Bilateral Lower Extremity Weakness Research Articles

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.

Unilateral Biportal Endoscopic Decompression for Thoracic Spinal Stenosis Secondary to Ossification of the Ligamentum Flavum

Ossification of the ligamentum flavum (OLF) in the thoracic spine is a cause of thoracic myelopathy that is more commonly found in East Asian populations (Koreans, Japanese, Chinese) than in others. Early diagnosis and sufficient surgical decompression can improve the functional prognosis for thoracic OLF. Surgical decompression is necessary and should be done as soon as the symptoms develop. There is a wide range of possible treatments, from standard open laminectomy to endoscopic decompression surgery. This video demonstrates the least invasive technique of decompressive laminectomy with bilateral decompression and removal of thoracic OLF through unilateral biportal endoscopic spinal surgery. The authors present the case of an 81-year-old male patient who presented with bilateral lower extremity weakness, numbness, pain, and ataxia. Magnetic resonance imaging and computed tomography scans of the whole spine showed the presence of OLF with severe spinal canal stenosis and a cord signal change at the T9–10 level. He underwent biportal endoscopic decompression and removal of thoracic OLF at T9–10. On the discharge day (postoperative day 7), motor power and pain had significantly improved. He could stand and independently ambulate with a walker.

6812 A Rare Case of Polyglandular Autoimmune Syndrome Type 3B Presenting with Unsteady Gait Due to Subacute Combined Degeneration of the Spinal Cord Associated with Pernicious Anemia

Abstract Disclosure: F. Mohammadrezaei: None. F. Sajid: None. C.A. Resta: None. J.G. Karam: None. A Rare Case of Polyglandular Autoimmune Syndrome Type 3B Presenting with Unsteady Gait due to Subacute Combined Degeneration of the Spinal Cord associated with Pernicious Anemia Background: The coexistence of Hashimoto's thyroiditis and pernicious anemia is well established and is recognized as Polyglandular Autoimmune Syndrome (PAS) Type 3B [1]. We describe a rare case of a patient presenting with subacute combined degeneration of the spinal cord leading to the diagnosis of PAS Type 3B. Case Presentation: A 37-year-old man with no known medical history presented to the Emergency Department after a fall in the subway station. On history, he complained of progressive bilateral lower extremity weakness of 10 months duration, necessitating assistance with ambulation, and significant weight gain, described as an increase in his pants size from 40 to 56. His physical examination was relevant for weight of 145kg, BMI of 46.5, normal motor strength and sensation of the upper extremities, decreased strength at 4 out of 5 of bilateral hip flexion and extension, and decreased vibration sensation of bilateral lower extremities. He could not fully stand or walk due to imbalance and weakness. Blood testing revealed a hemoglobin level of 11.4 gm/dl (14-18), MCV of 108.8 FL (80-94), a significantly low vitamin B12 at 89 pg/ml, and normal folate level. His TSH level was 9.26 MCIU/ML (0.39-4.08), with a free T4 of 0.74 ng/dl (0.8-1.8) and significantly elevated Thyroid Peroxidase Antibody titer at 4320 IU/ml (< 34.9). Intrinsic factor antibody and parietal cell antibody tests both came back elevated at 13.4 AU/mL (0.0-1.1) and 1.8 (<1.2), respectively. A head CT scan showed no acute pathology, but MRI spine with contrast revealed abnormal T2 hyperintensity, primarily affecting the lateral columns throughout the cervical and thoracic cord, likely indicative of subacute combined degeneration. The patient was diagnosed with autoimmune Hashimoto's thyroiditis, pernicious anemia, and subacute combined degeneration of the spinal cord due to vitamin B12 deficiency. He was treated with daily vitamin B12 1000mcg muscular injections, levothyroxine 75mcg orally daily (after ruling out adrenal insufficiency), and physical therapy. Conclusion: This case highlights the complexity of polyglandular autoimmune syndromes and the importance of recognizing atypical presentations early in the history of disease. Comprehensive evaluation and timely intervention are essential to mitigate the progression of these conditions and improve patients' quality of life. Reference: (1) Betterle C, Furmaniak J, Sabbadin C, Scaroni C, Presotto F. Type 3 autoimmune polyglandular syndrome (APS-3) or type 3 multiple autoimmune syndrome (MAS-3): an expanding galaxy. J Endocrinol Invest. 2023 Apr;46(4):643-665. Presentation: 6/1/2024

Surgical treatment and outcome of spinal arteriovenous malformations.

A rare and diverse set of abnormalities formed by spinal blood arteries known as spinal arteriovenous malformations (AVMs) has a higher risk of hemorrhage and morbidity, causing the shunting of blood from veins with an aberrant capillary bed to arteries. Knowledge of the vascular anatomy of spinal AVMs and the spinal cord's vascular supply is crucial to its therapy. The aim of this study was to report a case of SAVM healing after surgical excision, highlighting the importance of surgical excision in treating these rare lesions. A 56-year-old male with progressive bilateral lower extremity weakness and numbness was diagnosed with spinal cord arteriovenous malformation (SAVM). A thoracolumbar spine MRI was performed, and a vascular lesion was found. The feeding artery was coagulated and incised, and the surrounding hematoma was removed. Spinal cord arteriovenous malformation was diagnosed postoperatively. The Oswestry Disability Index (ODI) and the SF-36 questionnaires assessed functional outcomes and physical functioning in individuals with acute or chronic low back pain. In the reported case, the aneurysm showed spontaneous regression at follow-up after surgical removal of the AVM. Post-surgery rehabilitation improved his motor function. After seven months, he showed 90% physical functioning, 75% emotional well-being, 75% social functioning, 87.5% pain, and 55% general health. He could walk with a walking aid and perform daily activities. Treatment strategies for AVM-related spinal aneurysms should be carefully considered. Surgical treatment in several conditions has advantages and is reported to have good outcomes.

Plasmapheresis in post-COVID-19 myelitis: A case report.

Previous studies have delineated different neurological manifestations associated with coronavirus disease 2019 (COVID-19). Myelitis is identified as a rare neurological complication resulting from a COVID-19 infection. Limited information is available regarding the treatment of patients experiencing this condition. This report extracts data from the medical record of a post-COVID-19 myelitis patient at Buriram Hospital and follows up prospectively on the patient's symptoms after treatment. A 61-year-old man, previously vaccinated for COVID-19 and with a history of hypertension and dyslipidemia, experienced progressive bilateral lower-extremity weakness (recorded as muscle strength grade 2/5 in both lower extremities) for 6 weeks. He had a mild case of COVID-19 2 months earlier, which resolved in 10 days without specific treatment. However, 2 weeks after being diagnosed with COVID-19, he developed weakness in his lower limbs, numbness below the nipple, and urinary retention. Spinal magnetic resonance imaging revealed multifocal longitudinal myelitis. Despite initial treatment with methylprednisolone, the patient showed no clinical improvement. Consequently, he underwent five cycles of plasmapheresis. Three months after discharge, a notable improvement was observed, with his muscle strength graded at 4/5 in both lower extremities and the resolution of sensory and urinary symptoms. We presented the case of a COVID-19-vaccinated patient, in whom COVID-19 infection might have led to myelitis. We found promising results in treating prolonged COVID-19-related myelitis symptoms through the use of plasmapheresis.

Transarterial Embolization of Intracranial Dural Arteriovenous Fistulas Cognard Type V With Direct Drainage Into the Perimedullary Veins: Case Report and Literature Review.

Cognard type V fistula (CVF) is a rare type of dural arteriovenous fistula characterized by spinal perimedullary venous drainage. Owing to the lack of pathognomonic findings, misdiagnosis is common. Patients often undergo multiple spinal angiograms negative for spinal vascular malformations. Digital subtraction angiography is the gold standard diagnostic tool. The preferred treatment option is endovascular management with embolization through a transarterial, transvenous, or combined approach. Other options include open surgery, stereotactic radiosurgery, or a combination of both. The patient from case # 1 presented with progressive weakness and hypoesthesia in the bilateral lower extremities, with urinary and bowel incontinence. The DSA identified a CVF fed by the meningohypophyseal trunk and a draining perimedullary vein. Embolization with 0.1 ccs of Onyx-18 was performed with complete fistula occlusion. The patient from case # 2 developed bilateral lower extremity weakness, diffuse numbness, and urinary incontinence. The DSA showed a CVF fed by tributaries from the ascending pharyngeal artery and posterior meningeal artery branches of the V3 segment, draining into a perimedullary vein. Embolization with 0.3 cc of Onyx-18 was performed with 100% occlusion of the fistula. A 1-year follow-up angiogram confirmed complete fistula occlusion. Both patients consented to the procedure. Even if a patient only presents symptoms of myelopathy, CVF should be considered. Herein, we present 2 cases of CVF with direct drainage into the perimedullary veins which presented exclusively with myelopathy syndrome and describe treatment with trasarterial embolization with Onyx.

Longitudinally extensive transverse myelitis, a disabling disorder with a good prognosis: a case series from Nepal.

Longitudinally extensive transverse myelitis (LETM) is a rare spinal cord disorder with variable etiologies and presentations. It can present solely or as an association with other neurological disorders. It was a series of cases of LETM in a tertiary care hospital. The initial three cases presented with bilateral lower extremity weakness and were diagnosed as transverse myelitis while, the fourth case, already diagnosed as LETM presented with seizure followed by loss of consciousness. All four cases had a good prognosis to date with continued physiotherapy. The early diagnosis of the disease helps to guide the optimal management and decide the potential need for physiotherapy.

Autoimmune Retinopathy in a Patient with Stiff-Person Syndrome: A Case Report.

To describe a patient who developed retinal degeneration associated with autoimmune retinopathy (AIR) and who was also found to have anti-glutamic acid decarboxylase (GAD65) autoantibodies and the diagnosis of stiff-person syndrome (SPS). Ophthalmologic workup consisted of clinical examination, multi-modality retinal imaging, and electrophysiologic testing. Further neurologic assessment including relevant serum and cerebrospinal fluid studies was also conducted. We highlight the case of a 45-year-old patient who developed subacute, sequential vision loss, along with bilateral lower extremity weakness. On initial presentation, optical coherence tomography (OCT) of the left eye was notable for diffuse attenuation of the outer retinal bands. Fundus autofluorescence demonstrated a ring of hyper-autofluorescence encircling the fovea of the left eye. At fifteen-month follow-up, the right eye also became similarly affected. He was found to have elevated serum and cerebrospinal anti-GAD65 autoantibodies and was diagnosed with both SPS and AIR. There is a potential association of anti-GAD65 autoantibodies with the development of AIR.

Epidural mass in a 37-year-old male with progressive neurologic symptoms

Abstract Introduction/Objective A 37 yo male with progressive thoracic myelopathy (bilateral lower extremity weakness, hyperreflexia, left foot drop and bowel/bladder incontinence) visited our hospital. MRI of the thoracic spine revealed an intradural-extramedullary mass measuring 3.3 x 2.1 x 1.1 cm with severe compression and leftward and anterior displacement of the spinal cord suggestive of spinal nerve sheath tumor versus less likely meningioma. Surgery was done and an extradural mass was found which was resected via T5/6 laminectomy. Methods/Case Report Pathology received a red black irregular mass measuring 2.9 x 1.1 x 1.0 cm. H&E showed predominantly distended vascular channels lined by a single layer of flat endothelial cells and filled with red blood cells. Immunohistochemical stains CD34, S100, SOX10 and EMA were performed. CD34 was positive in endothelial cells lining the ectatic channels. S100 and SOX10 are negative ruling out spinal nerve sheath tumor. EMA was negative effectively ruling out a meningioma. The diagnosis of cavernous hemangioma was made. Results (if a Case Study enter NA) NA Conclusion Hemangiomas are benign tumors of circumscribed proliferating blood vessels and are most often found superficially in head and neck region. Cavernous hemangiomas have markedly dilated vessels as in our case. Cavernous hemangiomas of the spine are very rare and represent 5-12% of vascular lesions in the spine, with majority being an extensions from a vertebral hemangioma extending into spinal canal. Only 1-2 % are purely extradural spinal locations. Out of 54 cases, 36 were male and18 female, ages 5-78 showed 80% in the thoracic spine and 93% on the dorsal side. Patients typically present with insidious back pain, radiculopathy and myelopathy suspicious for disc herniation. They are most commonly found on the dorsal side of the thoracic spine. Epidural cavernous hemangiomas on MRI would often present as a mass and should be considered in the differential diagnosis along with nerve sheath tumors, meningiomas, abscess, and extradural hematoma.

Abstract 17951: Infective Endocarditis of an Amplatzer PFO Occluder: A Rare and Devastating Complication

Introduction: Patent foramen ovale (PFO) closure is indicated for secondary prevention of ischemic strokes in select patients. PFO closure devices are rarely complicated by infective endocarditis (IE). We present the case of a patient with a closure device infected by Methicillin-sensitive Staphylococcus Aureus (MSSA). Case: A 60-year-old woman with a history of ischemic stroke status post transcatheter PFO closure with a 25 mm Amplatzer occluder eight months prior to presentation was admitted for a two week history of lower back pain, bilateral lower extremity weakness, and urinary incontinence. She denied dental procedures following device implantation. MRI of the spine revealed L3-L4 osteomyelitis with an adjacent epidural abscess which was immediately drained. Blood and intra-operative cultures revealed MSSA. There was no evidence of endocarditis on transthoracic echocardiogram (TTE). Given persistent bacteremia, a transesophageal echocardiogram (TEE) was performed, revealing large, highly mobile vegetations measuring up to 3 cm in length, attached to both the left and right atrial surfaces of the Amplatzer occluder. Urgent surgical removal of the Amplatzer occluder and PFO closure was performed. She received a 4-week course of Cefazolin. Discussion: Despite periprocedural antibiotics and prophylaxis for dental procedures for six months after implantation, IE of PFO occluders can rarely occur days to years after implantation. In patients with PFO occluders and persistent bacteremia or fevers, TEE is the diagnostic modality of choice as TTE are often negative. Management consists of urgent surgical excision and long-term antibiotics.

Abstract 17245: Statin Induced Necrotizing Autoimmune Myositis

Description of Case: A 64 year old female with hypertension, hyperlipidemia, and type 2 diabetes presented to the emergency room for 3-4 months of progressively worsening bilateral lower extremity weakness with associated edema, but without any reports of pain. The patient had significant impairments in her daily function and now required a cane to ambulate. The patient was found to have significantly impaired strength in the bilateral lower extremities with particular involvement of the proximal thigh muscles. The patient was noted to have a markedly elevated creatine phosphokinase (CPK) to 16,000 and a moderate transaminitis. The patient underwent evaluation with an MRI which revealed several areas of bilateral necrotizing myositis. The patient’s autoimmune markers were remarkable for a positive HMG-CoA reductase antibody and a diagnosis of statin induced necrotizing autoimmune myositis was made. The patient was started on pulse dose steroids without much improvement in her weakness and functional ability. The patient was therefore started on intravenous immunoglobulin with a rapid improvement in her symptoms. The patient was discharged on long term glucocorticoid therapy with appropriate prophylactic medications. Discussion: Statin induced necrotizing autoimmune myositis (SINAM) is a very rare form of autoimmune myopathy that is characterized by rapidly progressive proximal muscle weakness along with other potential extramuscular manifestations in the setting of statin use. SINAM should be suspected in any patient on a statin, regardless of duration of treatment, who presents with proximal muscle weakness and a markedly elevated CPK, often > 1000. Treatment, like other forms of autoimmune myopathies, is often centered around immunomodulatory therapies such as high dose glucocorticoids, intravenous immunoglobulin, and long term immunosuppressants. Despite rapid initiation of aggressive treatment, severe forms of the disease have a high mortality as there may be involvement of the respiratory musculature leading to respiratory failure. Given the rarity of this condition, along with the variability in presentation related to the onset of statin therapy, diagnosis and treatment of this entity remains poorly understood.

Spontaneous Epidural Hematoma: A rare neurological complication in a patient following routine coronary angiography

Spinal epidural hematoma is an uncommon but potentially devastating complication associated with various medical interventions. We present a case report of this rare neurological complication following a routine coronary angiography. A 69-year-old male with Aortic Stenosis presented for a scheduled coronary angiography to assess his cardiac status before valve replacement. The procedure was uneventful but approximately 15 minutes after the angiography, he developed sudden-onset of rapidly progressing weakness and numbness in bilateral lower extremities. An urgent neurologic evaluation revealed signs of spinal cord compression, prompting a magnetic resonance imaging (MRI) scan which showed an extensive spinal epidural hematoma spanning multiple levels within the cervicothoracic spine. This case highlights the importance of recognizing this rare but a potential complication following routine coronary angiography even in the absence of signifcant procedural complications. Timely diagnosis and prompt surgical intervention are crucial for achieving favorable outcomes and minimizing morbidity associated with this condition.

SAT494 Impending Thyroid Storm during Pregnancy in a Patient with Gestational Trophoblastic Disease

Abstract Disclosure: R. Kulkarni: None. Z. Sibai: None. Background: Gestational trophoblastic disease (GTD) is a rare complication of pregnancy that arises due to abnormal proliferation of trophoblasts of the placenta. The incidence of GTD in the United States of America is about 110 to 120 per 100,000 pregnancies. Abnormal trophoblastic proliferation leads to higher than expected B-HCG levels compared to normal pregnancy. Since the beta subunit of β-hCG has similar structure to TSH, β-hCG stimulates TSH receptor and causes hyperthyroidism during pregnancy which can be asymptomatic, mild or it can lead to severe cases of thyroid storm. Clinical Case: A 19-year-old African American woman with no PMH, 8 weeks primigravida presented with complaints of nausea, abdominal pain and difficulty walking. Associated symptoms included blurry vision, excessive sweating, tremors and palpitations. Physical examination finding included sinus tachycardia and low BMIof 18.6kg/m2, bilateral hand tremors, weakness of bilateral lower extremities. Initial labs showed quantitative β-hCG level of 283,163(H) m(iU)/mL, TSH <0.010 (L) m(iU)/L , FT4 2.54 (H) ng/dL, negativeTSI and TPO Abs. Burch-Wartofsky Point Scale score was 35 suggestive of impending thyroid storm. Pelvic ultrasound showed intrauterine pregnancy with estimated gestation age of 7 weeks 3 days with findings suggestive of fetal demise. Patient underwent Dilatation and Curettage for suspected molarpregnancy. Final histopathological report of aborted specimen verified the diagnosis of partial molar pregnancy. Based on clinical history, physical finding and lab results, patient was diagnosed with gestational trophoblastic disease induced impending thyroid storm. Endocrinology initiated management with Propranolol 40 mg TID and Methimazole 10 mg daily withimprovement in her clinical symptoms in two days. One week follow up as an outpatient showed resolution of symptoms, normalization of free T4 and down trending β-hCG titers to 166 m(iU)/mL. Propranolol and methimazole were stopped. Conclusion:Gestational trophoblastic disease although a rare clinical entity, it is potentially a life-threatening condition. β-hCG is a glycoprotein, and its beta subunit is structurally similar to TSH, allowing it to bind to the TSH receptor and causing hyperstimulation of the thyroid follicular cells leading to various degrees of hyperthyroidism. GTD associated with significant high level of β-HCG, in rare cases can cause severe consequences like thyroid storm. Therefore, GTD should be considered as a differential diagnosis in patient’s presenting with sever thyrotoxicosis during pregnancy. The mainstay of management is evacuation of the molar pregnancy. In severe cases of hyperthyroidism, Beta blocker and thioamides can be considered for symptoms relief and to speed the recovery. Follow-up with serial quantitative β-hCG measurements is crucial to evaluate for persistent molar tissue. Presentation Date: Saturday, June 17, 2023

SAT624 Neurosarcoidosis Presenting With Panhypopituitarism

Abstract Disclosure: E.M. Niedzialkowska: None. T. Blazin: None. K. Strubchevska: None. M. Kozyk: None. N. Saini: None. Introduction: Neurosarcoidosis is estimated to occur in approximately 5-15% patients with sarcoidosis. Pituitary involvement may be present in up to 5% of those cases and can manifest as hypothalamus, adenohypophysis dysfunction or arginine vasopressin deficiency (AVP-D) in isolated fashion or variedly combined. Clinical case: 32 year old male presented with chief complaint of worsening bilateral lower extremity weakness, urinary incontinence, polyuria and polydipsia. 9 months prior to admission the patient was diagnosed with neurosarcoidosis. At that time brain MRI showed diffuse leptomeningeal enhancement, chest CT showed hilar lymphadenopathy, lumbar puncture with CSF analysis showed protein 227 mg/dl (N<38), ACE 6 U/l (N <2.5), WBC 10 (N<5) with 88% lymphocytes. Hilar lymph node biopsy was consistent with non-caseating granuloma. The patient was diagnosed with neurosarcoidosis and started on prednisone and methotrexate. The patient was referred to our institution for further evaluation. On examination gait ataxia and gynecomastia were noted. Laboratory results showed urine specific gravity of 1.001, LH 0.4 mIU/ml (N 0.6-1.2), FSH 1.1 mIU/ml (N 1-12), ACTH <5 pg/ml (N 6-46), cortisol 2.1 ug/dl (N 2.9-19.4), prolactin 10.5 ng/ml (N 2-18), total testosterone 4 ng/dl (N 240-1080), free testosterone 0.1 ng/dl (N 4.8-19), TSH 0.58 uIU/ml (N 0.4-4.5), free ft3 1.5 pg/ml (N 1.7-3.7), free ft4 0.6 ng/dl (N 0.7-1.5), IGF-1 52 ng/ml (N 71-234), Copeptin proAVP of <2.8 pmol/l( N>13.2), urine osmolality of 72 mOsmol/kg, blood osmolality of 297 mOsmol/kg (N <295), vit D 25-OH 9 ng/ml (N 30-100), vit D 1,25-OH 57.3 pg/ml (N 19.9-79.3). MRI of the spine showed extensive nodular enhancement of the leptomeninges of the cervical, thoracic spine and lumbar cauda equina nerve roots. Brain MRI was positive for numerous small nodular enhancement involving leptomeninges and pituitary stalk. The patient was started on desmopressin with significant improvement of symptoms and increase in urine osmolality to 709 mOsmol/kg. Due to risk of hypercalcemia and normal vit D 1,25-OH vitamin D supplementation was not initiated. For neurosarcoidosis the patient was started on pulse dose methylprednisolone and infliximab infusion. Upon discharge he was transitioned to oral hydrocortisone and started on levothyroxine and testosterone replacement therapy. Conclusion: We present a case of pituitary neurosarcoidosis presenting with AVP-D, secondary hypothyroidism, secondary adrenal insufficiency, hypogonadism and growth hormone deficiency. Panhypopituitarism with complete anterior and posterior pituitary dysfunction is extremely rare and might be found in up to 13% cases of pituitary sarcoidosis. The diagnosis should be considered in patients with sarcoidosis and endocrine dysfunctions. Presentation: Saturday, June 17, 2023

Video-assisted thoracoscopic surgery for Andersson lesion in ankylosing spondylitis: A case report and literature review.

Andersson lesion (AL), a phenomenon initially described by Andersson nearly 80 years ago, has been the subject of extensive research and various treatment modalities. The ongoing debate surrounding the need for anterior surgery in AL cases has spurred numerous proposed approaches. Despite the demonstrated efficacy of anterior surgery in achieving fusion and stabilization, its implementation is associated with prolonged operation time and heightened intraoperative bleeding. A 32-year-old male patient presented at our hospital in February 2019 with a 2-month history of bilateral lower extremity weakness and sensory disturbances. These symptoms were exacerbated by a recent fall. AL conbined with ankylosing spondylitis. A 1-stage posterior fixation and decompression procedure was performed to ensure spinal stability, minimize deformities, and reduce surgical trauma. To achieve these goals, a 2-stage approach was employed, which included video-assisted thoracoscope-guided vertebrectomy, spinal canal decompression, and bone graft fusion. No recurrences of significant pain, limb numbness, or other symptoms were reported, ultimately leading to an improved quality of life for the patient. We utilized video-assisted thoracoscopic surgery technology for anterior bone graft fusion in a patient with AL to minimize the trauma of secondary surgery. However, the 3-year follow-up showed insufficient bony fusion at the fracture site. Nevertheless, the patient maintained spinal stability with posterior internal fixation and no significant kyphosis or symptoms. Thus, standalone posterior fixation may suffice for favorable clinical outcomes in patients with AL.

ID: 218987 Utilization of Neuromodulation for Failed Back Surgery Syndrome in Setting of Recurrent Infections

A 70-year-old female with a past medical history significant for recurrent skin infections, fibromyalgia, Ehlers Danlos Syndrome, and chronic back pain secondary to T12 compression fracture, multilevel spondylosis, and stenosis s/p L2-S1 laminectomy/fusion presented to the ED for significant worsening of her chronic back pain. One-week prior, patient was seen by her primary care physician for a left forearm abscess, underwent incision and drainage in the office, and was discharged on oral antibiotics. One month later, patient began experiencing intractable back pain with associated bilateral lower extremity weakness and subsequently went to the ED. Manual muscle testing of her lower extremities was 3/5 throughout, with no other red flag signs or symptoms. A thoraco-lumbar MRI revealed a T9-T10 epidural collection suggestive of an epidural abscess with cord compression, in addition to a chronic T12 compression fracture. She underwent a T9-T10 laminectomy, with her post-operative course being uneventful. She was discharged to subacute rehab to complete a course of intravenous antibiotics. She was told to follow up for kyphoplasty after her infection clears. One month post-operatively patient continued to have radicular symptoms bilaterally and continued back pain and returned to the ED. An MRI of her thoraco-lumbar spine revealed worsening of T12 compression fracture, T10-T11 vertebral disc herniation with adjacent enhancing fluid collection in the right ventral epidural space suspicious for abscess vs extruded disc material.

Scapular Winging In Patients With Anti-Hydroxy Methyl Glutaryl CoA Reductase (HMGCR) Antibody Positive Immune Mediated Necrotizing Myopathy (IMNM): A Case Series (P9-8.005)

<h3>Objective:</h3> To report examination finding of scapular winging in two patients with Anti-hydroxymethylglutaryl CoA reductase (HMGCR) antibody positive immune mediated necrotizing myopathy (IMNM). <h3>Background:</h3> IMNM is a disabling auto-immune myopathy characterized by severe proximal muscle weakness, markedly elevated creatine kinase (CK) and muscle necrosis. Diagnosis is made via muscle biopsy and antibody testing in a patient with a suggestive history, examination and laboratory abnormalities. <h3>Design/Methods:</h3> Case Series <h3>Results:</h3> Patient 1, a 46 year old male presented for evaluation of muscular dystrophy. He had proximal lower extremity muscle weakness, dysphagia and 20 lbs weight loss over 1 year duration. He was on statin in the past which was discontinued after he was found to have elevated liver enzymes. Examination was notable for mild eye closure weakness, bilateral scapular winging, leg &gt; arm, proximal &gt; distal weakness and abdominal weakness. CK was elevated to 12, 000 u/L. HMCCoA Ab was positive with elevated titer to 435.8. He was started on methotrexate and IVIG Q2 weeks. CK level down trended to 2652 U/L. Patient had significant improvement in his symptoms. Patient 2, a 58 year-old male presented with proximal upper and lower extremity muscle weakness, dysphagia and 10 lbs weight loss of over 4 month duration. He was on atorvastatin for over 3 years which was discontinued 1 month after symptom onset. Examination was notable for proximal muscle weakness in bilateral upper and lower extremities, right scapular winging and neck flexion weakness. CK was elevated to 7500. HMG COA reductase antibody was positive with elevated titer to 169.4. He was started on methotrexate and IVIG Q2 weeks. He also noted improvement in his symptoms. <h3>Conclusions:</h3> Scapular winging can be seen with HMGCR Ab+ IMNM. Such patients can be misdiagnosed having muscular dystrophy. Neurologists should be aware of this clinical phenotype as these patients may respond favorably to immunotherapy. <b>Disclosure:</b> Dr. Katyal has nothing to disclose. Dr. Goyal has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Argenx. Dr. Goyal has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for UCB. An immediate family member of Dr. Goyal has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Goyal has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion.

A Case of Acute Motor Sensory Axonal Neuropathy, a Rare Form of Guillain-Barre Syndrome, Developing After Multiple COVID-19 Infections (P13-8.009)

<h3>Objective:</h3> Guillain-Barre syndrome (GBS), an autoimmune polyneuropathy characterized by ascending weakness and characteristic cerebrospinal fluid (CSF) findings, has been identified in patients previously diagnosed with COVID-19, treated for COVID-19 infection, or vaccinated against the same. GBS is divided into axonal and demyelinating subtypes, and confirmatory testing such as electromyography and nerve conduction studies can help differentiate between subtypes. Here we present a case with recurrent COVID-19 infections, most recently treated with bebtelovimab, who developed acute motor sensory axonal neuropathy (AMSAN), a rare variant of axonal GBS. <h3>Background:</h3> A 33-year-old female presented with progressive ascending weakness and sensory loss that started two weeks after COVID-19 infection. She was fully vaccinated, however, had four episodes of COVID-19 infection within the past year and a half. Two weeks after the most recent infection, which was treated with bebtelovimab, she developed rapidly progressive weakness and sensory impairment in bilateral lower extremities quickly involving upper extremities. She received two rounds of intravenous immunoglobulin (IVIG) three and five weeks after symptoms onset. Her symptoms continued to progress requiring hospitalization. Exam showed severe weakness in all limbs with distal muscle atrophy in upper extremities. Imaging was negative. CSF demostrated albuminocytological dissociation. Nerve conduction studies concluded severe motor and sensory axonal neuropathy with active denervation which led to the suspicion of Acute Motor and Sensory Axonal Neuropathy (AMSAN), a rare variant of GBS. She underwent plasmapheresis with only minimal improvement. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> N/A <h3>Conclusions:</h3> Many cases of GBS have been reported after COVID-19 infection but only a few of them are about AMSAN. This case presents unique features including multiple COVID-19 infections despite prior vaccination, and the development of this rare variant of GBS. It raises the concern of multiple COVID-19 infections having a cumulative impact on peripheral nervous system leading to more severe forms of GBS with poor response to treatment. <b>Disclosure:</b> Dr. Narvaez Caicedo has nothing to disclose. Mr. Rao has nothing to disclose. Dr. Patel has nothing to disclose. Dr. Wu has nothing to disclose.

A Case of Seronegative NMOSD-Related Myelitis Masquerading as VZV-Related Myelitis (P13-5.013)

<h3>Objective:</h3> NA <h3>Background:</h3> Longitudinally extensive transverse myelitis (LETM) is caused by a myriad of etiologies. We present a case of Neuromyelitis Optica Spectrum Disorder (NMOSD)-related myelitis, initially seronegative, masquerading as VZV-related myelitis. <h3>Design/Methods:</h3> A case report. <h3>Results:</h3> A woman in her sixties reported a one-month history of bilateral lower extremity paresthesias and weakness. Days prior, she felt tingling on her back with eruption of a painful rash, and was diagnosed with varicella zoster (VZV). She had transient improvement, before worsening 2 months later with involvement of the arms and signs of myelopathy on exam. MR imaging revealed a long segment T2 hyperintense cord expansion extending from the medulla throughout the thoracic spine with additional hyperintensities in the conus medullaris with patchy enhancement. Work up was significant for lymphocytic-predominant CSF pleocytosis with elevated protein and IgG index. Serum VZV IgG was positive with negative IgM. CSF VZV PCR was negative. Aquaporin-4 antibody was negative. Testing for other causes of myelopathy was unrevealing. She was treated with IV Acyclovir, IV pulse steroids, and subsequently plasma exchange. She improved without further immunomodulation. Three months later, she had another attack and imaging showed new occipital enhancing lesion consistent with demyelination. At this visit, aquaporin-4 antibodies returned positive with a titer of 1:100. She received IV steroids and started rituximab. The patient was diagnosed with LETM from NMOSD. Due to symptom severity, she underwent plasma exchange despite a potential alternative etiology. VZV myelitis and her history of shingles were likely a red herring in the setting of her positive NMO testing. This emphasizes the importance of rechecking antibodies for high clinical suspicion, as the results can drastically influence management and outcome. In the presence of unconfirmed alternative etiologies, neurologists should have low threshold for aggressively treating inflammatory myelitis, using intravenous immunoglobulin or plasma exchange if indicated. <h3>Conclusions:</h3> NA <b>Disclosure:</b> Dr. Alshaer has nothing to disclose. Dr. Cavanagh has nothing to disclose. Dr. Antzoulatos has nothing to disclose.

Post Infectious Triggered Anti-Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD) via CMV in an Immunocompetent Host (P10-5.030)

<h3>Objective:</h3> N/A <h3>Background:</h3> A previously healthy, immunocompetent 38-year-old female presented with initial complaints of subacute, waxing and waning high grade fevers, general malaise, and muscle aches for two weeks. Acutely she developed severe lumbar radiculopathy with significant urinary retention and bilateral lower extremity weakness leading to ED presentation. The patient was subsequently admitted with rapidly progressive lower extremity paraplegia and brisk reflexes over a 24-hour span. MRI imaging was notable for longitudinally extensive transverse myelitis (LETM) over continuous cervical and thoracic segments with significant gray matter involvement and left parahippocampal FLAIR hyperintensities Serum subsequently demonstrated positive anti-CMV IgM/IgG antibodies with a PCR load of 61 copies/mL indicative of a CMV infection with low viremia. LP CSF was CMV PCR negative with mildly elevated opening pressures and trace neurotrophic pleocytosis. Given stagnant clinical changes after five days of IV steroids, plasmapheresis was initiated with marked improvements in functional lower extremity strength. Soon afterwards, initial presenting lab work resulted, demonstrating high serum titers (1:2560) of anti-MOG antibodies. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> N/A <h3>Conclusions:</h3> In immunocompetent hosts, CMV infection itself is a rarely described cause of inflammatory myelopathy with only 14 prescribed cases. With both deep gray matter FLAIR hyperintensities and cervical and thoracic LETM, a concomitant para-infectious CMV and new MOGAD is unlikely in our clinical setting. Additionally, systemic findings typically indicative of severe CMV infections such as retinitis, pericarditis/myocarditis, transaminitis, thrombocytopenia, and leukopenia were absent. Given prior reports of sequence similarities between MOG T-cell epitopes and a major capsid protein of CMV, molecular mimicry between antigen presenting cells has been hypothesized. Here we detail a first described case report of a possible post-infectious MOG antibody transverse myelitis triggered presumptively via CMV infection. Furthermore, presenting in an immunocompetent host, our case report highlights a further wrinkle into the pathophysiology and presence of post infectious triggered autoimmunity for MOGAD. <b>Disclosure:</b> Dr. Chen has nothing to disclose. Dr. Kwon has nothing to disclose.