Shoulder Weakness Following Intramuscular COVID- 19 Vaccine

History: Transient and generalized adverse effects are common following COVID-19 vaccination;among other adverse effects, shoulder injuries related to vaccine administration (SIRVA) have been known to occur. In this case, a previously healthy right-hand dominant 62-year-old male presented with left shoulder pain and weakness 3 months after receiving a COVID-19 intramuscular vaccine in the left deltoid. Approximately 2 weeks after the injection, he started experiencing pain and numbness around the injection site along with ipsilateral shoulder weakness. Despite conservative management with Motrin, Medrol Dosepak, gabapentin and physical therapy (PT), the pain and weakness persisted. Physical Exam: Left Shoulder-No calor or erythema;significant atrophy of the anterior and middle deltoid muscle relative to right side;abduction 4/5;external rotation with shoulder adducted 4/5;range of motion for active forward flexion was 150 degrees and passive was 170 degrees;passive range of motion for external rotation was 70 degrees;internal rotation to the level of L5;sensation to light touch was intact. Right Shoulder-Range of motion, strength, and sensation were intact. Cervical Spine-Full ROM;no cervical paraspinal tenderness noted. Negative Spurling's and Lhermitte's tests. Differential Diagnosis: 161. Axillary Nerve Palsy 2/2 Chemical Neurotoxicity 162. Brachial Neuritis 163. Mechanical Axillary Nerve Palsy 2/2 Vaccination 164. Partial-Tear of Left Supraspinatus Tendon 165. Acromioclavicular Osteoarthritis Test Results: Left Shoulder-XR:Mild pseudo-subluxation;MRI w/o contrast: 8x9mmpartial-thickness articular surface tear of the distal supraspinatus tendon (<50%fiber thickness). Minimal subacromial bursitis. Mild acromioclavicular joint osteoarthritis. EMG/NCV: Left and Right Axillary Motor Nerves: prolonged distal onset latency;Left Deltoid: increased insertion activity, moderately increased spontaneous activity, reduced recruitment;Remaining LUE muscles without evidence of electrical instability Final Diagnosis: Axillary Nerve Palsy Secondary To Chemical Neurotoxicity from Intramuscular COVID-19 Vaccine. Discussion(s): We postulate that the neurologic deficits presented in our case may be attributed to chemical neurotoxicity to the axillary nerve following vaccination as the delayed onset of pain and weakness are most consistent with this differential. There are several cases of brachial neuritis following vaccination for the prevention of COVID- 19, however, EMG/NCV results in our patient were not consistent with brachial plexopathy. Additionally, while there have been a handful of reported cases of bursitis following COVID-19 vaccines falling under the SIRVA classification of injuries, this is the first case of reported axillary nerve neurapraxia. Outcome(s): The patient's left shoulder numbness and pain improved with PT and medical management. While mild improvement in strength was noted, weakness and atrophy persisted even on the third follow up visit 6 months after the initial appointment. He was counseled on his injury and was recommended to undergo repeat EMG testing to document recovery after his 6-month follow-up appointment. Follow-Up: The patient did not follow-up for a repeatEMG after his 6-month follow-up appointment. At that time, the patient was clinically stable, tolerating PT, and expecting recovery of his deltoid function.

An 81-Year-old Woman With Proximal Weakness

An 81-year-old woman developed progressive proximal weakness and myalgias several months following a COVID-19 infection. She was admitted to her local hospital for progressive weakness, peripheral edema, and exertional dyspnea. Neurology evaluation noted proximal arm and leg weakness. She had creatine kinase 740 U/L, white blood cells 21,000/mL (with abnormal differential), and abnormal antibody serologies. Additional diagnostic testing obtained included a thigh MRI and muscle biopsy. During her COVID-19 admission, a mediastinal mass had been detected, which was increased in size on this current admission. Notably, she had a remote history of an incidentally discovered mediastinal mass, which had been incompletely resected 18 years prior. At neuromuscular follow-up one month later, she reported improvement in peripheral edema and dyspnea but ongoing weakness. Strength exam noted symmetric Medical Research Council grade 4 weakness in neck flexion/extension, shoulder abduction, elbow flexion/extension, wrist extension, hip flexion/abduction/extension, and knee flexion. She had no fatiguability and no facial or bulbar weakness. Remainder of her neuromuscular examination was unremarkable. Her white blood cell count differential remained abnormal but had improved from her initial presentation. Her recent muscle biopsy slides were reviewed again. Bone marrow biopsy and mediastinal mass biopsy were obtained. A unifying diagnosis was made, and she was started on therapy with resolution of her weakness, myalgias, and abnormal cell counts.

An occurrence of immune-mediated necrotizing myopathy following treatment of polymyositis in a Filipino female: A case report

Background: Necrotizing myopathy has been previously described but was not included in the Peter and Bohan criteria until 2004, when immune-mediated necrotizing myopathy (IMNM) was distinguished from polymyositis (PM) based on immunologic and histopathologic differences. IMNM is currently a well-recognized autoimmune myopathy and represents up to 20% of these cases. Case: A 60-year- old female with biopsy-proven PM achieved sustained clinical remission with Rituximab. Her co-morbid conditions include hypertension, diabetes mellitus, and dyslipidemia. The patient noted a recurrence of gradual progressive, proximal muscle weakness and easy fatigability after receiving her first mRNA Covid-19 vaccine. Four months after onset of symptoms, CK Total was 9600 U/L. Rituximab was administered and muscle weakness and total CK levels (1247 U/L) improved within 10 days. She was prescribed rosuvastatin and fenofibrate for dyslipidemia within 7 days of completing the rituximab course. Two weeks later, proximal muscle weakness recurred. She became wheelchair-bound and experienced dysphonia. MMT score was 2/5 in proximal muscles and total CK total increased to 19,935 U/L. The patient received Methylprednisolone 500 mg IV once a day for 3 days. She had a good response with resolution of dysphonia and improvement of MMT to 4/5 on shoulder abduction and hip flexion on the 6th hospital day. She was discharged on oral methylprednisolone at 1 mg/kg/day. Muscle biopsy was consistent with an immune-mediated necrotizing myopathy, revealing necrotic fibers, intracellular macrophages, fatty infiltrates, irregular staining patterns on NADH stain with no evidence of endomysial inflammation, perifascicular atrophy, ragged red fibers, or rimmed vacuoles. Antibodiy against 3-hydoxy- 3- methylglutarylcoenzymeA reductase (HMGCR) result is pending but the other myositis-specific antibodies are negative.(including anti-SRP). Conclusion(s): IMNM is an autoimmune myopathy associated with anti-HMGCR and anti-SRP antibodies that clinically present similarly to polymyositis. The temporal occurrence of worsening muscle weakness with initiation of statin therapy make statin toxic myopathy or immune mediated necrotizing myopathy as diagnostic considerations. This case emphasizes the need to re-evaluate the etiology of new onset muscle weakness in patients with idiopathic inflammatory myopathy and highlights the role of myositis-specific antibodies and muscle biopsy in confirming the diagnosis.

Multi-System and Cavernous Sinus Inflammatory Syndrome Presenting with Evolving Multiple Cranial Neuropathies Following Zoledronate Infusion

Case report - Introduction: Bisphosphonates are known to rarely cause multi-system inflammation, including multiple cranial neuropathies. This is possibly via provoking transient cytokine storm. The literature reports bisphosphonate-associated orbital inflammatory syndrome, and one case of retrobulbar optic neuritis following zoledronate. Bisphosphonate manufacturers report conjunctivitis, blurred vision, scleritis, orbital inflammation, uveitis and episcleritis as ocular side effects. Separately, neurological sequalae, including cranial neuropathies, are reported following COVID-19 infection and vaccination. Here, we report the first case of cavernous sinus inflammation temporally related to both zoledronate infusion, and more remotely, to Pfizer- BioNTech COVID-19 vaccination. Case report - Case description: A 76-year-old white man developed fever, bony leg pain - which rendered him unable to walk - and frontal headache, within 8 hours of his first zoledronate infusion for osteoporosis. A few weeks earlier he received his first Pfizer-BioNTech COVID-19 vaccine. His General Practitioner commenced a short course of lowdose oral prednisolone for the episode. One week later, off prednisolone, the headache localised around the left eye. He developed horizontal diplopia associated with abduction deficit. He was diagnosed with left VIth nerve palsy. He was started on high-dose steroids and clopidogrel (with PPI) with neuroimaging to exclude stroke or venous sinus thrombosis. Two weeks later, the diplopia worsened over 4 days, with new left adduction deficit (-2 limitation), left ptosis 1-2mm and anisocoria 0.5-1mm R>L suggestive of partial third nerve palsy and early Horner's syndrome. Ocular and neurological examinations were otherwise normal. He wore varifocals and had migraines, osteoporosis, and asthma, for which he used inhalers. He worked in visual arts and was an ex-smoker (>50 years) with moderate alcohol intake. Blood results revealed CRP 38mg/L, but otherwise normal inflammation/ vasculitis/infection screen;anti-thyroglobulin antibodies were >4000 U/ml;GQ1P, Creatinine Kinase, anti-ganglioside, and Anti- AChR/MuSK antibodies were normal. CT head and Optical Coherence Tomography were unremarkable. An enhanced MRI of the brain and orbits revealed abnormal thickening and T2 hyper-intensity of the left oculomotor nerve, most notably involving the left canalicular portion. The left cavernous sinus also appeared asymmetrically bulky with a rind of abnormal enhancing soft tissue in the left cavernous sinus. Subtle STIR hyper-intensity was also observed in the ipsilateral CN IIIinnervated extra-ocular muscles. After a 6-week course of tapering prednisolone, the vertical diplopia and leg swelling persisted;the horizontal diplopia and headaches had resolved. By 3months, there was resolution with mild residual visual changes. Case report - Discussion: We report a constellation of symptoms relating to multi-system inflammatory syndrome involving the cavernous sinus. There is a lack of epidemiological data on the incidence of this rare presentation in the population. This case has close temporal association to bisphosphonate infusion (<12h) and weaker association to coronavirus vaccination (<3wk). It is difficult to determine whether this is a rare presentation of a known drug reaction, a more delayed presentation of a vaccine reaction or whether these events were coincidental. A further possibility in this case is a combined predisposition resulting from both vaccination and bisphosphonate infusion. This case highlights a wider issue relating to the challenging possibility of ascertainment bias and increased 'Yellow Card' reporting of rare presentations during this historic global coronavirus pandemic, which may or may not have any true causal association to vaccination. There is difficulty in disentangling a true vaccine reaction from an unrelated presentation of a rare condition with an unknown baseline incidence rate. This is especially topical given that the majority of the population are receiving the coronavirus ccination at this time. We also question what a plausible cut-off point would be to propose a temporal relationship for an adverse reaction;in the literature, adverse reactions have been postulated to develop beyond 1 month after the provoking agent. Case report - Key learning points: . This case highlights the need for urgent assessment, investigations including neurological imaging and consultant input in patients with evolving cranial neuropathy. The priority is to rule out thrombotic, compressive, inflammatory and infectious pathology in the cavernous sinus, venous sinus, orbit and orbital apex. . Pathology of the cavernous sinus presents with variable involvement of CN III, IV, V and VI and Horner's syndrome. A differential for this case would be superior orbital fissure syndrome, which also presents with multiple oculomotor cranial neuropathies;it involves these cranial nerves and the ophthalmic branch of CN V. Orbital apex syndrome is SOF with a loss of vision due to additional CNII involvement. . The neuro-radiology differential included inflammatory, infiltrative, granulomatous and neoplastic aetiologies and that there was sufficient existing evidence to exclude brainstem pathology. . Through communication between specialties, the temporal relationship was established, and clinical examination and extensive investigation further honed the differential to either inflammatory or vascular. Since it was temporally related to the zolendronate infusion, it seemed plausible it was related. We demonstrate the need for multi-disciplinary collaboration for these patients between rheumatology, ophthalmology and neuro-radiology.

Decision-making and treatment results of complex proximal humeral fractures in geriatric patients: retrospective study from a level 1 trauma centre

Proximal humeral fractures are common in elderly, but despite the high incidence, optimal treatment is still discussed and remains a topic of controversy. Nonoperative treatment continuous to be the main modality. However, due to advancements in surgical technology with new techniques and implants, operative treatment could lead to better outcomes and less complications, even in older patients. Decision-making in elderly should incorporate comorbidities, activity level and patient expectations. This study was performed with the intention to find out, if there is a significant difference in treatment strategy and number of operations, in the last five years. Patients older than 65 years with proximal humeral fractures were included. Retrospective analysis of radiographic material and post-injury data was performed, from patients treated in 2015, 2019 and 2020. Last two years were also compared separately to exclude the effect of Coronavirus disease 2019 (COVID-19) pandemic. Epidemiological data assessment, fracture type and treatment strategy were analysed for corresponding years. Statistical analysis was focused on complex three-and four-part fractures. There were no statistically significant differences regarding incidence between the analysed years. Low energy fall was the mechanism of injury in majority of patients. Patients with tuberosity fractures were in average younger than patients in other groups. Although there were more computed tomography (CT) scans done in younger elderly patients, there was no significant difference in number of CTs compared to older patients (year 2015: p = 0.246;year 2019: p = 0.710, year 2020: p = 0.849). The number of operative interventions was the lowest in 2019 (p = 0.498) and the same was for the osteosynthesis using intramedullary nails (p = 0.014). Frequency of reversed shoulder arthroplasty surgeries is increasing, but the difference is not significant (p = 0.390). Both operative and nonoperative treatment result in similar range of motion (ROM) measurements (p = 0.164 for anteflexion. p = 0.163 for abduction), however the groups were not comparable regarding exact fracture types. In the analysed period of 5 years, epidemiology and treatment strategy of proximal humeral fractures did not change. Nonoperative approach remained the main treatment modality. No significant difference was noted in number of interventions or implants used, although there seemed to be an increased trend towards treatment with reverse shoulder arthroplasty (RSA) in complex fractures. A strong correlation was observed between radiographic indications for conservative treatment and actual implementation of it. However, when surgical treatment was indicated using the same radiological criteria, there were more than half of patients, who were not operated on. Radiologic indications are thus not enough for decision-making in treatment of three-and four-part fractures, and patient factors, such as comorbidities and pre-injury activity level, play a major role. Copyright © 2023 The Author(s). Published by MRE Press.

How Does COVID-19 Affect the Brain: Neurological Complications and Cognitive Effects of COVID-19

The coronavirus disease (COVID-19) with its rapid spread and high mortality rate has caused major disruptions. It involves the nervous system. COVID-19 also causes infection in the brain stem which may influence chemosensory neural cells related with respiratory and cardiovascular regulation and also neurons of the respiratory center. This study evaluates the effects of COVID-19 on neurological complications and cognitive. Several studies were reviewed for the effects of COVID-19 on neurological complications and cognitive function. COVID-19 causes signs such as headache, altered mental status, anosmia, myalgia, ischemic stroke, developed cerebral hemorrhage, and cerebral venous sinus thrombosis, olfactory disorders, anosmia, losing taste, mental retardation, migraine, Guillain-Barre syndrome, encephalopathy, severe abduction deficits in both eyes, esotropia, epilepsy, hypogeusia, hyposmia, faulted consciousness and seizures. It also caused cognitive function such as Alzheimer's disease, cognitive worsening, depression, anxiety, tiredness, anxiety, decrease in BDNF, stress and fatigue. In conclusion, COVID-19 causes negative effects on neurological system and cognitive function which must be considered for the treatment of the disease in alongside clinical treatments. Copyright © 2022 Wolters Kluwer Medknow Publications. All rights reserved.

Pain in the Neck: A Case Report of Suprascapular Neuropathy Due to Parsonage Turner Syndrome

Case Diagnosis: Parsonage Turner Syndrome Case Description or Program Description: A 34-year-old male presented with 1 month of suddenonset left neck pain radiating to the shoulder. Symptoms began upon waking from sleep without preceding triggers or infection. Pain was severe and rated 8/10. Nonsteroidal anti-inflammatories and muscle relaxants provided moderate relief, but he developed weakness weeks later manifested as difficulty with carrying his child, donning a coat, and overhead activities. Exam demonstrated decreased infraspinatus bulk and weakness with external rotation and abduction. Nerve conduction studies were normal but electromyography (EMG) demonstrated moderate supraspinatus membrane instability and severe infraspinatus instability without residual motor units or reinnervation signs. MRI of the shoulder confirmed intrinsic constriction of the suprascapular nerve consistent with Parsonage-Turner Syndrome (PTS). Subsequent autoimmune, hepatitis, Covid-19, and HIV studies were unremarkable. Setting(s): Outpatient Clinic Assessment/Results: The patient underwent several courses of physical therapy with slow progress but interval improvement in childcare and dressing capabilities. Discussion (relevance): PTS is a rare disorder that can present with a complex constellation of symptoms. PTS may mirror other pathologies including cervical spondylosis, rotator cuff tendinitis, adhesive capsulitis, or nerve compression by mass lesion. The typical pattern involves abrupt pain followed by weakness after pain has diminished. PTS is often attributed to prior viral infection, immunization, recent surgery, or heavy exercise but can also be idiopathic without identifiable triggers. EMG in conjunction with MRI can be crucial in grading severity of denervation and differentiating PTS from true compression which often requires more invasive interventions. While the majority of patients recover functionally by 3 years with conservative treatments, progress may be slow and physicians should consider long term follow-up with repeat electrodiagnostics to track recovery. Conclusion(s): In patients with abrupt shoulder or neck pain followed by progressive neurologic deficits, PTS needs to be considered. Electrodiagnostic studies can both aid in diagnosis and be used to track recovery over time.

Parsonage Turner Syndrome After COVID-19 Vaccine Booster: A Case Report and Review of the Literature

Case Diagnosis: A 78-year-old man with Parsonage- Turner Syndrome (PTS). Case Description or Program Description: The patient developed acute left-sided neck and shoulder pain upon awakening five days after receiving a Moderna COVID-19 vaccine booster shot. Clinical examination, electrodiagnostic studies, and magnetic resonance imaging were consistent with a diagnosis of PTS. Setting(s): Tertiary referral center. Assessment/Results: His physical exam revealed severe weakness in left shoulder abduction and external rotation without sensory deficits. An urgent MRI of his cervical spine demonstrated multilevel degenerative changes including severe bilateral C5-6 neural foraminal narrowing, and an MRI of the left shoulder showed mild degenerative changes. He was treated with a sixday course of an oral methylprednisolone dose pack and his pain and weakness significantly improved. He was referred for electrodiagnostic testing 24 days after the onset of his symptoms, and by the time of the study, his pain and weakness had improved by 50%. The test revealed no significant abnormalities in the sensory and motor nerve conduction studies. Needle electromyography showed abnormal spontaneous activity in both the left infraspinatus and left deltoid with decreased recruitment of polyphasic motor unit action potentials in the left deltoid. Notably, the left mid/low cervical paraspinals, and other left C5/C6 innervated muscles including the biceps, and brachioradialis were all normal, making a diagnosis of cervical radiculopathy unlikely. Discussion (relevance): There have been eight published reports of PTS related to COVID-19 vaccinations at the time of this publication, which are also reviewed. Reports have occurred in three separate vaccines with variable onset of symptoms and recovery patterns as detailed in the table provided. Conclusion(s): Our case report and review of the literature highlights the importance of recognizing PTS as a potential cause of severe shoulder/arm pain and weakness after administration of a COVID-19 vaccine.

Ulnar Neuropathy During COVID-19 Infection: A Case Report

Case Diagnosis: Ulnar Mononeuropathy following COVID-19 infection. Case Description or Program Description: A 44-year-old male with history of morbid obesity presented with coronavirus disease 2019 (COVID-19) infection resulting in a prolonged hospitalization of 37 days. While admitted he was largely proned, however never required intubation. While hospitalized, he developed numbness, tingling, and weakness in digits 4-5 of his left hand exacerbated by pressure on the elbow. No symptoms were present in the contralateral arm. Setting(s): Academic Acute Care Hospital Assessment/Results: Magnetic resonance imaging of the brain and cervical spine were unremarkable. Physical examination of digits 4-5 in the left hand revealed reduced sensation to light touch, an inability to fully extend the digits, and 4/5 strength with abduction. Electrodiagnostic testing demonstrated absent left ulnar sensory nerve action potential when recorded from the left 5th digit and reduced motor conduction velocity across the elbow (39m/s) compared to the below-elbow segment (53m/s) when recorded from the left ulnar abductor digiti minimi, consistent with conduction block. He declined needle electromyography due to potential pain. His hemoglobin A1c was 7.3%. Discussion (relevance): Peripheral nerve injuries (PNIs) may occur in up to 14.5% of patients with COVID-19 who undergo prone positioning, and the ulnar nerve is the most frequently affected. We present a case of ulnar mononeuropathy during COVID-19 hospitalization. Etiology is likely multifactorial, with prone positioning, similar risk factors, or direct pathogenicity contributing. Compressive injuries of the ulnar nerve have been associated with improper prone positioning. Additionally, PNI shares risk factors with severe COVID-19, namely obesity and diabetes in this patient. The hyperinflammatory state associated with COVID- 19 also increases the risk of PNI. Lastly, COVID-19 invades cells by binding angiotensinogen converting enzyme-2 receptors, which are present in the nervous system. Conclusion(s): COVID-19 infection may be associated with an increased risk of peripheral nerve injuries through a multifactorial mechanism. Further research is needed to establish the association.

What is the prevalence and distribution of upper limb weakness early post-stroke?

Background: A growing body of international research suggests the prevalence of upper limb weakness early after stroke is currently lower (40-57%) than widely cited values of 70-80% from two decades ago. Recent work also indicates the distribution of upper limb weakness may be bimodal, with a higher proportion of people with severe or little/no weakness as compared to mild/moderate weakness. Aim: To describe the prevalence and distribution of upper limb weakness early post-stroke. Methods: Patients admitted to a tertiary acute stroke unit with a suspected stroke were screened between November 2018 to February 2020 (interrupted by COVID-19) and April to November 2021. Upper limb weakness was captured via Shoulder Abduction and Finger Extension (SAFE) score (0-10), which was prospectively assessed at first contact by the unit therapist. Data on stroke type, acute medical intervention received, and National Institute of Health Stroke Scale (NIHSS) were also extracted. Results: A total of 662 individuals with confirmed stroke (median NIHSS score 6, IQR 2-13) were administered SAFE a median 1 (IQR 1,2) day after unit admission. Only 46.2% had upper limb weakness (SAFE score ≤9). Three most common SAFE scores were 10 (53.8%), 8 (11.5%) and 0 (9.4%). The subgroup severity distribution was 59.2% little to no impairment (SAFE 9-10), 24.1% mild to moderate impairment (SAFE 5-8), and 16.7% severe impairment (SAFE 0-4). Approximately one third (29.8%) received ≥1 acute interventions (e.g., thrombolysis, thrombectomy). Data collection remains ongoing, and a larger total sample will be presented. Conclusion: The prevalence of upper limb weakness at this single tertiary centre aligns with recent international data. A better understanding of the upper limb weakness profile will help inform service delivery e.g., shifting resources to subgroups which are more common. Furthermore, it can guide researchers in target population selection in trials, which can enhance generalisability of findings.

Quick Clinical Outcome Assessments

The Covid-19 pandemic has highlighted the diffi- culty in the management of neuromuscular patients and the need for continued implementation of the standard of care. With the new pandemia psychometrically robust but quick outcome measures are needed to monitor patients' clinical status. The slow progressive nature of several muscle disorders and the wide pattern of involvement in muscular dystrophies and myopathies make it difficult to establish the prognosis, predict clinical evolution and perform trials and define the impact of natural history, and use new therapies that are becoming available. We constructed a motor function test that is easy and quick to use. This quick Motor Function test: Gait, Stair, Gower's, Chair (GSGC) was constructed based on the clinical expertise of several physicians involved in the care of DMD;LGMD, and Pompe patients. The GSGC score can be integrated by the use of the motor function of the upper motor limbs with the arm function test (GSGCA). It consists of a simple standardized functional test which grades the ability of the patient to raise their upper arms over the head. Grade 0 corresponds to a full circle of arm abduction, while with grade 6 the patients cannot raise their arms to their mouth and effectively use their hands. The Gardner-Medwin Walton (GMW) scale even modifi ed appears in comparison rather insensitive. The GSGC test includes 4 items. The test provides a detailed picture of motor function by including a quantitative measure of four performances i.e. time to perform four activities: Gait =walking for 10 meters, S=climbing 4 steps on a Stair, G= Gower's maneuver, C= rising from a Chair (Figure) The GSGC final score is obtained by adding the grades of the four functional tests and ranges from a minimum of 4 (normal performance) to a maximum of 27 (worst performance).GSGCA test includes 5 tests (total score from 5 to 32). Validity and test reliability were determined in a cohort of 9 adult Pompe patients (15 to 54 years of age) and then validated in 40 LOPD cases by a collaborative group. The responsiveness of the GSGCA scale to changes in clinical course over time was examined in a subgroup of 13 LGMD 2B/R2 untreated patients. Interrater and intrarater reliabilities were most usually confirmatory. The motor outcomes are different in various myopathies and depend on a correct diagnosis, while exercise in myopathy patients should be moderate, but not necessarily discouraged. The muscle MRI imaging might be helpful for follow-up of the proximal or distal muscle involvement, to detect fat, and connective tissue replacement, which might be usually absent in metabolic myopathies, except for LOPD. Diet and exercise in LOPD might be an additional therapeutic option synergistic to ERT. In this presentation, we examine the use of the GSGC scale in LOPD, DMD, and GSGCA scales in the natural history of LGMD R2. The development of smart care using telemedicine and eHealth technologies to share images, clinical data, reports, and video meetings of collaborative groups should be implemented. Keywords: GSGC scale, Covid-19, DMD, LGMD, Pompe.

A Challenging Case of Central Nervous System (CNS) Involvement with Chronic Lymphocytic Leukaemia (CLL): A Case Report

Background: CNS involvement in CLL is rare and it usually occurs in late-stage CLL disease. There is usual delay in the diagnosis due to its variable manifestations, challenging diagnosis process and possible misdiagnosis with a mimicker condition. I am sharing our relative successful experience with this challenging case that had satisfied outcome after going through comprehensive investigations and treatment journey treating his symptoms until arriving the final diagnosis and getting the best treatment option. Material(s) and Method(s): A 42 years old male, with recent COVID-19 infection, presented with multiple progressive neurologic symptoms over one month;started as numbness around the mouth, reduced facial sensation and a feeling of band like sensation below the costal margins. On exam, he had left abduction restriction, diplopia on left gaze and upbeat nystagmus, reduced facial sensation and hyperesthesia. The reflexes were 1+ in the upper limbs, 3+ in the lower limbs, up going planters, tingling from the feet up to T6 level and postural tremor bilaterally. His CSF showed high protein level. MRI brain/ spine revealed left frontal juxtacortical white matter and bilateral middle cerebral peduncles lesions with post-contrast enhancement and long segment spinal cord demyelinating plaques. He was initially treated as a case of Acute disseminated encephalomyelitis (ADEM) post viral infection in a background of CLL. The delayed diagnosis was due to temporal relation of neurological manifestation to viral infection, similar MRI lesions to ADEM and multiple negative CSF results of cytology and flow cytometry. He had persistent disabling symptoms and enhancing lesions in MRI despite being treated with IVMP, IVIG and PLEX. He was managed for ADEM based on responsiveness to the recommended therapy step by step. Firstly, he received a high-dose corticosteroids, secondly IV immunoglobulin but he was still progressing and considered as steroid-unresponsive ADEM. lastly, plasma exchange was done when he exhibited progressive symptoms with fair improvement. Interestingly, the patient showed significant improvement in the clinical and radiological parameters after starting him with a new anti-leukemia medication (Acalabrutinib) for his concurrent active condition. He run out of his medication for around 1 week and he experienced recurrent of the neurological manifestation and the previous lesions in the images. A repeated flow cytometry for the third time came positive for CLL cells and the final diagnosis of CNS involvement by CLL was established. The diagnosis was made after the exclusion of other etiologies. Result(s): The patient received Ibrutinib at a standard dose and as a monotherapy. It is an efficient chemotherapy that crosses the blood brain barrier and has showed a favorable clinical, biological and radiological outcome. The patient is back to his work and his daily activities have improved. Conclusion(s): In case of inconclusive work up, CSF analysis should be repeated testing for cytology and flow cytometry\immunophenotypes as the false negative results are common. Our patient had an active CLL proved in his investigations, and the fact that the patient responded very well to the new chemotherapy should alert the diagnosis of CNS involvement by CLL and directs towards repeating investigations and introducing aggressive treatment strategy to target both hematological and neurological complications of the condition.

WHY YOU SHOULDN'T AIM TOO HIGH ALL THE TIME: A CASE OF SHOULDER INJURY RELATED TO VACCINE ADMINISTRATION (SIRVA) OF COVID-19 MRNA

CASE: A 23-year-old female presented to resident clinic for 7 months of right shoulder pain. She received her second COVID-19 mRNA vaccine just prior to onset of pain. She noted vaccine administration was “traumatic” with significant bleeding and bruising. She started noticing pain with overhead activities several days later. She is very active with cardiovascular exercises. She lifts weights but none requiring overhead motions. The pain was worst at the front of the shoulder but radiated to the lateral aspect. She had not tried, ice, heat, medications or physical therapy. Because of her injury, she was hesitant to receive her COVID-19 booster. BMI was low at 16.65. Exam showed thin build and overall low muscle bulk. Right shoulder showed no signs of muscle atrophy. There was tenderness of subacromial and coracoid areas. No pain along biceps tendon or AC joint. She had full ROM with shoulder abduction, internal and external rotation. She had full strength of supraspinatus, infraspinatus, teres minor, and subscapularis muscles. She noted pain with abduction, internal rotation and lift-off maneuver. Her Hawkins and Neer's maneuvers were positive. No pain with Yergason's and Speed's maneuvers. The patient was diagnosed with right shoulder subacromial bursitis and impingement syndrome. IMPACT/DISCUSSION: Mild shoulder pain is expected after vaccine administration and typically resolves in days. However, SIRVA is an increasingly recognized complication of improper vaccine administration particularly in the occupational setting. SIRVA results from vaccine being delivered inadvertently within the subdeltoid bursa or joint space. It is thought to result from an immune mediated reaction to the vaccine components as injury tends to be greater than expected from a needle injury. We were able to find 5 cases of reported SIRVA related to the COVID vaccine. All included some form of subacromial, subdeltoid, or subcoracoid bursitis. One case noted a supraspinatus tear. Ultrasound has demonstrated the subacromial bursa can extend distal to the acromion by up to 6 cm, so administration to bursa is possible in the superior deltoid. Appropriate injection technique can reduce the risk of injury;administrators should use landmarks of the acromion and distal insertion point of deltoid mid-humerus. Proper needle length is important. It has been suggested a smaller deltoid fat pat and smaller deltoid muscle bulk are risk factors for SIRVA. Women tend to have a higher incidence. CONCLUSION: We presented the case of a slender female who developed shoulder bursitis and impingement following administration of COVID-19 mRNA vaccine. She was referred to PT for rotator cuff strengthening, instructed to refrain from aggravating activities, and provided NSAIDs for pain relief. She reports pain relief. Another option for a more severe case would be a subacromial bursa steroid injection. It is important for providers to be aware of this pathology to provide appropriate treatment and decrease vaccine hesitancy.

SHOULDER INJURY RELATED TO VACCINE ADMINISTRATION (SIRVA)

CASE: A 62-year-old woman presented with 4 months of sharp progressive left shoulder pain, radiating down her arm with associated weakness, numbness and tingling most pronounced at the 4th and 5th digit. Her symptoms began within hours of receiving the influenza vaccine to her left shoulder. She denied prior left shoulder or neck pain, headaches, changes in vision, other neurologic symptoms, or trauma. Exam: Left upper extremity without skin changes or deformity, normal muscle bulk, tone and DTRs, lateral upper arm tenderness to light and deep palpation, reduced sensation to light touch at the 4th and 5th left digit with loss of two-point discrimination, reduced active and passive ROM of the glenohumeral joint to flexion/extension/abduction, and restricted internal and external rotation. Cervical x-rays showed spondylosis at C5-6, C5-6 neural foramen narrowing. Normal left shoulder x-ray. Left shoulder MRI showed high grade bursal surface, full-thickness tear of the distal supraspinatus tendon at its insertion, mild subscapularis tendinosis, and small subacromial subdeltoid bursitis. She was treated with a topical NSAIDs, tramadol and cyclobenzaprine as needed and referred to physiotherapy and PM&R. Despite maximum therapy, there was only marginal improvement of left shoulder pain and function at 9 months, she is still unable to perform her ADLs or return to work, and currently receiving home care through her daughter as a caregiver. IMPACT/DISCUSSION: The MRI findings and the temporal relationship between vaccine administration and onset of symptoms, suggest Shoulder Injury Related to Vaccine Administration (SIRVA) as the most likely diagnosis. SIRVA is defined as shoulder pain with limited ROM that commences within 48 hours after vaccine receipt in individuals without prior history of pain, inflammation, or dysfunction of the affected shoulder. SIRVA occurs when a vaccine is delivered into the sub-deltoid bursa or joint space, leading to a robust inflammatory response. The single most important factor in SIRVA diagnosis is the temporal association between vaccine administration and symptom onset. Commonly reported symptoms include shoulder pain, decreased limb mobility, numbness/tingling and muscle tightness. SIRVA complications include bursitis, tendonitis, rotator cuff tear, and adhesive capsulitis. Approximately 65% of patients with SIRVA will have pain lasting more than 3 months. SIRVA is challenging to treat, but there has been some success with early corticosteroid injection within 5 days of symptom onset. Given the current increase in vaccine administration with COVID-19, this case highlights SIRVA as a diagnostic consideration for patients who present with shoulder pain post-vaccination. CONCLUSION: SIRVA should be considered in any patient with new-onset shoulder pain that began within 48 hours of vaccine administration. SIRVA is a post-vaccination complication resulting in shoulder injury that can be prevented with proper vaccine administration technique.

Multiple Cranial Neuropathies After COVID-19 Vaccination

Objective: To present a patient with acute-onset of multiple cranial neuropathies associated with recent COVID-19 vaccination. Background: Vaccine-associated neurologic adverse effects have been well-described over the decades;the influenza vaccine as well as others have been thought to precede Guillain-BarréSyndrome (GBS), Miller-Fisher Syndrome (MFS), and similar processes. Hyper-inflammatory responses have been frequently reported with SARS-CoV-2 infection and immunization, along with various neurologic pathologies. In this case report we describe a cranial polyneuropathy (3, 6, 7 and 12) associated with the COVID-19 vaccine. Design/Methods: Case Report with Video/Photos Results: A 52-year-old R-handed female presented with acute-onset, rapidly progressive deficits including left upper lid ptosis, left eye ophthalmoplegia, leftward tongue deviation, left facial paresis and dysarthria. History includes congenital left eye cataract s/p lens exchange, remote strabismus surgery and slight ptosis at baseline. She denied recent illness or injuries, though had completed single-dose vaccination for SARSCoV-2 11 days prior to symptom onset. Exam revealed new L eye esotropia with restriction in abduction and supraduction. Also noted was worsening of baseline ptosis, weak tongue protrusion with right-sided fasciculations and leftward deviation. Patient endorsed dysphagia and dysarthria. Workup consisted of three unexplanatory MRIs during week of symptom onset, lumbar puncture, evaluation by ENT and neuro-ophthalmology as well as other serum and CSF studies to investigate other autoimmune causes. Consent-obtained videos and photographs were taken for documentation/educational purposes. Follow-up visits revealed slow improvement starting three months after symptom onset. Conclusions: We outline a case of a female patient who presented with progressive, multiple cranial neuropathies with onset 11 days after single-dose SARS-CoV-2 vaccination. This constellation of symptoms in the setting of COVID-19 vaccination suggests propensity towards autoimmune neurologic processes. Further investigation is needed to determine the true incidence of similar polyneuropathies with the COVID-19 vaccine and to guide providers and patients to make informed decisions.

TRACKING DOWN AUTOANTIBODIES

Background/Aims Vaccine-associated autoimmunity is not infrequent, pertaining to either the cross-reaction between antigens or the action of adjuvant. This issue is more inexplicable to the COVID-19 vaccine, because of nucleic acid formulation and the hastened development process inflicted by the urgent pandemic condition. Here we are presenting a young patient who developed a significant abnormal autoimmune profile immediately post covid vaccination. Methods A 31-year-old IT engineer was referred to Rheumatology with postvaccine arthralgia. He had a history of recent aortic root aneurysm repair after having chest pain on exertion. Echocardiography showed dilated aortic root with significant aortic regurgitation, CT aortogram confirmed spiral type A dissection. He underwent an emergency cardiothoracic surgery in October 2020, followed by an uneventful recovery. He received the first dose of Pfizer COVID-19 vaccine on 2nd February, the very next day he developed painful ankles, knees, left hip, and right shoulder. Blood tests showed elevated CRP of 45, ESR 34, rheumatoid factor positive at 92, anti-CCP >340, ANA 13, ds-DNA 202, U1RNP positive, anti-SM antibody positive, Ro and La antibodies positive, antiJo1 antibody positive, with normal complements. He denied any swelling of the joints. No history of hair loss, photosensitive skin rashes, Raynaud's, sicca symptoms, oro-genital ulceration, or cracking of the skin. There were no constitutional symptoms, chest pain, or bowel issues. He was previously labeled as asthmatic, which is stable after surgery. He doesn't smoke or drinks alcohol. There was no family history of autoimmune conditions. On examination, he has tenderness across both hands and wrists with palmar erythema but no synovitis. He has painful right shoulder abduction with left hip pain on flexion and extension. Cardiovascular and GI examination was unremarkable apart from sternotomy scar and metallic valvular heart sounds. His dipstick urinalysis was negative for blood and protein. In recent x-rays hands and feet were normal. We agreed on a trial a tapering course of prednisolone started with 20mg daily. Three weeks later in follow-up, he reported partial response to steroids. His inflammatory markers were coming down. We have started azathioprine as a steroid-sparing agent. Results This gentleman with negative autoimmune screening prior to cardiothoracic surgery expressed florid newly detected autoantibodies straightaway after the COVID-19 vaccine. This is suggestive of undifferentiated connective tissue disease with the likelihood of overlap syndrome between rheumatoid arthritis and SLE. Conclusion COVID-19 vaccination showed a beacon of light to end the pandemic by achieving herd immunity. There is an excusable socioeconomic rush towards mass vaccination without long-term safety analysis, however, it is also crucial that any vaccine licensing process should entail meticulous scrutiny of the human proteome against vaccine peptide sequences. This will minimize the risks of acute autoimmune reactions to inoculation and future chronic autoimmune pathology.

HETEROTOPIC OSSIFICATION AFTER COVID-19 INFECTION - CASE REPORT

Background/Aims Heterotopic ossification (HO) is the abnormal formation and deposition of mature lamellar bone within soft tissue, associated with trauma, surgery, neurologic injury and prolonged immobilisation. Several recent case reports have demonstrated this condition in COVID-19 patients requiring mechanical ventilation. Methods We present a case of heterotopic ossification in the shoulder after a long stay in intensive care unit (ICU) due to COVID-19 infection. Results A 55-year-old man with stable psoriatic arthritis on sulfasalazine was admitted to ICU for mechanical ventilation after contracting COVID-19 infection. After discharge from ICU, he began noticing increasing right shoulder pain with restricted movements of abduction, internal and external rotation. His serum alkaline phosphatase was moderately elevated. Despite physiotherapy and NSAIDs, there was slow improvement. Shoulder x-ray showed significant bony overgrowth around proximal humerus which was initially thought to be part of his psoriatic arthritis. It was confirmed at Rheumatology/Radiology MDT to be heterotopic ossification. A computed tomography of the right shoulder was requested to evaluate the extent of the condition and orthopaedic advice was sought. Conclusion There are many factors contributing to the development of heterotopic ossification including trauma, spinal cord injury, brain injury, hypoxia, prolonged immobilisation with limitation of joint movement and prolonged bed rest which cause alterations in calcium homeostasis, male sex and over 60 years of age. New onset joint pain and stiffness in patients who have recovered from COVID-19 especially those who had long ICU stay should be further investigated for this condition. Treatment includes analgesia and physiotherapy with potential surgical intervention.

Return travel and Covid-19 as a grave risk of harm in Hague Child Abduction Convention cases

Since February, 2020, courts have been faced with many novel arguments concerning the Covid-19 pandemic in return proceedings under the “grave risk exception” provided in Article 13(1)(b) of the 1980 Hague Convention. This article presents an analysis of judgments delivered by courts internationally which concern arguments regarding the safety of international travel in return proceedings during the Covid-19 pandemic. While courts have largely taken a restrictive approach, important clarity has been provided regarding the risk of contracting Covid-19 as against the grave risk of harm, as well as other factors such as ensuring a prompt return despite practical impediments raised by Covid-19 and about quarantine requirements in the context of return orders. Given that the pandemic is ongoing, it is important to reflect on this case law and anticipate possible future issues. © 2022 Informa UK Limited, trading as Taylor & Francis Group.

Effect of Home-Based Tele-Pilates Intervention on Pregnant Women: A Pilot Study.

Pilates is effective for training the core muscles and stabilizing the hip joints, which provides relief from pelvic pain and low back pain during pregnancy. However, there are no specific guidelines on appropriate physical exercises for pregnant women due to the current pandemic. We aimed to apply the exercise standard proposed by the American College of Obstetricians and Gynecologists to home-based tele-Pilates exercise (HTPE), to determine its effect on the physical and mental health of pregnant women. We randomly divided the subjects into the following two groups who completed 8 weeks of HTPE (50 min/day, 2 days/week): (a) Pilates exercise (PE, n = 7) and (B) non-Pilates exercise (CON, n = 7). HTPE was performed by adjusting the program every 3 weeks, based on pain and physical fitness levels. We measured body composition, muscles of the hip joint, pelvic tilt, Oswestry Disability Index (ODI), and Pittsburgh Sleep Quality Index (PSQI), before and after HTPE. Following HTPE, while the percentage of body fat and body mass index had significantly decreased, the body fat mass did not change in the PE group (p < 0.05). The PE group showed an increase in strength of the left and right hip flexion and hip abduction, compared to the CON group (p < 0.01). The ODI and PSQI were significantly decreased in the PE group (p < 0.05). Therefore, the 8-week HTPE program is an effective exercise for pregnant woman that reduces body fat metabolism and strengthens muscles of the hip joint, thus alleviating pregnancy-induced low back pain and insomnia.