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.

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.

[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.

[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.

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.

A West Nile Virus infection expressed as unilateral limb paralysis and complicated by Parsonage-Turner syndrome: a case report.

BACKGROUND: West 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 PRESENTATION: In 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. CONCLUSIONS: West 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.

[A 24-year-old female with acute onset shoulder pain]. / Een 24-jarige vrouw met acute schouderpijn.

A 24-year-old female presents with sudden debilitating right shoulder pain that started 5 days ago, with reduced range of motion (ROM) in shoulder abduction and a scapula alata. These findings are indicative of neuralgic amyotrophy (NA). NA usually has a diagnostic delay which complicates treatment. Long-term impact can be severe which sometimes justifies referral to a physiatrist.

Ultrasound Diagnostic and Physiotherapy Approach for a Patient with Parsonage-Turner Syndrome-A Case Report.

Parsonage-Turner syndrome (PTS) is a rare neurological disorder that causes major diagnostic problems. This paper presents a case report of a patient with PTS and proposes a new physiotherapy program. CASE DESCRIPTION: a 23-year-old man presents a sudden severe pain of his right arm. The man is consulted by several doctors and physiotherapists. Three magnetic resonance imagings (MRI), a nerve conduction study (NCS), and needle electromyography (EMG) are performed. After 6 months, based on medical history, physical examination and ultrasound imaging (UI), the physiotherapist suggests PTS, which is confirmed by a neurologist. INTERVENTION: due to the lack of physiotherapy treatment standards in PTS, we apply neurodynamic techniques. OUTCOMES: neurodynamic techniques are effective in reducing pain and paraesthesia, improving sensation, and reducing nerve swelling (assessed by UI), as well as improving manual dexterity and overall health status. CONCLUSIONS: the patient with PTS is challenging for making an accurate diagnosis. This study shows an important role for UI, which shows changes in the musculocutaneous nerve, despite the lack of abnormalities in the MRI, NCS, and EMG, and helps in making an accurate diagnosis. This report also confirms that physiotherapy based on neurodynamic techniques may have beneficial effects in PTS.

Parsonage-Turner Syndrome mimicking musculoskeletal shoulder pain: A case report during the SARS-CoV-2 pandemic era.

Parsonage-Turner Syndrome or neuralgic amyotrophy is a peripheral neuropathy typically characterized by an abrupt onset of pain, followed by progressive neurological deficits (e.g. weakness, atrophy, occasionally sensory abnormalities) that involve the upper limb, mainly the shoulder, encompassing an extensive spectrum of clinical manifestations, somehow difficult to recognize. This case report describes the proper management of a 35-year-old, bank employee and sports amateur who reported subtle and progressive upper limb disorder with previous history of neck pain. SARS-CoV-2 pandemic era made patient's access to the healthcare system more complicated. Nevertheless, proper management of knowledge, relevant aspects of telerehabilitation-based consultation for musculoskeletal pain, advanced skills, tools and technologies led the physiotherapist to suspect an atypical presentation of Parsonage-Turner Syndrome. Further, neurologist consultation and electromyography suggested signs of denervation in the serratus anterior and supraspinatus muscle. Therefore, an appropriate physiotherapist's screening for referral is conducted to correct diagnosis and thorough treatment.

Un caso de neuralgia amiotrófica tras vacunación frente a COVID-19 / A case of neuralgic amyotrophy after vaccination against COVID-19

Introducción: La neuralgia amiotrófica es un trastorno inflamatorio del plexo braquial con una fisiopatología poco conocida, posiblemente relacionada con fenómenos inmunológicos. Se caracteriza por dolor agudo e intenso, acompañado de debilidad muscular y alteración de los resultados en los estudios electromiográficos y de conducción nerviosa. Al no tener un tratamiento específico, se aborda a través de fisioterapia y el control sintomático. Caso clínico: Presentamos el caso de una mujer de 37 años, sin antecedentes relevantes, que desarrolló el cuadro tras ser vacunada contra el SARS-CoV-2. La paciente respondió de forma discreta a la fisioterapia, analgesia convencional y corticosteroides. Después de 10 meses de tratamiento, se tomó la decisión de utilizar radiofrecuencia pulsada y bloqueo eco-guiado del plexo, lo que logró mejores resultados. Un año después de la aparición del dolor, los síntomas de la paciente habían mejorado, aunque no había podido reincorporarse a su actividad profesional previa como policía. Discusión: Se han publicado muy pocos casos de neuralgia amiotrófica después de la vacunación contra el SARS-CoV-2. El diagnóstico diferencial incluye diversos trastornos musculoesqueléticos y neurológicos comunes, que pueden despistar al profesional y retrasar la identificación de esta entidad, especialmente en el contexto de campañas de vacunación masivas con gran volumen de reacciones adversas. La analgesia convencional a menudo es insuficiente para abordar los problemas de estos pacientes, por lo que se debe prever la necesidad de programar técnicas invasivas. Dada la rareza de la afección y su impacto en la vida personal y profesional del paciente, se destaca la importancia de un diagnóstico precoz y una comunicación fluida. Finalmente, se subraya el valor de la declaración de reacciones adversas como un signo de profesionalidad y un activo para establecer una relación médico-paciente constructiva.(AU)

Introduction: neuralgic amyotrophy is an inflammatory disorder of the brachial plexus with a poorly understood pathophysiology, possibly related to immunological phenomena. It is characterized by acute and intense pain, accompanied by muscle weakness and altered results in electromyographic and nerve conduction studies. Having no specific treatment, it is approached through physiotherapy and symptomatic control. Case history: we present the case of a 37-year-old woman, with no relevant history, who developed the condition after being vaccinated against SARS-CoV-2. The patient responded discreetly to physical therapy, conventional analgesia and corticosteroids. After 10 months of treatment, the decision was made to use pulsed radiofrequency and ultrasound-guided plexus block of the plexus, which achieved better results. A year after the onset of pain, the patient’s symptoms had improved, although she had not been able to return to her professional activity as a policewoman. Discussion: very few cases of neuralgic amyotrophy have been reported after SARS-CoV-2 vaccination. The differential diagnosis includes many common muskuloskeletal and neurologic disorders, which can mislead professionals and delay identification of the disease, especially in the context of massive vaccination campaigns. Conventional analgesia is often insufficient to address these patients’ complaints; thus, scheduled invasive techniques need to be considered. Given the rarity of the condition and its impact on the personal and professional life of the patient, we highlight the importance of an early diagnosis and smooth communication with the patient. Finally, we stress the value of declaring adverse reactions as a sign of professionalism and an asset in establishing a constructive doctor-patient relationship.(AU)

Diaphragmatic Dysfunction due to Neuralgic Amyotrophy After SARS-CoV-2 Vaccination: A Case Report.

Neuralgic amyotrophy is an idiopathic neuropathy characterized by acute-onset pain, typically in the upper extremity or shoulder, followed by weakness of the associated muscles. Phrenic nerve involvement is rare. We report a 63-year-old man who presented with dyspnea and right shoulder pain after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination. His chest radiograph showed an elevated right hemidiaphragm that was absent before vaccination. A pulmonary function test showed a restrictive pattern with a significant reduction (40%) in forced vital capacity in the supine position. Diaphragm ultrasonography revealed a reduction in both diaphragmatic excursion and a thickening fraction of the right hemidiaphragm. Electrophysiological studies suggested a right upper brachial plexopathy. Considering the temporal relationship between the vaccination and absence of other causes, SARS-CoV-2 vaccination was thought to be the reason for neuralgic amyotrophy with diaphragmatic dysfunction. As there was no evidence of hypoventilation or sleep disturbance that may require noninvasive ventilation, the patient was followed with conservative treatment with analgesics. During 8 months of follow-up, his shoulder pain was relieved significantly but dyspnea improved only slightly. Neuralgic amyotrophy is an under-diagnosed etiology of diaphragmatic dysfunction and should be considered in patients with dyspnea and shoulder pain.

Bilateral Parsonage-Turner syndrome after COVID-19 vaccination. A case report and review of the literature / Kétoldali Parsonage-Turner-szindróma COVID-19-vakcinációt követoen. Esetismertetés és az irodalom áttekintése

Parsonage---Turner syndrome (PTS; neuralgic amyotrophy) is a generally unilateral neuritis with sudden onset, severe shoulder or upper arm pain. Although the intense pain is usually self-limiting, two-thirds of patients experience pro-gressive motor weakness, narrowed range of motion, reflex changes, dysesthesias and chronic neuropathic pain in the shoulder girdle musculature and proximal upper limb muscles. The aetiology is unclear, in addition to some idiopath-ic cases the most common triggers of PTS are surgery, trauma, infection or vaccination. It is reported after SARS-CoV-2 infection, and unilateral PTS has been described in some cases following different types of COVID-19 vaccines. We are currently presenting the case of a middle-aged woman who developed partial neuralgic amyotrophy on the right shoulder one month after receiving the second dose of the BNT162b2 COVID-19 mRNA vaccine (Pfizer-BioNTech), and seven months later the symptoms appeared in the contralateral upper limb. The diagnosis of PTS was also confirmed by magnetic resonance and electrodiagnostic examination. The PTS is not an uncommon condi-tion, but in the absence of knowledge it is rarely thought of. The purpose of this report is to draw attention to the possibility of PTS in shoulder or upper arm pain following both SARS-CoV-2 infection and COVID-19 vaccination, as early diagnosis and adequate therapy may help to shorten the course of the disease.

Neuralgic amyotrophy triggered by cytomegalovirus: to be aware of this clinical diagnosis.

Neuralgic amyotrophy (NA) is a multifocal inflammatory neuropathy. Although the exact etiopathogenesis of the latter is unknown, the literature reports frequent associations with immunological events such as different infectious diseases. Our case reveals a rarely described etiology of NA. NA is mainly a clinical diagnosis. The etiology shown in our case study is interesting for the scientific community, because CMV is an ubiquitous disease. NA is frequently under-recognized and misdiagnosed. This is particularly common in the early phase of the disease, when neurologic signs have not yet developed.