Weakness Of Upper Extremities Research Articles

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

Background: C5 palsy (C5P) is a recognized potential postoperative complication of cervical spine surgery but has rarely been reported following an open esophageal diverticulectomy. Methods: A 61-year-old underwent an open esophageal diverticulectomy for symptomatic Zencker’s diverticulum. Results: Postoperatively, she presented with right upper extremity weakness and sensory deficits consistent with a C5P that was later confirmed by electromyography. Conclusion: 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.

An unusual finding of an anaplastic meningioma in NF2-related schwannomatosis.

NF2-related schwannomatosis (NF2) is a rare autosomal-dominant genetic disorder characterized by bilateral vestibular schwannomas and multiple meningiomas. This case report presents the extremely rare occurrence of an anaplastic meningioma in a 12-year-old male with previously undiagnosed NF2. The patient presented with a history of abdominal pain and episodic emesis, gait unsteadiness, right upper and lower extremity weakness, and facial weakness. He had sensorineural hearing loss and wore bilateral hearing aids. MR imaging revealed a sizable left frontoparietal, dural-based meningioma with heterogeneous enhancement with mass effect on the brain and midline shift. Multiple additional CNS lesions were noted including a homogenous lesion at the level of T5 indicative of compression of the spinal cord. The patient underwent a frontotemporoparietal craniotomy for the removal of his large dural-based meningioma, utilizing neuronavigation and transdural ultrasonography for precise en bloc resection of the mass. Histopathology revealed an anaplastic meningioma, WHO grade 3, characterized by brisk mitotic activity, small-cell changes, high Ki-67 proliferation rate, and significant loss of P16. We report an anaplastic meningioma associated with an underlying diagnosis of NF2 for which we describe clinical and histopathological features.

Combined Open Surgical and Endovascular Management of a Giant Posterior Cerebral Artery Aneurysm: 2-Dimensional Operative Video.

A 17-year-old female patient presented with worsening intractable headaches which were initially diagnosed as migraine headaches. The patient's headaches were refractory to medical management and continued to worsen in both severity and frequency. Computed tomography angiography and digital subtraction angiography demonstrated a giant posterior cerebral artery aneurysm with contributions from the internal carotid artery through the posterior communicating artery. During the surgical planning period, the patient continued to have worsening headaches, intractable nausea, vomiting, left upper extremity weakness, and associated visual obfuscations. Repeated angiography revealed spontaneous thrombosis of the posterior communicating artery contribution with additional sudden onset hemianopsia on balloon test occlusion of the posterior cerebral artery, prompting urgent surgical intervention. A right subtemporal approach was used for a superficial temporal artery to posterior cerebral artery bypass, followed by coil occlusion of the aneurysm inlet at the posterior cerebral artery on postoperative day 1. The patient consented to the procedure. Postoperative angiography demonstrated successful superficial temporal artery to posterior cerebral artery anastomosis with occluded posterior cerebral artery after coiling. At 1-year follow-up, the patient remained neurologically intact with a patent bypass and regression of the trapped aneurysm. We present this case to demonstrate the successful management of a complex posterior cerebral artery aneurysm with both surgical bypass and endovascular parent artery coil occlusion.1-6.

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.

Diagnosis and Treatment of Neurogenic Thoracic Outlet Syndrome in a Collegiate Ice Hockey Player: A Case Report

A 22-year-old male ice hockey player reported days of worsening bilateral upper-extremity weakness, hand tremors, and difficulty grasping his stick. Subjective information included sleeping in a “curled-up” position due to lack of heat in their residence. Provocation tests and imagining studies were positive for thoracic outlet syndrome. Primary treatment focused on decreasing stress on the anterior chest wall while secondary treatment focused on strengthening the posterior thorax to improve posture. Emphasis is given to core stability and scapular mobility. Removal from sport never occurred, but symptom alleviation occurred after 3 weeks. Thoracic outlet syndrome can be resolved quickly if proper recognition and adequate treatment are utilized. Athletic trainers fill a sociomedical role for their patients and should perform patient-centered care rather than tunnel vision on the pathology.

Navigating the Unexpected: Iatrogenic Aortic Injuries during Transcatheter Aortic Valve Replacement (TAVR).

The introduction of transcatheter aortic valve replacement (TAVR) has undeniably changed the landscape of valvular heart disease management over the last two decades. A reduction in complications through improvements in techniques, experience, and technology has established TAVR as a safe and effective alternative to surgical aortic valve replacement. However, it is important to consider the potential risks associated with TAVR and ways in which life-threatening complications can be identified and managed in a timely fashion. In this article, we review some catastrophic iatrogenic aortic injuries that are described in the literature and present a case of an acute iatrogenic type A aortic dissection that occurred during a transcatheter aortic valve replacement (TAVR). After valve deployment, a routine neurologic examination noted the new onset of a left-sided facial droop and upper extremity weakness. Urgent imaging revealed an extensive type A aortic dissection, and the patient was taken to the operating room for surgical repair. The coordination of our multidisciplinary team allowed for prompt recognition of her neurologic symptoms, urgent imaging, and timely transport to the operating room, all of which contributed to the successful management of this life-threatening procedural complication.

Incidence of Motor Neuron Disease (MND) in Patients Diagnosed with Cervical Spondylotic Myelopathy (CSM)

Objective: Motor neuron disease (MND) can occur in patients with cervical spondylotic myelopathy. The purpose of this study was to determine the prevalence of motor neuron disease in individuals suffering from cervical spondylotic myelopathy (CSM). Materials & Methods: A prospective study was conducted at the Lady Reading Hospital. The patients(n = 70) included were between ages 55 to 75 with established clinical and radiological diagnoses of CSM. Motor neuron disease was diagnosed clinically by Awaji criteria. The patients were evaluated with NCS/EMG (Nerve Conduction Studies/Electromyography) and followed for 6 months. The motor neuron disease was confirmed by modified El Escorial criteria. Results: 40 patients had bilateral upper limb weakness (57%), however, the rest of the 30 patients had unilateral upper extremity weakness (42.8%). Hoffman’s sign was positive in 60% of patients. Lhermitte's sign was also positive in 31% of patients. The majority of patients (55.7%) were found with grade 4 for deltoid followed by 51.4% patients for biceps brachii. MRI showed anterior cord compression at the level of C5 and C6 in 27.14% of patients and C6 C7 in 32.8% of patients. 15.7% of patients were suspected of having both CSM and MND. Conclusion: CSM and MND can occur in the same patients. The incidence of MND is 5.7% in patients with cervical spondylotic myelopathy, hence in CSM patients, workup must be done for MND as it affects the outcome of surgery in these patients.

Reliability and clinical validity of the Kang MRI grading system for cervical central spinal stenosis

BackgroundThe Kang MRI grading system for central cervical spinal stenosis has been previously explored in literature. The aim of the current study was to re-visit the system before adopting it into our institute’s cervical spine MRI reports and to evaluate its reliability through measuring the degree of agreement between radiologists of different levels of experience. The secondary aim was to evaluate its clinical validity by correlating between the radiological grade and the clinical symptoms as well as the subsequent treatment received by the patients.ResultsA total of 82 patients (21 males and 61 females, mean age = 42.8 years) that underwent cervical spine MRI in our institute were included in the study. Two specialized musculoskeletal radiologists and two radiology residents at different levels of experience independently graded the central cervical spinal stenosis at the narrowest level using the Kang grading system. Substantial agreement was found between all the participating radiologists (K = 0.665–0.799, percentage of agreement = 78.04–86.58%). Correlation between radiological grade and the presence of neuropathic symptoms (upper extremity paresthesias and/or numbness), motor affection (upper extremity weakness and/or weak hand grip), and treatment received by the patients was moderate, with R ranging from 0.325–0.440, 0.420–0.506, and 0.452–0.592 respectively. Correlation between grade and the presence of cervical pain was weak, with R = 0.197–0.253. On subdividing the grade 2 patients into grade 2a (demonstrable spinal cord deformity at the anterior or posterior aspects of the cord only) and grade 2b (demonstrable spinal cord deformity at both the anterior and posterior aspects of the cord), a statistically significant difference was found regarding the prevalence of motor affection among the patients in both sub-groups (P < 0.001).ConclusionDespite being reliable and clinically valid, the Kang grading system needs revision of the use of sagittal T2 images only as well as the definition of grade 2. We showed that the current definition of grade 2 to be rather broad, putting a wide array of patients into a single category. Sub-dividing it might increase the accuracy of the grading system.

Passive Range of Motion for Conservative Management of Brachial Plexus Birth Injury: A Survey of Expert Therapists

Brachial plexus birth injury (BPBI) occurs in almost 1/1000 live births potentially resulting in lifelong upper extremity weakness and joint contracture. Early referral and initiation of passive range of motion (PROM) are recommended but there is no standard of care to guide treatment. A descriptive survey study design was used to target occupational and physical therapists who work in brachial plexus clinics to identify current practices for early therapeutic intervention. With 26 respondents, therapists recommend initiating PROM by 4 wks of life performed at every diaper change. Seventy-four percent of therapists report differences in outcomes for children whose caregivers are more adherent, however, there was no significant relationship between frequency of PROM and outcomes. More outcome research is needed to identify an optimal frequency and duration, examine the efficacy of methods for parent education, and identify training methods to facilitate adherence.

Pathophysiology and surgical decision-making in central cord syndrome and degenerative cervical myelopathy: correcting the somatotopic fallacy.

Our understanding of Central Cord Syndrome (CCS), a form of incomplete spinal cord injury characterized by disproportionate upper extremity weakness, is evolving. Recent advances challenge the traditional somatotopic model of corticospinal tract organization within the spinal cord, suggesting that CCS is likely a diffuse injury rather than focal lesion. Diagnostic criteria for CCS lack consensus, and varied definitions impact patient identification and treatment. Evidence has mounted for early surgery for CCS, although significant variability persists in surgical timing preferences among practitioners. A demographic shift toward an aging population has increased the overlap between CCS and Degenerative Cervical Myelopathy (DCM). Understanding this intersection is crucial for comprehensive patient care. Assessment tools, including quantitative measures and objective evaluations, aid in distinguishing CCS from DCM. The treatment landscape for CCS in the context of pre-existing DCM is complex, requiring careful consideration of pre-existing neurologic injury, patient factors, and injury factors. This review synthesizes emerging evidence, outlines current guidelines in diagnosis and management, and emphasizes the need for ongoing research to refine our understanding and treatment strategies for this evolving patient population.

Cervical spinal cord compression from subdural hematoma caused by traumatic nerve root avulsion: illustrative case.

Posttraumatic intradural hematomas of the cervical spine are rare findings that may yield significant neurological deficits if they compress the spinal cord. These compressive hematomas require prompt surgical evacuation. In certain instances, intradural hematomas may form from avulsion of cervical nerve roots. The authors present the case of a 29-year-old male who presented with right upper-extremity weakness in the setting of polytrauma after a motor vehicle accident. He had no cervical fractures but subsequently developed right lower-extremity weakness. Magnetic resonance imaging (MRI) demonstrated a compressive hematoma of the cervical spine that was initially read as an epidural hematoma. However, intraoperatively, it was found to be a subdural hematoma, eccentric to the right, stemming from an avulsion of the right C6 nerve root. Posttraumatic cervical subdural hematomas require rapid surgical evacuation if neurological deficits are present. The source of the hematoma may be an avulsed nerve root, and the associated deficits may be unilateral if the hematoma is eccentric to one side. Surgeons should be prepared for the possibility of an intradural hematoma even in instances in which MRI appears consistent with an epidural hematoma.

Abstract 16396: A Tale of Two Maladies: An Interplay of Mendelian Principles

A 22-year-old male with no medical history presented in acute decompensated heart failure and cardiogenic shock. Physical exam showed a thin body habitus, reduced muscle bulk, bilateral ptosis, limited extraocular motion, and upper extremity weakness. An echocardiogram revealed severe biventricular dilation and systolic dysfunction. Invasive hemodynamics revealed elevated cardiac filling pressures and low cardiac output. Coronary angiography excluded coronary artery disease and anatomical variants. He was initiated on dobutamine and eventually milrinone given persistently low index and elevated lactate. An evaluation for the etiology of his cardiomyopathy focused on nutritional deficiencies (e.g., Wet Beriberi), myocarditis, genetic mutations, infiltrative cardiomyopathy (e.g., Friedrich’s ataxia), and mitochondrial and myopathy-related cardiomyopathy. CMR had globally elevated T1 and normal T2 values ruling out acute myocarditis or infiltrative processes. Serum thiamine, TSH and B12 were within normal range. Muscle biopsy and mitochondrial genetic testing revealed a pathogenic deletion (m.7416_14556) and 70% heteroplasmy. Physical exam, biopsy and mitochondrial genetics were diagnostic of Kearns-Sayre Syndrome (KSS). Notably KSS does not typically cause severe cardiomyopathy suggesting an alternative cause. A genetic cardiomyopathy panel was sent revealing a pathologic mutation in the plakophilin 2 gene which was the suspected cause of his cardiomyopathy. A heart transplant evaluation was initiated as he developed refractory shock requiring mechanical support via an Impella 5.5, placed via the right innominate artery due to his small body and vessel size. He underwent an uncomplicated heart transplantation and is doing well. This case underscores the significance of a broad differential diagnosis, multidisciplinary collaboration, and a detailed physical examination in diagnosing and managing complex cardiac conditions.

Abstract 17085: An Unusual Case of Severe Hypertrophic Cardiomyopathy in a 23-Year-Old Systemic Lupus Erythematosus Patient on Hydroxychloroquine

A 23-year-old female with Systemic lupus erythematosus (SLE), lupus nephritis, antiphospholipid syndrome, venous thromboembolism, stroke, Libman-Sacks endocarditis, and hypertension presented with right upper and lower extremity weakness, right facial droop, and aphasia. Her home medications included hydroxychloroquine, mycophenolate, apixaban, and losartan. ECG showed normal sinus rhythm, left atrial enlargement, ST and T wave abnormalities in V5 and V6. Computed tomography angiography of the brain showed left middle cerebral artery occlusion. Transthoracic echocardiogram (TTE) showed left ventricular ejection fraction of 65%, severe left ventricular hypertrophy, moderate pericardial effusion, and moderately thickened mitral valve (MV) leaflets. Transesophageal echocardiogram showed large vegetations on atrial aspect of both MV leaflets concerning for Libman-Sacks endocarditis. We attempt to investigate the cause of this hypertrophic cardiomyopathy (HCM) in a young patient with SLE. Few reported cases of HCM in patients with SLE were medication induced. Tacrolimus has been observed to induced HCM in a Japanese patient with dermatomyositis. Hydroxychloroquine has been associated with myocardial thickening. Its use can lead to enlarged myocardiocytes with intracytoplasmic vacuoles consisting of concentric lamellar myelin figures in single membrane bound vesicles, curvilinear bodies, glycogen accumulation and fused mitochondria. Hydroxychloroquine use led to reversible inhibition of lysosomal enzyme activity. Cessation of medication use in both cases led to reduction in left ventricle thickness. These patients, like our own, has no family history of aortic valve stenosis, mitral valve prolapse, or HCM. However, this patient’s use of hydroxychloroquine to treat her SLE can be assumed to be a contributing factor to her HCM. This could suggest possible screening with ECG and TTE in patients taking hydroxychloroquine.

Abstract 15696: Simultaneous Presentation of ST-Elevation Myocardial Infarction, Ischemic Stroke, and Pulmonary Embolism in a Patient With Thrombus-in-Transit Across a Patent Foramen Ovale

Case Description: A 70-year-old woman with a history of hypertension, hypothyroidism, and sigmoid colectomy, presented to the ER with altered mental status, slurred speech, and left-sided weakness. She was hemodynamically stable, and exam was notable for dysarthria, left-sided facial droop, and left-sided upper and lower extremity weakness. ECG showed ST-elevation in leads I, aVL, and V1-V3, and ST-depression in II, III, and aVF. CT Head was without intracranial hemorrhage. Emergency coronary angiography showed subtotal thrombotic occlusion of the left main, LAD, and LCX arteries consistent with embolic etiology. Coronary thrombectomy re-established TIMI 3 flow. The patient then underwent CTA of the head and neck, which visualized both right anterior M2 occlusion and large pulmonary emboli in the right and left main PAs. She underwent emergency mechanical thrombectomy of the right MCA. With induction of anesthesia she suffered PEA arrest, for which she received 5 minutes of CPR and epinephrine and ROSC was obtained. She then underwent successful thrombectomy of the right anterior M2. Patient then underwent bilateral pulmonary thrombectomy and IVC filter placement. Over the ensuing 24 hours she weaned from pressors, was extubated, and was alert with only mild left hemiparesis. Bilateral lower extremity venous dopplers were negative for DVT, and an echocardiogram showed a serpiginous echodensity in both atria and across the intra-atrial septum, consistent with a large thrombus-in-transit through a PFO. CT Surgery was consulted, and patient underwent successful bi-atrial thrombectomy with PFO closure. Discussion: Simultaneous thromboembolic MI, stroke and PE is an exceedingly rare and catastrophic clinical presentation. Initial management should involve a promptly-assembled multidisciplinary team to determine if the patient is a candidate for procedural or operative intervention, and, if so, to determine the order in which they should be completed. Subsequently, a workup should be initiated to determine an underlying cause of the presentation, including appropriate thrombophilia workup and imaging. If feasible, surgical thrombectomy and PFO closure may be indicated to prevent further embolic phenomena.

Abstract 15274: STEMI as Initial Manifestation of Essential Thrombocythemia

Background: Essential thrombocythemia (ET) is a myeloproliferative neoplasm of myeloid cell expansion, mainly excessive platelet production, associated with thrombotic complications. Myocardial infarction (MI) in the setting of ET can result from thrombosis in the absence of coronary atherosclerosis. Here we present a case of ST elevation MI in the setting of previously undiagnosed ET. Case Report: A 59-year-old male with hypertension presented to the ED with left upper extremity weakness which started upon waking. On exam, he was in acute distress and diaphoretic. EKG revealed sinus rhythm with ST elevation in II, III, aVF, V5, and V6 suggesting inferolateral STEMI (Figure 1A). Labs were notable for Hgb 8.5g/dL, white blood cell count 33k/μL, and platelets 1.9million/μL. Decision Making: He was loaded with aspirin and clopidogrel (dual antiplatelet therapy-DAPT) and taken for coronary angiogram which revealed 80-90% thrombotic occlusion of the left circumflex artery (Figure 1b). Thrombectomy was performed and a drug-eluting stent was placed. A bone marrow biopsy showed JAK2 positive ET with myelofibrosis. He was immediately started on hydroxyurea. Platelets remained elevated, thus pegylated IFN alpha-2a was started which normalized platelet count. He remained on DAPT through discharge with no recurrent ischemic events or in-stent thrombosis during the 45-day hospitalization post stent placement. Conclusion: Patients with ET are at high risk for arterial thrombosis regardless of the presence of traditional atherosclerotic risk factors. High-risk patients, which includes those age over 60 with JAK2 mutation or history of arterial thrombosis, should be treated prophylactically with aspirin and hydroxyurea for cytoreduction. MI may be the first clinical manifestation of ET, even in patients without atherosclerotic risk factors. Initial management in patients with ET presenting with STEMI includes PCI with DAPT and early cytoreductive therapy.

Abstract 110: Autoangiogenesis Secondary to Chronic Cocaine Use Mimicking Moya‐Moya

Introduction We present the case of a young male presenting with syncope who was found to have incidental hypodensities on computed tomography (CT) of his head, with magnetic resonance imaging (MRI) concerning for Moya‐Moya related occlusion of the right internal carotid artery, but with digital subtraction angiography (DSA) showing extensive autoangiogenesis and collateralization from all other intracranial vascular territories, without Moya‐Moya related changes. This presumably developed as reactive changes to severe chronic cocaine use. Methods We present a case from a tertiary care center in the borough of Manhattan, New York City, USA. All information is deidentified and exempt from IRB approval. Results A 36‐year‐old man with history significant for asthma, active smoker status, and active cocaine use, presented for loss of consciousness. The patient reported 3 days of upper respiratory symptoms and decreased oral intake with subsequent loss of consciousness immediately preceded by lightheadedness and nausea, and without any post‐event confusion, lacerations, or incontinence. Neurologic history is significant for right upper extremity weakness from 9/2022‐12/2022 that self‐resolved and was believed to be a peripheral nerve pathology. Admission exam without focal deficits. Admission CT of his head showed multifocal confluent hypodensities in the subcortical right frontal lobe. CT Angiogram of head and neck showed severe narrowing at right Internal Carotid Artery (ICA) terminus and right M1 segment with lenticulostriate arterial collateralization, severe bilateral A1 stenosis with distal Anterior Cerebral Artery (ACA) flow suggestive of chronic collateralization, and significant right Posterior Cerebral Artery (PCA) collateralization. Given concern for Moya‐Moya disease, MRI of the brain was obtained and despite no acute infarct, MRI was significant for multifocal white matter T2/FLAIR hyperintensities with the largest in the right frontal lobe. The patient subsequently underwent digital subtraction angiography (DSA) to assess collaterals and stratify for possible need for future encephaloduroarteriosynangiosis (EDAS). DSA showed significant auto‐angiogenesis between numerous vascular territories, including recruitment of right Posterior Cerebral Artery and External Carotid Artery branches to perfuse the territory of the severe right ICA terminus steno‐occlusive disease. With minimal anterograde flow into the right Anterior Choroidal Artery and none to the right Middle Cerebral Artery (MCA), the characteristic Moya‐Moya appearance was not appreciated on DSA given minimal anigogenesis around the right ICA occlusion site. Rather, distal perfusion of the R ICA territory was dependent on collaterals from the right PCA, right ACA via the Anterior Communicating Artery, angiogenesis between the right Anterior Falcine Artery and R ACA, and angiogenesis between the right MCA and ACA/MCA cortical branches. These proliferative changes involved all cranial vascular territories, including those without steno‐occlusive disease. Conclusion Acute cocaine use is associated with increased risk of acute ischemic stroke[1], due in part to its sympathomimetic effects causing vasospasm and to its arrhythmic properties[2]. Neuroadaptation after chronic cocaine use is less well understood though rat models suggest that as cerebral ischemia develops from chronic cocaine use, the HIF‐VEGF pathway is activated to promote angiogenesis to restore local blood flow[3]. We present this case as evidence of a similar angiogenic physiologic response in humans to minimize the microvascular ischemic injury from chronic cocaine use.