muscle MRI were compatible with the clinical findings. Conservative treatment with pain medication and oral corticosteroids relieved the pain to minimum discomfort, whereas weakness remained for approximately 3 months. For surgical exploration, lesions above the elbow and fascicles of the median nerve before branching to the PT/FCR were indicated on neurological examinations; thus, we performed high-resolution imaging to detect possible pathognomonic fascicular constrictions. While fascicular constrictions were not evident on ultrasonography, MR neurography indicated fascicular constriction proximal to the elbow joint line, of which the medial topographical regions of the median nerve were abnormally enlarged and showed marked hyperintensity on short-tau inversion recovery. In patients with AINS, when spontaneous regeneration cannot be expected, timely surgical exploration should be considered for a good outcome. In our case, MR neurography was a useful modality for assessing fascicular constrictions when the imaging protocols were appropriately optimized based on clinical assessment.

Neurological manifestation of HEV infection: still a rare disease entity?

Hepatitis E virus (HEV) infection is the most common form of viral hepatitis and is reported to cause neurological manifestation in up to 30% of diagnosed infections. We evaluated the medical reports of all patients (n = 29,994) who were discharged from the Department of Neurology of Ulm University between 01.01.2015 and 30.09.2022 to detect neurological manifestations of HEV. In addition, we retrospectively analyzed the serum samples of n = 99 patients representing different neurological diseases possibly related to HEV for anti-HEV-IgM and anti-HEV-IgG. At the time of discharge from hospital, the etiology of neurological symptoms in these patients was unclear. Overall, five cases of extrahepatic neurological manifestation of HEV (defined as anti-HEV-IgM and HEV-IgG positive) could be detected. An increase of both, anti-IgM- and anti-IgG-serum levels was significantly more common in neuralgic amyotrophy/plexus neuritis/radiculitis than in AIDP/CIDP (P = 0.01), meningitis/encephalitis (P = 0.02), idiopathic peripheral facial paralysis (P = 0.02) and tension headache (P = 0.02). In 15% (n = 15 out of 99) of retrospectively analyzed serum samples, conspicuous positive anti-HEV-IgG levels were detected. This finding was most common in AIDP/CIDP. In conclusion, results of this study indicate neurological manifestation of HEV to be a rare but still underestimated course of disease, occurring at any age and gender. Therefore, testing for HEV should be considered in patients with neurological symptoms of unknown origin, especially in those with neuralgic amyotrophy/plexus neuritis.

Effects of indirect foraminal decompression during anterior cervical disc fusion procedure: preliminary results of a prospective study with clinical and radiological outcomes.

INTRODUCTION: The respective effects of direct and indirect decompression in the clinical outcome after anterior cervical disc fusion (ACDF) is still debated. The main purpose of this study was to analyze the effects of indirect decompression on foraminal volumes during ACDF performed in patients suffering from cervico-brachial neuralgias due to degenerative foraminal stenosis, i.e. to determine whether implant height was associated with increased postoperative foraminal height and volume. METHODS: A prospective follow-up of patients who underwent ACDF for cervicobrachial neuralgias due to degenerative foraminal stenosis was conducted. Patient had performed a CT-scan pre and post-operatively. Disc height, foraminal heights and foraminal volumes were measured pre and post operatively. RESULTS: 37 cervical disc fusions were successfully performed in 20 patients, with a total of 148 foramina studied. Foraminal height and volume were measured bilaterally on the pre- and post-operative CT scans (148 foramina studied). After univariate analysis, it was found a significant improvement for every radiological parameter, with a significant increase in disc height, foraminal height and foraminal volume being respectively +3,22 mm (p < 0,001), +2,12 mm (p < 0,001) and +54 mm3 (p < 0,001). Increase in disc height was significantly associated with increase in foraminal height (p < 0,001) and foraminal volume (p < 0,001). At the same time, increase in foraminal height was significantly correlated with foraminal volume (p < 0,001), and seems to be the major component affecting increasing in foraminal volume. CONCLUSION: Indirect decompression plays an important part in the postoperative foraminal volume increase after ACDF performed for cervicobrachial neuralgias.

[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.

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.

Neuralgic amyotrophy: sensitivity and specificity of magnetic resonance neurography in diagnosis: A retrospective study.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Persistent Hourglass Constriction in Recovered Anterior Interosseous Nerve Palsy Due to Neuralgic Amyotrophy: A Case Report.

The current articles recommended the interfascicular neurolysis for anterior interosseous nerve (AIN) palsy with hourglass-like fascicular constrictions (FCs) detected by ultrasonography or surgical exploration to realign to the fascicular torsion for those who failed to recover spontaneously. We present the case report of spontaneous AIN palsy recovered after conservative treatment; however, ultrasonographic findings showed persistent FCs of AIN in the arm at the beginning, at 6 weeks, and subsequent 3-year follow-ups, even after complete clinical recovery of palsy. This finding calls into question the current notion that AIN paralysis is due to FCs and that neurolysis is the best surgical treatment when spontaneous recovery does not occur for a considerable observation period. Level of Evidence: Level V (Therapeutic).

[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.

Hereditary Neuralgic Amyotrophy with a Lesion Distal to the Brachial Plexus on Magnetic Resonance Imaging.

A 35-year-old woman first experienced left upper limb weakness at 17 years old, after which it repeatedly recurred and then remitted. She was diagnosed with carpal tunnel syndrome with median nerve hyperintensity by magnetic resonance imaging (MRI). Surgical treatment was ineffective. We suspected hereditary neuralgic amyotrophy because of enlargement distal to the brachial plexus on MRI and administered steroid therapy, after which the weakness improved. Genetic testing revealed a point mutation in SEPT9. Because lesions outside the brachial plexus can be seen in hereditary neuralgic amyotrophy, the diagnosis should be based on typical characteristics and the family history.

Association of Parsonage-Turner syndrome with COVID-19 infection and vaccination: a systematic review.

OBJECTIVES: The exact etiology of Parsonage-Turner syndrome is unknown, but it is known to be preceded by infection, vaccination, or surgical intervention. In this review, we describe associations of Parsonage-Turner syndrome with COVID-19 infection and vaccination. METHODS: A systematic literature search was conducted using PubMed/MEDLINE, ScienceDirect, and Google Scholar. Microsoft Excel was used for data extraction and statistical analysis. The quality of case reports and case series was assessed using the Joanna Briggs Institute Critical Appraisal Tool. RESULTS: We selected 44 case reports and 10 case series, including 68 patients (32 post-vaccination and 36 with post-COVID-19 infection Parsonage-Turner syndrome). Middle-aged males were predominantly affected in both groups. The most frequently administered vaccine was Comirnaty (Pfizer) (53%). The mean latency was 11.7 days in the post-vaccination group and 20.3 days in the post-infection group. The most affected nerves in both groups were the axillary, suprascapular, and musculocutaneous nerves; and 78.1% and 38.9% of patients showed partial amelioration of their symptoms in the post-vaccination and post-infection groups, respectively. CONCLUSION: Post-vaccination Parsonage-Turner syndrome presents earlier than post-infection disease. Pain and sensorimotor deficits of the upper limb are common in both situations. Complete or partial recovery occurs in most cases.

Increased 18 F-FDG uptake in denervated muscles in a case of Parsonage-Turner syndrome.

BACKGROUND: Parsonage-Turner Syndrome (PTS) is a rare brachial plexopathy characterized by the sudden onset of pain in the shoulder girdle followed by upper limb weakness. PTS is frequently under-recognized or misdiagnosed as other more common neurological disorders presenting in a similar fashion, such as cervical radiculopathy which may require surgical intervention. Accurate diagnosis and prompt management implicate a good prognosis. Although electrophysiological studies are considered the most important for evaluating peripheral nerve injuries, it usually takes time, up to 3 weeks after the initial insult of the nerve for electromyogram (EMG) and nerve conduction studies (NCS) to display abnormalities. In the cases of PTS, especially when initial EMG/NCS and magnetic resonance neurography (MRN) results are inconclusive, 18 F-FDG positron emission tomography and computed tomography (18 F-FDG PET-CT) may be useful in helping the early detection of muscle denervation. CASE PRESENTATION: A 60-year-old right-handed Taiwanese woman presented with sudden onset of intense and sharp left shoulder girdle pain without radiating to the arm, followed by muscle weakness of her left arm in abduction and elevation 3 days after the onset of pain. A detailed neurological examination and EMG and NCS suggested the clinical diagnosis of left brachial plexopathy. MRN imaging revealed no significant abnormality. 18 F-FDG PET-CT showed increased uptake in denervated muscles (supraspinatus, deltoid, and biceps muscles). Treatment with oral prednisolone and physiotherapy significantly improved pain and muscle weakness. CONCLUSIONS: We present increased 18 F-FDG uptake in denervated muscles detected by 18 F-FDG PET-CT. 18 F-FDG PET-CT may serve as an adjunct examination to evaluate PTS, which has been suggested previously but rarely reported.