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.

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).

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.

Parsonage-Turner syndrome following monkeypox infection and vaccination.

Beginning in May 2022, monkeypox infection and vaccination rates dramatically increased due to a worldwide outbreak. This case highlights magnetic resonance (MR) neurography findings in an individual who developed Parsonage-Turner syndrome (PTS) 5 days after monkeypox symptom onset and 12 days after receiving the JYNNEOS vaccination. MR neurography of the patient's left suprascapular nerve demonstrated intrinsic hourglass-like constrictions, a characteristic finding of peripheral nerves involved in PTS. Other viral infections and vaccinations are well-documented triggers of PTS, an underrecognized peripheral neuropathy that is thought to be immune-mediated and results in severe upper extremity pain and weakness. The close temporal relationship between monkeypox infection and vaccination, and PTS onset, in this case, suggests a causal relationship and marks the first known report of peripheral neuropathy associated with monkeypox.

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.

Shoulder palsy following SARS-CoV-2 infection: two cases of typical Parsonage-Turner syndrome.

BACKGROUND AND PURPOSE: Coronavirus disease 2019 (COVID-19) is now known to cause neurological complications in both the central and the peripheral nervous system. Two new cases of typical neuralgic amyotrophy or Parsonage-Turner (PT) syndrome following coronavirus 2 infection (SARS-CoV-2) are reported here with explicit electrophysiological and imaging pathological features, underlining the possible association between COVID-19 and PT syndrome. CASE REPORTS: Case 1 was a 45-year-old schoolteacher presenting with acute pain in the right shoulder a few days after SARS-CoV-2 infection, with shoulder abduction and elbow flexion weakness. Needle electromyography showed a decrease in motor unit recruitment in the biceps brachii, and plexus magnetic resonance imaging (MRI) revealed a hyperintense signal involving the right C6 root and the superior truncus of the brachial plexus. Case 2 was a 21-year-old man hospitalized for dyspnea secondary to SARS-CoV-2 infection. Ten days after symptom onset, he presented right shoulder pain with difficulty in raising his right arm, revealing an isolated deficit of the serratus major muscle with a right scapula winging. Electrophysiological evaluation exhibited an isolated involvement of the long thoracic nerve with a neurogenic recruitment pattern in the serratus major muscle. Plexus MRI displayed a thickening and hyperintense signal involving the right long thoracic nerve. DISCUSSION: Parsonage-Turner syndrome triggered by SARS-CoV-2 seems to present clinical, electrophysiological and MRI characteristics similar to classic para-infectious PT syndrome, including the time frame between viral infection and neurological symptom onset. Conclusion SARS-CoV-2 might be a new infectious trigger of PT syndrome.

Applying a clinical decision-making model to a patient with severe shoulder pain ultimately diagnosed as neuralgic amyotrophy.

Objective and Purpose Shoulder symptoms are often encountered in physical therapy and a myriad of etiologies can cause these symptoms, either locally or remotely. The purpose of this case report is to describe the physical therapist's differential diagnostic process for a patient with acute and severe onset of shoulder pain. Case Description: The patient was a 37-year-old female with sudden onset of right shoulder pain that awakened her at night. Pain was associated with decreased range of motion and shoulder weakness. Faced with an uncertain diagnosis, the physical therapist followed a systematic approach to clinical decision-making. Outcomes: Neuralgic amyotrophy was the primary diagnostic hypothesis but other causes of shoulder pain could not be ruled out. Conclusion: The clinical decision-making process helped the physical therapist narrow down the differential diagnosis list and make a decision to send the patient for further testing. Magnetic resonance imaging and electromyogram confirmed the diagnosis of neuralgic amyotrophy.

Diaphragmatic paralysis resulting in respiratory failure as a feature of hepatitis E virus-associated neuralgic amyotrophy.

Hepatitis E virus (HEV)-associated neuralgic amyotrophy (NA) is often bilateral and severe, involving structures outside the brachial plexus, such as the phrenic nerves or the lumbosacral plexus. We report a case of an HEV-positive man who had presented with brachial neuritis, with significant phrenic nerve involvement, resulting in diaphragmatic paralysis requiring non-invasive ventilation. Prognosis of HEV-associated NA is often unfavourable and recovery is usually incomplete. Identifying HEV-associated NA early could potentially aid in prognostication and management planning, as clinicians and patients would be expectant of its potential features and severity. Respiratory function should be monitored in patients with HEV who suffer from NA, as diaphragmatic paralysis could potentially lead to severe respiration difficulties requiring ventilatory support.

Suprascapular neuropathy: A review of 87 cases.

INTRODUCTION: Suprascapular neuropathy (SSN) is rare, with an estimated prevalence of 4.3% in patients with shoulder pain. METHODS: This retrospective chart review included patients with SSN seen during a 16-year period. Demographics and clinical information were recorded. Descriptive statistics, including percentages, means, and standard deviations, were computed for the variables of interest for all patients. RESULTS: Of 87 patients included in this study, trauma (n = 27) was the most common cause of SSN, followed by neuralgic amyotrophy (n = 21). Fifty-seven patients had isolated SSN. Others had SSN associated with axillary neuropathy (23 patients), brachial plexopathy (3 patients), and long thoracic, radial, or spinal accessory neuropathy (1 patient each). DISCUSSION: SSN is commonly associated with axillary neuropathy. Trauma remains the most common cause of SSN. Electrodiagnostic findings aid in the initial diagnosis and may indicate the need for close clinical follow-up based on the severity of the axonal injury.

Phrenic nerve palsy in patients with neuralgic amyotrophy: case series study.

BACKGROUND: Neuralgic amyotrophy (NA) is a relatively uncommon syndrome causing brachial nerves dysfunction. However, it can also affect other nerves including phrenic nerve, which is often misdiagnosed. CASE REPORT: To determine the incidence and characteristics of phrenic nerve palsy in patients with NA in our population, we analyzed the records of all patients with phrenic nerve palsy and/or NA at the University Hospital and the county hospital within the last 10 years. We found that totally, seven patients were confirmed to have NA and phrenic nerve palsy. All of them are male of average age 51.9 years old (51.9±7.4) and had shortness of breath following shoulder and/or neck pain. All of them had elevated diaphragm found in SNIFF test and/or on chest X-ray. Pulmonary function test done in 6 patients demonstrated restrictive lung disease. Six patients needed long-term bi-level positive airway pressure (BiPAP) treatment but mechanic ventilation was not needed. CLINICAL REHABILITATION IMPACT: Our cohort represents one of the largest case series yet reported for phrenic nerve involvement in NA. Most of these patients have had significant pulmonary compromise in the early stage of onset of shoulder/neck pain requiring ongoing BiPAP and specialist monitoring. Recognition of this subset of patients may further require nerve conduction studies/electromyography and respiratory testing.

Isolated spontaneous posterior interosseous nerve palsy: a review of aetiology and management.

Isolated posterior interosseous nerve palsy is an uncommon condition and its management is controversial. Existing literature is sparse and a treatment algorithm based on existing best evidence is absent. A comprehensive review was undertaken to elucidate the causes of spontaneous posterior interosseous nerve palsy and suggest a management strategy based on the current evidence. Posterior interosseous nerve palsy can be broadly categorized as compressive and non-compressive, and the existing evidence supports surgical intervention for compressive palsy. For posterior interosseous nerve pathology with no compressive lesion on imaging, conservative management should be tried first. Surgery is therefore reserved for compressive lesions and for failure of conservative management. The commonly performed operative procedures include decompression and neurolysis, neurorrhaphy and nerve grafting, and tendon transfers with or without nerve grafting performed as a salvage procedure. The prognosis is poorer in patients aged > 50 years, those with a delay to surgery, and those who have had long-standing compression with severe fascicular thinning.

Herpes zoster increased risk of neuralgic amyotrophy: a retrospective, population-based matched cohort study.

Although neuralgic amyotrophy (NA) has occasionally been reported to be associated with reactivated herpes zoster, their associated risk remains unknown. The aim of this study was to assess the risk of developing NA following preceding herpes zoster. The authors used the National Health Insurance Research Database of Taiwan to select 41,548 patients with newly diagnosed herpes zoster during the period 2000 to 2010 and randomly extracted 166,192 matched control subjects. All participants in the study and control groups were followed for 3 months after the diagnosis to identify those who developed NA. Cox proportional hazards regression analyses were performed to evaluate the subsequent risk of NA. Twenty-one subjects from the group with herpes zoster (0.05%) developed NA over the 3-month period and 46 from the group without herpes zoster (0.03%). The patients with herpes zoster had a higher risk of developing NA (adjusted hazard ratio = 1.408, 95% confidence interval = 1.013-2.319, P = 0.030). In the patients with herpes zoster, female sex, age ≥ 65, hepatitis E virus (HEV), and having had a recent infectious event including pneumonia and influenza were risk factors for developing NA (adjusted HR 2.746, 1.998, 2.735, 2.016, and 1.718, respectively, all P < 0.05). Patients with herpes zoster attack have a higher risk of developing NA over a 3-month period after diagnosis, especially those who are female, age ≥ 65, HEV, or have experienced a recent infectious event or pneumonia and influenza.

Phrenic neuropathy and diaphragm dysfunction in neuralgic amyotrophy.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Hepatitis E virus as a cause of acute hepatitis acquired in Switzerland.

BACKGROUND: Autochthonous hepatitis E is increasingly recognized as zoonotic infection in western countries. Serological assays have varying sensitivity and specificity. METHODS: We implemented molecular testing to identify and characterize acute hepatitis E acquired in Switzerland. RESULTS: Ninety-three cases of mostly symptomatic acute hepatitis E acquired in Switzerland were documented by PCR between November 2011 and December 2016. Median HEV RNA was 7.5 x 104 IU/mL (range, 5.3 to 4.7 x 107 IU/mL). HEV genotyping was successful in 78 patients, revealing genotype 3 in 75 and genotype 4 in three patients. Phylogenetic analyses revealed a few limited geographical and temporal clusters. Of the 91 patients with available anti-HEV IgM serology, four were negative; three of these were also IgG-negative, likely as a result of immunosuppression, and one was IgG-positive, a constellation compatible with HEV reinfection. Median age of the patients was 58 years (range, 20-80 years); 71 (76.3%) were men and 49 of these (69.0%) were ≥ 50 years old. The clinical course was particularly severe in patients with underlying chronic liver disease, with fatal outcome in two patients. Six patients (6.5%) presented with neuralgic amyotrophy. CONCLUSIONS: Nucleic acid-based diagnosis reveals HEV as a relevant cause of acute hepatitis in Switzerland. Middle-aged and elderly men constitute the majority of symptomatic patients. Testing for HEV should be included early in the diagnostic workup of acute hepatitis and of neuralgic amyotrophy, a typical extrahepatic manifestation of HEV genotype 3 infection.