Weakness Of Upper Extremities Research Articles

Salmonella Brain Abscess in Sickle Cell Disease Patient: Case Report

Background and Clinical Significance: A brain abscess, defined as a localized intracranial infection that evolves into a purulent collection encased by a vascularized capsule, has higher prevalence among immunocompromised populations. Patients with sickle cell disease (SCD) are particularly vulnerable to bacterial infections due to their compromised immune systems, increasing their susceptibility to pathogens like Salmonella. While Salmonella is typically associated with gastroenteritis, osteomyelitis, and septicemia, its involvement in brain abscesses is exceedingly rare. There are few documented cases of Salmonella brain abscesses in the general population, and among patients with SCD, only one such case has been reported to date. In this report, we describe the second known case of a brain abscess caused by Salmonella infection in a patient with sickle cell disease, contributing to the limited literature on this rare and life-threatening condition. Case Presentation: A 32-year-old African American woman with sickle cell disease presented to the ER after a generalized seizure, reporting two weeks of worsening headaches, fevers, and left upper extremity weakness. Imaging revealed a right frontoparietal brain abscess, which was surgically drained, and cultures identified Salmonella enterica. After antibiotic treatment and a 23-day hospital stay, she was discharged. Four months later, she returned with another seizure during a sickle cell crisis, but follow-up MRI showed only minor scarring, and she was discharged on anticonvulsant therapy. Conclusions: This case emphasizes that Salmonella infections, though typically linked to osteomyelitis and sepsis, can also cause brain abscesses in immunocompromised patients like those with sickle cell disease. It highlights the need to consider infections alongside vascular causes in acute neurological cases and underscores the value of a multidisciplinary approach in managing such complex conditions.

Characteristics of Patients with Adult-Onset Dermatomyositis at 2 Tertiary Care Centres in Ontario, Canada.

Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by progressive muscle weakness and distinctive cutaneous findings. The exact incidence and prevalence of DM in the general population is largely unknown, and data on demographic and clinical features in patients in Canada are lacking. This study aimed to comprehensively evaluate the patients with DM in Southwestern Ontario, Canada. A retrospective chart review was conducted for patients with adult-onset DM at 2 tertiary care centres in Ontario, Canada, over a 13 year period. One hundred fourteen patients were included. The mean age was 59, and 80% of patients were female. Skin and muscle involvement coincided in 63% of patients, while skin predated muscle involvement in 11%. Most common characteristic skin manifestations included Gottron's papules (69%), Heliotrope sign (66%), V sign (61%), and Shawl sign (56%). Among the 114 patients, 88 (77%) were myopathic while 26/114 (23%) were clinically amyopathic dermatomyositis. In the myopathic type, upper extremity weakness, lower extremity weakness, and dysphagia were present in 87%, 81%, and 29% of patients, respectively. Elevated creatine kinase, C-reactive protein, lactate dehydrogenase, and erythrocyte sedimentation rate were found in 66%, 35%, 33%, and 32%, respectively. Lung disease was reported in 39%, particularly interstitial lung disease. Other associated features included malignancy, cardiovascular disease, and less commonly gastrointestinal disease. DM is a multifaceted autoimmune disease with distinct cutaneous and muscular findings. Our study results align with the growing body of evidence supporting DM as a complex systemic disease with the potential involvement of other organs such as the pulmonary, cardiovascular, and gastrointestinal systems.

NCMP-28. CASE REPORT: REFRACTORY MYASTHENIA GRAVIS FLARE INDUCED BY IMMUNE CHECK POINT INHIBITOR

Abstract Immune checkpoint inhibitors (ICIs) are used for treating many types of cancers. Neurological adverse effects are uncommon (incidence of 7.2%), however these effects can have life-threatening outcomes when they occur. We present a case of a 78-year-old male with an existing diagnosis of Myasthenia Gravis (MG) with metastatic Basal Cell Carcinoma (BCC). He was diagnosed in 2015 with ocular myasthenia gravis that was acetylcholine receptor antibody positive. He developed an exacerbation with double vision, fatigue, and upper extremity weakness that resolved with intravenous immunoglobulin (IVIG). During this year, he also had a thymectomy and he was controlled on Mestinon. Five months after his MG diagnosis, he was diagnosed with BCC and was given Vismodegib, however he had metastasis to the spine and lymph nodes. Unfortunately, he had no other treatment options besides immunotherapy, thus Cemiplimab was given in 2023. Ten days after receiving his 2nd dose, he developed left eye ptosis, neck weakness, shortness of breath, dysphagia, bilateral lower extremity weakness, and urinary retention. Workup additionally revealed concern for myocarditis and myositis. He received IVIG and intravenous methylprednisolone initially, and then had maintenance IVIG 1g/kg every 2 weeks with a prednisone taper. Due to ongoing symptoms, he got Rituximab which improved most of the symptoms except for dysphagia. He then received Efgartigimod alfa-fcab (Vyvgart) but had no improvement in dysphagia. Later, plasmapheresis (PLEX) was trialed but there was a partial response. Unfortunately, he obtained a PEG tube for dysphagia and declined significantly due to disease progression and multiple medical comorbidities. Myasthenia Gravis crisis from ICI therapy is a rare but serious treatment complication. In addition to the challenging nature of treating this condition, our case highlights the importance of a comprehensive work up to include consideration of overlap syndromes, such as myocarditis and myositis as identified in this case.

Post-procedural Brachial Neuritis: Clinical, Electrodiagnostic and Neuroimaging features.

Brachial neuritis is a monophasic condition affecting the brachial plexus and its branches, manifesting as acute shoulder and upper arm pain, followed by weakness and paresthesias. It can be triggered by antecedent events, including procedures such as surgery. Misdiagnosis and delay in diagnosis is common. Imaging is important to confirm the diagnosis of post-procedural brachial neuritis and exclude other etiologies. Clinical, electrodiagnostic, and neuroimaging features of patients with post-procedural brachial neuritis from a single quaternary care institution were identified and analyzed. Six (2 female) patients were identified with median age 62 (range 49-70) years. Antecedent procedures included 4 cervical spine surgeries, 1 rotator cuff repair, and 1 central venous catheter placement. Time to symptom onset ranged from 1-day to 2-weeks. The initial symptom for 5/6 patients was severe upper extremity pain followed by weakness. All patients had electrodiagnostic tests and MR neurography consistent with brachial neuritis. MR neurogram showed plexus and/or terminal branch abnormalities with associated muscular denervation edema. The C5 or C6 root, and/or upper trunk were always involved. The most common branches affected were the suprascapular, long thoracic, and axillary nerves. Hourglass constrictions of these nerves were seen in 3/6 patients. The average time to diagnosis was 3.4 (range 1.5-5) months. Post-procedural brachial neuritis is an underrecognized cause of acute upper extremity pain and weakness. MR neurography can exclude iatrogenic causes and document the presence of hourglass constrictions in affected nerves. Diagnostic neuroradiologists should be aware of this clinical entity and associated neuroimaging findings. HGC = hourglass constriction; EDX = electrodiagnostic; IRB = Institutional Review Board; ACDF = anterior cervical discectomy and fusion; NRS = Numerical Rating Scale; MUAP = motor unit action potential.

Abstract 428: The Unusual Suspect ‐ Hypereosinophilia as the Cause for Acute Stroke

Background Eosinophilic granulomatosis with polyangiitis (EGPA) causes inflammation of small blood vessels. EGPA is rare, with an annual incidence of 1 per million, with a mean age of onset of 40‐60 years. EGPA is divided into 2 phenotypes, ANCA‐positive vasculitis type and ANCA‐negative eosinophilia type. There is an overlap between other hypereosinophilic syndromes and EGPA. There are no diagnostic criteria for EGPA, however clinical features of asthma, chronic rhinosinusitis with polyps, eosinophilia, neuropathy, lung infiltrates, and eosinophilic cardiomyopathy or gastroenteritis are supportive. Central nervous system involvement manifests as cerebral infarcts and subarachnoid hemorrhages. Case: Here we present a 53‐year‐old Caucasian female with no past medical history who presented with chest pain and shortness of breath. She reported dyspnea on exertion for 8 months prior, and more recently paroxysmal nocturnal dyspnea and orthopnea, with minimal relief from inhalers. She was noted to have pansinusitis and was started on amoxicillin‐clavulanic acid without improvement. She noted acute right upper extremity weakness. Initial workup showed elevated troponin and a white blood cell (WBC) count of 28.4 with 11.64 eosinophils. CT chest showed bilateral bronchial wall thickening with bilateral nodular consolidative opacities in the right upper lobe and left lower lobe. She was started on a heparin drip. Coronary angiography was negative, and the initial echocardiogram revealed mild circumferential pericardial effusion without tamponade. CT head showed an 11 mm right cerebellar hemorrhage. Heparin was stopped. MRI brain revealed multiple scattered areas of restricted diffusion in bilateral cerebral hemispheres and a right cerebellar hemisphere. CTA head and neck showed normal vessels, and after transfer to our facility, a repeat MRI brain revealed scattered new infarcts in the left parietal and occipital regions. A repeat echocardiogram showed a small loculated pericardial effusion anterior to the right ventricle but no intracardiac shunt. The patient was exposed to wildfire smoke before symptom onset, raising suspicion of fungal infection. The patient underwent fungal evaluation in both serum and sinus aspirate, including beta‐D‐glucan in the serum, which was negative, and a bronchoscopy, which revealed white plaques and nodules with negative fungal and tuberculosis studies. Lumbar puncture revealed an elevated opening pressure of 30 mmHg but otherwise unremarkable infectious studies. Antibiotic therapy included vancomycin, cefepime, and amphotericin, but with consistently elevated WBCs of more than 24 with eosinophilic predominance. Endomyocardial biopsy showed numerous eosinophils. Subsequent endobronchial biopsy showed eosinophilic infiltrates and features suspicious for vasculitis. ANCA and bone marrow biopsy were negative. The primary diagnosis was considered hypereosinophilic syndrome. She was started on pulse dose steroids, and WBC count fell from 24.1 to 11.8 with a decrease in eosinophils from 13.05 to 0.01. Outpatient rheumatology was planned to initiate cyclophosphamide or rituximab. Dual antiplatelet therapy was initiated before discharge. Conclusion Central nervous system sequelae of EGPA are rare, but this diagnosis should be considered in patients with symptoms of sinusitis, elevated eosinophils, and acute stroke, especially without other risk factors. Since ANCA is only positive in about 30‐40% of patients, an early biopsy is needed for confirmation and early treatment with steroids.

Abstract 460: Variation Of Bowhunter's Syndrome: Right Vertebral Artery Occlusion From Cervical Disc Herniation ‐ A Case Report

Introduction Bowhunter's syndrome (BHS) occurs when an individual turns their head, often greater than 45 degrees, causing mechanical occlusion of the ipsilateral vertebral artery (VA). Common causes include osteophytes or disc herniations [1]. This dynamic stenosis results in symptoms of vertebrobasilar insufficiency (VBI) such as vertigo, nystagmus, dizziness, and changes in vision and gait [3]. BHS is a rare but curable cause of VBI. Methods/Case Presentation A 59‐year‐old woman with a history of congestive heart failure, hypertension, obstructive sleep apnea, cervical spondylosis, thoracic spondylosis, atherosclerotic cardiovascular disease, hyperlipidemia, and type 2 diabetes mellitus presented with recurrent presyncope, vertigo, and progressive right upper and lower extremity weakness. Her symptoms worsened with head rotation to the right. Cerebral angiogram showed chronic occlusion of the left VA and rotational occlusion of the V2 segment of the right VA, which had 50% baseline occlusion and 80% occlusion with head turned to the right. The stenosis occurred greatest at C3‐4 due to a lateral cervical disc herniation. The patient possessed a small right posterior communicating artery and lacked collateral flow from the left VA. Results She was treated with a C3‐4 anterior cervical discectomy and fusion (ACDF) and skeletonization of right VA with resolution of positional occlusion of the VA. Postoperatively, her dizziness and instability resolved, but she experienced new dysphagia and operative site discomfort. Right extremity weakness persisted with 4/5 muscle strength in the right deltoid, bicep, tricep, wrist extension, grasp, and iliopsoas. Postoperative x‐rays showed good positioning of her C3‐4 interbody graft, anterior cervical plate, and screws. Two months later, a follow‐up cerebral angiogram confirmed resolution of right VA stenosis in all head positions. Conclusion This case provides insights into alternative presentations of BHS. Notably, the right VA was the primary symptomatic vessel, whereas the left VA is historically more affected [2]. More common causes like cervical spine trauma, neoplastic growths, and osteophytes may present with a larger constellation of symptoms that delay diagnosis [4]. A key takeaway is the body's adaptation to changes in head and neck perfusion. In healthy individuals, the contralateral VA compensates for posterior circulation demands [3]. This patient showed a combination of contralateral VA atherosclerosis and a lack of protective collateralization. Conservative management is effective for approximately 75‐90% of cervical spine disc herniations, but the risk of cerebrovascular complications in this patient necessitated surgical intervention [5]. The anterior approach to discectomy has shown better outcomes compared to posterior decompression, with fewer instances of VA injury [6]. Vertebral stenting is an alternative that has historically shown success [7]. However, the risk of stent deformation and fracture in the cervical spine was considered too great. Her persistent right extremity weakness suggests possible secondary etiology or lasting deficits from transient cerebral hypoperfusion. As with any cause of VBI, BHS should be considered as a risk factor for severe cerebrovascular events. This case continues to support digital subtraction angiograms for diagnosis and ACDF for surgical treatment as a successful pathway for those with BHS [6].

Abstract 232: Complex Management Of Cerebral Venous Sinus Thrombosis In A Pregnant COVID‐19 Patient: Case Report

Introduction Cerebral venous sinus thrombosis (CVST) is a rare but serious condition characterized by blood clot formation in the dural venous sinuses. It can manifest with symptoms ranging from mild headaches to severe complications such as cerebral herniation and death. CVST predominantly affects women aged 20‐40, comprising about 0.5‐1% of acute ischemic events. Although systemic anticoagulation is the first‐line treatment, 10‐20% of cases require further interventions like mechanical thrombectomy and/or localized thrombolysis when medical management alone fails. This case report describes a complex instance of CVST in a 30‐year‐old pregnant woman with COVID‐19 refractory to systemic anticoagulation requiring careful further neurointervention. Results 30‐year‐old pregnant female with COVID‐19 infection presented to the emergency department with acute progressive encephalopathy, nausea, vomiting, and aphasia. She was eight weeks pregnant at this time. Initial brain imaging revealed extensive thrombi in the superior sagittal sinus, vein of Galen, bilateral transverse sinus, sigmoid sinus, and jugular sinuses accompanied by cerebral edema. Due to severe agitation and worsening neurological status, the patient was intubated for further management. Magnetic resonance imaging (MRI) confirmed the extensive bilateral thrombosis and revealed left frontoparietal edematous changes consistent with distal venous infarcts. Given the severity of her condition, a heparin drip was started, but the thrombotic burden persisted. After consulting with neurointerventional radiology and discussing the risks with her husband and family, the team proceeded with an emergent venous thrombectomy. Thrombus retrieval was attempted with five passes using an ACE68 aspiration catheter. Despite multiple attempts, post‐thrombectomy angiography showed persistent extensive thrombosis. A follow‐up Computerized Tomography (CT) scan revealed evolving venous infarcts with signs of increased intracranial pressure including sulci effacement but no hemorrhage. Given the clot burden, further mechanical intervention was not possible, and an infusion of alteplase (tPA) was started via right internal carotid catheter. The following day, new small cortical hemorrhages were noted on CTB, leading to the cessation of thrombolytic therapy. However, repeat CT venogram showed significant reduction of clot burden. Following days, the patient was extubated and transitioned to oral anticoagulation therapy with Lovenox, later switched to Coumadin. Hematological evaluation revealed she was a heterozygous carrier for Factor V Leiden and Factor II mutation, increasing her risk for thrombotic events. Over time, she showed gradual improvement, with mild residual left upper extremity weakness responding well to physical therapy. Conclusion This case highlights the challenges of managing extensive CVST in a pregnant patient with COVID‐19. It demonstrates the successful use of both neurointervention and thrombolytic therapy in a scenario where conventional anticoagulation failed. It highlights the potential for improved patient outcomes by integrating these advanced therapeutic strategies in complex, refractory cases, underscoring the importance of a multidisciplinary approach.

Beyond the Surface: Uncovering the PML::RARA Fusion in Cytogenetically Cryptic and FISH-Negative Acute Promyelocytic Leukemia- A Case Report and Review of the Literature

Abstract Introduction/Objective Chromosomal t(15;17)(q22;q21), resulting in a fusion between the PML gene on chromosome 15q22 and the RARA gene on chromosome 17q21, is the cytogenetic hallmark of acute promyelocytic leukemia (APL). Conventional karyotyping, along with fluorescence in situ hybridization (FISH), can detect the PML/RARA rearrangements in up to 98% of APL cases. Reverse transcriptase-polymerase chain reaction (RT-PCR) is often used to confirm the initial diagnosis. APL with PML::RARA fusion, which is negative with FISH and conventional karyotyping approaches but positive with RT-PCR, is extremely rare. Herein, we report a de novo APL case that was positive by RT-PCR but negative by cytogenetics and FISH studies. A comprehensive review of the literature was also performed. Methods/Case Report A 23-year-old male presented to the ED for left upper extremity weakness 2 days after post- dental cleaning. Upon admission, he developed intracranial hemorrhage, necessitating urgent decompressive hemicraniectomy. Blood smear revealed promyelocytes; flow cytometric analysis showed a CD13+, CD33+, MPO+, CD117+, CD34-, and HLA-DR- abnormal cell population. STAT interphase FISH analyses for PML/RARA fusion and RARA break-apart were negative. A diagnosis of APL was made based on abnormal promyelocytes and characteristic flow cytometry findings. ATRA was immediately administrated. However, the post-operative course was complicated by significant coagulopathy, pancytopenia, and multi-organ failure, and the patient expired 7 days later. RT-PCR returned positive for PML::RARA long fusion transcript; the conventional cytogenetics study was uninformative. The clinical, pathological, and genetic features of 31 cases of FISH/Cytogenetics-negative APL harboring cryptic rearrangements of PML-RARA, detected by RT-PCR in the literature, have been reviewed. Various detection methods were also discussed. Results (if a Case Study enter NA) N/A Conclusion Conventional cytogenetics, FISH and RT-PCR analysis are complementary studies for all clinical suspected APL cases. The clinicopathological characteristics also play critical roles in APL diagnosis when genetic confirmation of the PML::RARA fusion is unavailable.

8291 Graves’ Disease and Moyamoya Syndrome: A Complex Case of Thyroid Dysfunction and Neurovascular Abnormalities

Abstract Disclosure: A. Jamil: None. Q.V. Luong: None. A. Llorens Bonilla: None. A. Siddiqui: None. Background: Moyamoya disease is a cerebrovascular disease characterized by progressive stenosis and occlusion of cerebral arteries, particularly the internal carotid arteries, proximal anterior and middle cerebral arteries associated with a net-like collateral vessel formation. Moyamoya disease in association with certain systemic conditions (e.g. Graves’ disease, sickle cell anemia, neurofibromatosis type 1, and Down Syndrome) has been referred to as moyamoya syndrome. Graves’ disease is an autoimmune disease in which the development of thyroid-stimulating hormone (TSH) receptor autoantibodies leads to overproduction of thyroid hormones. Although the pathophysiology is unclear, Graves’ disease can adversely impact the clinical course of moyamoya syndrome.Clinical Case: A 26-year-old woman diagnosed with Graves’ disease at the age of 20 years who was in remission presented to the ED with right upper extremity weakness and headache. A stroke work-up was performed, and an initial CT head without contrast showed an acute infarct in the left temporal area. Extensive workup for hypercoagulability and cardiac causes were negative. MRI brain showed multiple infarcts of the left cerebral hemisphere with ivy sign, which are prominent linear or punctate high intensity areas in the subarachnoid space reflecting the development of collaterals that occurs in moyamoya disease. TSH at the time was noted to be <0.01 uIU/mL (range: 0.30-4.20 uIU/mL), indicating a thyrotoxic state. She was discharged on methimazole and antiplatelet therapies. However, the patient continued to have hypertension and headaches which did not resolve with the return to euthyroidism. At age 29, she again developed acute right upper and lower extremity weakness, and consequently presented to the ED. Imaging studies showed multiple punctate infarcts in her bilateral frontal lobes and bilateral ICA stenosis consistent with moyamoya disease. However, her biochemical studies showed euthyroidism. Ultimately, she underwent right frontotemporal craniotomy and direct parietal superficial temporal artery to middle cerebral artery bypass by neurosurgery to decrease her future stroke risk for her moyamoya syndrome, and her Graves’ disease remains in remission.Conclusions: The coexistence of Graves’ disease in moyamoya syndrome may suggest a pathophysiologic connection. It has been suggested that hemodynamic changes in cerebrovascular flow due to thyrotoxicosis might contribute to ischemic neurologic deficits. Surgical intervention for revascularization procedures should be attempted after optimal control of thyrotoxicosis, as this may help to prevent further ischemic events in the future and improve patient outcomes. Presentation: 6/1/2024

Prediction of Upper Limb Motor Recovery by the PREP2 Algorithm in a Nonselected Population: External Validation and Influence of Cognitive Syndromes

Background Early prediction of poststroke motor recovery is challenging in clinical settings. The Prediction recovery potential (PREP2) algorithm is the most accurate approach for prediction of Upper Limb function available to date but lacks external validation. Objectives (i) To externally validate the PREP2 algorithm in a prospective cohort, (ii) to study the characteristics of patients misclassified by the algorithm, and (iii) to compare the performance according to the presence of cognitive syndromes (aphasia, neglect, cognitive disorders). Methods We enrolled 143 patients with stroke and upper extremity weakness persistent at Day 3. Evaluation to predict the recovery status according to the PREP2 algorithm included age, SAFE and NIHSS scores at Day 3 and transcranial magnetic stimulation to determine the presence of the motor-evoked potential before day seven. Actual recovery (excellent, good, limited, or poor) was defined based on the Action Research Arm test score at 3 months. Accuracy was computed by comparing the predictions of the PREP2 and the actual category of the patient. Additionally, to investigate misclassifications and the impact of cognitive syndromes, we recorded SAFE and NIHSS scores at Day 7, the Montreal Cognitive Assessment (MoCA) score, the presence of aphasia and neglect and Magnetic Resonance Imaging was used to evaluate the corticospinal tract lesion load. Results The PREP2 algorithm showed a very good predictive value with 78% accuracy [95% CI: 71.2%-86.1%], especially for the extreme categories of recovery (EXCELLENT 87.5% [95% CI: 78.9%-96.2%] and POOR 94.9% [95% CI: 87.9%-100%]), and only 46.5% [95% CI: 19.05%-73.25%] for the GOOD category and even worse than chance for the LIMITED category 0%. Pessimistic predictions (false-negative cases) had a drastic improvement in the SAFE score acutely compared to that of well-predicted patients with unfavorable recovery (P < 001). The predictive value of PREP2 decreased significantly when patients had cognitive disorders (MoCA score <24) versus not (69.4% [95% CI: 52.8%-86.1%] vs 93.1% [95% CI: 83.9%-100%], P = .01). Conclusion Our study provides an external validation of the PREP2 algorithm in a prospective population and underlines the importance of taking into account cognitive syndromes in motor recovery prediction.

Effect of Preoperative Motor Weakness on Postoperative Clinical Outcomes in Patients Undergoing Cervical Disk Replacement

Study Design: This is a retrospective review Objective: To examine the effect of preoperative motor weakness on clinical outcomes in patients undergoing cervical disk replacement (CDR). Summary of Background Data: Studies examining the effect of preoperative motor weakness on postoperative clinical outcomes in CDR are limited. Methods: Patient cohorts were based on documented upper-extremity motor weakness on physical exam versus no motor weakness. Demographics, perioperative characteristics, and preoperative patient-reported outcome measures (PROMs) were compared using univariate inferential statistics. PROMs consisted of Visual Analog Pain Scale-Neck (VAS-N), Patient-Reported Outcomes Measurement Information System Physical Function (PROMIS-PF), VAS-Arm (VAS-A), 12-Item Short Form (SF-12) Physical Component Score (PCS), Oswestry Neck Disability Index (NDI), and SF-12 Mental Component Score (MCS). Postoperative PROMs were collected at the 6-week, 12-week, 6-month, and final follow-up up to 1-yeartime points, and intercohort minimum clinically important difference (MCID) achievement was compared through multivariable linear logistic regression adjusting for significant differences in preoperative characteristics. Results: A total of 118 patients formed cohorts based on documented upper-extremity weakness (n=73) versus no weakness (n=45). The average time to postoperative follow-up was 9.7±7.0 mo. The differences in insurance type between the 2 cohorts were significant (P&lt;0.042). Perioperative diagnosis of foraminal stenosis was significantly more common in the motor weakness cohort (P&lt;0.013). There were no differences in reported PROMs between cohorts. Patients with motor weakness reported significant MCID achievement for PROMIS-PF at 6-/12-weeks (P&lt;0.012, P&lt;0.041 respectively), SF-12 PCS at 6-months (P&lt;0.042), VAS-N at final follow-up (P&lt;0.021), and NDI at final follow-up (P&lt;0.013). Conclusions: CDR patients with preoperative muscle weakness achieved MCID across several PROMs compared with patients without muscle weakness. Patients with motor weakness reported greater improvement in mental health, pain, and disability as early as 6 weeks and up to 1 year after CDR. This information serves to inform physicians that motor weakness may not indicate a negative overall outcome.

Focal motor weakness and recovery following chronic subdural hematoma evacuation.

The incidence of chronic subdural hematomas (cSDHs) is expected to climb precipitously in the coming decades because of the aging populous. Neurological weakness is one of the most common presenting neurological symptoms of cSDH. Yet, the recovery rates of motor strength recovery are seldom documented, as neurological outcomes have predominantly focused on broader functional assessment scores or mortality. In this study, the authors performed one of the first detailed analyses on functional motor weakness and recovery in patients who underwent cSDH evacuation. Patients who underwent evacuation of a cSDH at a tertiary academic medical center between November 2013 and December 2021 were retrospectively identified using ICD-9 and ICD-10 billing codes. The presence of focal motor weakness was subcategorized by location as upper extremity (UE) or lower extremity (LE). Postoperative improvement, worsening, or resolution of weakness was recorded at the time of discharge. Statistical analysis included univariate and backward stepwise multivariable logistic regression modeling. A total of 311 patients were included in the analysis. Patients were significantly more likely to experience UE weakness than LE weakness (29% vs 18%, p < 0.001). Forty-one percent (43/104) had both UE and LE weakness present. Risk factors for the development of focal motor weakness at the time of presentation were older age (OR 1.02, p = 0.03), increased cSDH size (OR 1.04, p = 0.02), and the presence of a unilateral cSDH (OR 2.32, p = 0.008). The majority of patients (68%, 71/104) experienced motor strength improvement following cSDH evacuation, with 58% (60/104) having complete resolution of weakness. Multivariable logistic regression analysis revealed that longer symptom duration was associated with lower rates of improvement (OR 0.96, p = 0.024). Older age was also associated with reduced resolution of weakness (OR 0.96, p = 0.02). This study represents one of the first in-depth analyses investigating the rates of motor strength weakness and recovery following cSDH evacuation. Nearly two-thirds of all patients had complete resolution of their weakness by the time of discharge, and more than three-quarters had partial improvement. Risk factors for impaired neurological recovery were longer symptom duration prior to treatment and older age.

Hirayama disease successfully treated by posterior fixation: a case report

Introduction and importance: Hirayama disease is a rare form of motor neuron amyotrophy that usually presents with weakness and atrophy of the distal upper extremities in young males. It is believed that it is caused by spinal cord compression during neck flexion because of the widening of the posterior extradural space. This case has been brought to attention due to its extraordinary rarity, serving as an educational tool for medical professionals and to advocate for surgical intervention when deemed necessary. Case presentation: The authors present a case of a young male in his 20s who was diagnosed with Hirayama disease, had weakness and atrophy in both of his upper limbs, and has been successfully treated by posterior fixation at C4, C5, and C6 with lateral mass screws. Clinical discussion: The majority of cases stabilize after 2–3 years of progression; therefore, cervical collars are generally sufficient for therapy. However, in certain serious cases with progression even after that time, surgical intervention is an option. Because this is such an uncommon incidence, surgical therapy has not been explored and is controversial. Conclusion: The use of posterior fixation at C4, C5, and C6 with lateral mass screws as a therapy for Hirayama disease may be regarded as a successful approach.

P.066 A 65 year-old male with subacute asymmetric, proximal more than distal upper extremity weakness with associated paresthesias and pain

Background: Neuroborreliosis affects approximately 10-15% of people with untreated Lyme disease and typically declares itself 2-18 weeks after infection. North American neuroborreliosis often manifests with cranial nerve palsy, meningitis, and/or radiculoneuritis. Methods: Here we describe a case of North American neuroborreliosis and also highlight some of the rare manifestations of systemic Lyme disease. A 65-year old male presented with a subacute history of progressive upper extremity weakness, neck pain, and headache. This occurred in the context of a recent tick exposure. Results: MRI of the brachial plexus, serology and CSF studies, and EMG/NCS were consistent with a diagnosis of polyradicular neuroborreliosis. However, whole body imaging identified some concerning features suggestive of lymphoma: specifically a large necrotic mediastinal lymph node and a number of vascular abnormalities. In light of these findings, the differential also included neurolymphomatosis and a PET scan was conducted. Reassuringly, there was no increase in FDG avidity in the distribution of his affected nerves. Moreover, his neurologic symptoms exhibited clinical improvement following treatment of his neuroborreliosis. Conclusions: This case provides an excellent example of the clinical features of neuroborreliosis, but more importantly also highlights some of the rarer potential manifestations, which warrant further investigation.

An almost forgotten neurological entity: man in a barrel syndrome

Man in a barrel syndrome (MIBS) presents with bilateral upper extremity weakness but preserved strength in face, neck, and lower extremities. In this case report, two cases of MIBS were admitted to the emergency department (ED) presented. The first patient injured his neck after falling off a ladder. The second patient was a victim of a traffic accident. Bilateral upper extremity weakness was examined in the first patient at first admission to the ED. On the other hand, progressive weakness was shown in the second patient during ED follow-up. The cervical imaging findings consisted with MIBS. This case report and review of literature highlights that physicians should consider every possible cause, even the rarest ones when a patient complains of neck trauma with non-specific symptoms. Rapid identification and treatment of treatable causes such as cerebral hypoperfusion are vital for patient prognosis.

C5 palsy following esophageal diverticulum resection.

C5 palsy (C5P) is a recognized potential postoperative complication of cervical spine surgery but has rarely been reported following an open esophageal diverticulectomy. A 61-year-old underwent an open esophageal diverticulectomy for symptomatic Zencker's diverticulum. Postoperatively, she presented with right upper extremity weakness and sensory deficits consistent with a C5P that was later confirmed by electromyography. The potential for C5P after esophageal diverticulectomy for symptomatic Zencker's diverticulum is rare. Postoperative recognition and appropriate management are critical to recovery.

Clinical Subsets of Central Cord Syndrome: Is It a Distinct Entity from Other Forms of Incomplete Tetraplegia for Research?

Central cord syndrome (CCS) is the most prevalent and debated incomplete spinal cord injury (SCI) syndrome, with its hallmark feature being more pronounced weakness of the upper extremities than of the lower extremities. Varying definitions encapsulate multiple clinical features under the single umbrella term of CCS, complicating evaluation of its frequency, prognosis discussions, and outcomes research. Often, people with CCS are excluded from research protocols, as it is thought to have a favorable prognosis, but the vague nature of CCS raises doubts about the validity of this practice. The objective of this study was to categorize CCS into specific subsets with clear quantifiable differences, to assess whether this would enhance the ability to determine if individuals with CCS or its subsets exhibit distinct neurological and functional outcomes relative to others with incomplete tetraplegia. This study retrospectively reviewed individuals with new motor incomplete tetraplegia from traumatic SCI who enrolled in the Spinal Cord Injury Model Systems (SCIMS) database from 2010 to 2020. Through an assessment of the prevailing criteria for CCS, coupled with data analysis, we used two key criteria, including the severity of distal upper extremity weakness (i.e., hands and fingers) and extent of symmetry, to delineate three CCS subsets: full CCS, unilateral CCS, and borderline CCS. Of the 1490 participants in our sample, 17.5% had full, 25.6% had unilateral, and 9% had borderline CCS, together encompassing >50% of motor incomplete tetraplegia cases. Despite the increased sensitivity and specificity of these subsets compared with existing quantifiable criteria, substantial variability in clinical presentation was still observed. Overall, individuals meeting CCS subset criteria showed a higher likelihood of American Spinal Injury Association (ASIA) Impairment Scale (AIS) D grade than those with motor incomplete tetraplegia without CCS. Upper Extremity Motor Score (UEMS) for those with CCS was lower on admission, a difference that diminished by discharge, whereas their Lower Extremity Motor Score (LEMS) consistently remained higher than for those without CCS. However, these neurological distinctions did not result in significant functional differences, as lower and upper extremity functional outcomes at discharge were mostly similar, with some differences observed within those with AIS D grade. The AIS grade, rather than the diagnosis of CCS, remains the foremost determinant influencing neurological and functional outcomes. We recommend that future studies consider incorporating motor incomplete tetraplegia into their inclusion/exclusion criteria, instead of relying on criteria specific to CCS. Although there remains clinical value in characterizing an injury pattern as CCS and perhaps using the different subsets to better characterize the impairments, it does not appear to be a useful research criterion.

Pancoast Syndrome Due to Metastatic Hepatocellular Carcinoma

Pancoast tumors are aggressive and locally invasive apical lung cancers that often compress and infiltrate adjacent structures including the brachial plexus, stellate ganglion, ribs, and vertebral bodies. Symptoms of Pancoast tumors include Pancoast Syndrome, characterized by Horner Syndrome and pain and weakness of the ipsilateral upper extremity. Pancoast tumors by definition are primary lung cancer and are nearly always non-small cell lung cancer (NSCLC). There are rare case reports of metastatic non-pulmonary malignancies forming apical lung tumors mimicking Pancoast tumors and causing Pancoast Syndrome. Here, we present a case of a 66-year-old male with a history of unresectable hepatocellular carcinoma (HCC) who presented with one year of progressive left wrist and forearm pain and hand weakness and was found on cross-sectional imaging to have a left upper lobe lung mass invading the T1 and T2 vertebral bodies. Pathology showed very poorly differentiated carcinoma that required specialized testing to achieve diagnostic clarity. The tumor was positive for albumin mRNA, which aided in confirming the lung mass as metastatic HCC. This case highlights a rare incidence of Pancoast Syndrome due to metastatic HCC and emphasizes the importance of considering this diagnosis as an etiology of left upper extremity pain in a patient with previously diagnosed malignancy. This case also demonstrates the utility of albumin mRNA in-situ hybridization as an additional tool in identifying metastatic HCC and reviews general principles of symptomatic management of Pancoast Syndrome.

Arteriovenous malformation presenting as complex regional pain syndrome: illustrative case.

Complex regional pain syndrome (CRPS) is typically described as a peripheral nerve disorder in which exaggerated allodynia and hyperalgesia follow a minor injury. Some researchers propose a central mechanism, although current evidence is lacking. A 14-year-old female presented with classic CRPS symptoms of left upper-extremity weakness and hyperalgesia after a bout of sharp pain in her thumb while shoveling snow. A possible seizure prompted magnetic resonance imaging, revealing a right frontal Spetzler-Martin grade II arteriovenous malformation (AVM) adjacent to the primary motor cortex. Brodmann areas 1, 3a, and 3b, which are responsible for localizing and processing burning and painful sensations, were also involved. The patient underwent transarterial Onyx embolization in two sessions and microsurgical resection, after which her CRPS symptoms completely resolved. To our knowledge, this is the first reported case of a cerebral AVM presenting as CRPS, which supports a central mechanism. The authors propose that rapid growth of the AVM led to a vascular steal phenomenon of surrounding parenchyma, which disrupted the patient's normal motor function and nociceptive processing. Further validation in other series is needed.

Trans-Sternal Multilevel Corpectomy for Cervicothoracic Renal Cell Metastasis: 2-Dimensional Operative Video.

The operative management of pathological fractures at the cervicothoracic junction is a surgical challenge. Here, we present the case of a 48-year-old male patient presenting with 2 months of progressive left upper extremity weakness as well as back and bilateral arm pain (Karnofsky Performance Status 60%) who was found to have pathological fractures from C7, T1, and T2 due to metastatic renal cell carcinoma. Renal cell carcinoma is known to metastasize to bone and cause cord compression.1 Given the extensive metastasis with this highly vascular tumor, endovascular embolization was performed preoperatively to minimize intraoperative blood loss.2 Surgical management consisted of a two-stage procedure. Posterior spinal fusion from C2-T7 with C7-T2 decompression was performed during stage 1. Stage 2 consisted of a trans-sternal approach for C7, T1, and T2 corpectomy for cord decompression and placement of a cage and plate for anterior column support.3 Although prior surgeons have suggested to access upper thoracic pathology through an interaortocaval window, in this case we demonstrate a trans-sternal approach to C6-T3 that starts superior to the innominate vein and aortic arch and angles inferiorly dorsal to these vascular structures.4 When planning for a manubriotomy/trans-sternal approach, access to T1/T2 remains the most decisive factor and is most successful with a sternotomy.5 At 12-month follow-up, the patient demonstrated improvement in his left upper extremity strength and overall functional status (3/5 strength in hand grip and interossei with 5/5 in all remaining motor groups; Karnofsky Performance Status 80%). The patient consented to participate in the surgery and surgical video.