United States

Background: With the worldwide resurgence of secondary syphilis cases within the last decade, including in the United States, atypical presentations are being observed. Syphilis is called the &quot;great imitator” due to its variable presentation affecting multiple organ systems. Thusly, remaining an elusive condition to diagnose especially in the United States, as it is a relatively uncommon disease despite its incidence dramatically increasing since 2000. Syphilis is caused by the bacterium Treponema Pallidum and is clinically subdivided into three stages: primary (a painless chancre at an inoculation site), secondary (rash, lymphadenopathy, and constitutional symptoms), and tertiary (cardiovascular or central nervous system involvement). Although extremely rare, cases have been reported of syphilis affecting the kidney causing proteinuria and various kidney injuries.

Case Presentation: We present the case of a 37-year-old male who presented with flulike illness with fever, chills, arthralgias and was noted to have acute renal insufficiency. Due to the uncertain etiology of his acute renal insufficiency, the patient underwent renal biopsy, which revealed membranous glomerulonephritis. He was ANA positive and initially started on prednisone and mycophenolate mofetil for suspected lupus nephritis. Due to an accelerating rash presenting on the patient’s palms and soles, RPR was obtained. He received a single dose of intramuscular (IM) penicillin with resolution of rash and normalization of his renal function. Social history was remarkable for multiple sexual partners in the past year and negative HIV and hepatitis panels.

Discussion: Although kidney involvement is well described in literature in congenital syphilis, there is still sparse literature regarding histopathologic changes in adult patients. Current literature suggests that there is much variability of renal manifestations of syphilis including rapidly progressive glomerulonephritis (RPGN) and syphilis-associated crescentic glomerulonephritis. Membranous nephropathy is the most described lesion amongst these rare presentations. Recovery usually follows treatment with IM penicillin and due to its vague nature, often it is initially erroneously suggestive of connective tissue disease versus infectious etiology. We recommend that syphilis be considered in the differential diagnosis of sexually active patients with proteinuria or suspected glomerulonephritis.

AMA Research Challenge 2022

Secondary Syphilis Masquerading Lupus Nephritis Heralding Diagnosis of Membranous Glomerulonephritis

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Charles Lopresto, DO

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Welcome to the largest national, multi-specialty research event featuring research from 750 medical students, residents and fellows, and international medical graduates.


Background: With the worldwide resurgence of secondary syphilis cases within the last decade, including in the United States, atypical presentations are being observed. Syphilis is called the "great imitator” due to its variable presentation affecting multiple organ systems. Thusly, remaining an elusive condition to diagnose especially in the United States, as it is a relatively uncommon disease despite its incidence dramatically increasing since 2000. Syphilis is caused by the bacterium Treponema Pallidum and is clinically subdivided into three stages: primary (a painless chancre at an inoculation site), secondary (rash, lymphadenopathy, and constitutional symptoms), and tertiary (cardiovascular or central nervous system involvement). Although extremely rare, cases have been reported of syphilis affecting the kidney causing proteinuria and various kidney injuries.

Case Presentation: We present the case of a 37-year-old male who presented with flulike illness with fever, chills, arthralgias and was noted to have acute renal insufficiency. Due to the uncertain etiology of his acute renal insufficiency, the patient underwent renal biopsy, which revealed membranous glomerulonephritis. He was ANA positive and initially started on prednisone and mycophenolate mofetil for suspected lupus nephritis. Due to an accelerating rash presenting on the patient’s palms and soles, RPR was obtained. He received a single dose of intramuscular (IM) penicillin with resolution of rash and normalization of his renal function. Social history was remarkable for multiple sexual partners in the past year and negative HIV and hepatitis panels.

Discussion: Although kidney involvement is well described in literature in congenital syphilis, there is still sparse literature regarding histopathologic changes in adult patients. Current literature suggests that there is much variability of renal manifestations of syphilis including rapidly progressive glomerulonephritis (RPGN) and syphilis-associated crescentic glomerulonephritis. Membranous nephropathy is the most described lesion amongst these rare presentations. Recovery usually follows treatment with IM penicillin and due to its vague nature, often it is initially erroneously suggestive of connective tissue disease versus infectious etiology. We recommend that syphilis be considered in the differential diagnosis of sexually active patients with proteinuria or suspected glomerulonephritis.

poster

Background: Chronic Abdominal Wall Pain is a poorly recognized, debilitating, clinical problem that is often difficult to treat. Few clinical trials are available in regards to management of this condition. Injection of local anesthesia in combination with steroids provides relief for some patients, and when pain persists despite these treatments, neurectomy is an option. We report the first case of a transnasal sphenopalatine ganglion block that repeatedly provided greater than 80% pain relief for a patient suffering from intractable Chronic Abdominal Wall Pain.

Case Presentation: A 28-year-old female with Crohn's disease and asthma presented with intractable chronic abdominal wall pain after several abdominal surgeries. The patient reported 1.5 years of “very severe,” constant, left upper quadrant abdominal pain that was exacerbated by all physical activity and was significantly affecting her quality of life. Imaging did not demonstrate any pathologic abnormality. Physical/occupational therapy, muscle relaxants, selective serotonin reuptake inhibitors, transcutaneous electrical nerve stimulation, trigger point injections, transversus abdominis plane block, and surgery provided minimal relief. Considering that the sphenopalatine ganglion block (SPGB) has previously been shown to relieve pain of various etiologies, we offered it to this patient. Long cotton-tips were soaked in viscous Lidocaine 2% and were advanced, atraumatically, into each nasal sinus until gentle resistance was met, and left in place for a total of 15 minutes, while the patient was supine. This process was repeated two more times, for a total of 45 minutes. After the first SPGB session, the patient, for the first time, had 80% pain relief that lasted for 3 weeks, with significant improvement in her daily function. Since then, the patient has been receiving this block monthly and each time experiencing the same effect, with sometimes even more than 80% pain relief. The patient reports that this is the only treatment that has so far provided her significant benefit.

Discussion: Chronic Abdominal Wall Pain is often difficult to manage with physical/occupational therapy, analgesic medications, and invasive procedures.
This case report demonstrates that the lidocaine sphenopalatine ganglion block could be a simple, safe, non-expensive, and non-invasive treatment option for those affected by chronic abdominal wall pain.



Chronic Abdominal Wall Pain Relieved by Sphenopalatine Ganglion Block

Rare vallecular cysts can have severe consequences in an anesthetized patient. The presence of a vallecular cyst makes obtaining and maintaining an airway difficult. We report a case of an unexpected difficult airway of a 9-year-old boy who presents for an MRI of the brain with and without contrast, to rule out a structural cause for his nocturnal headaches. After sedation, the patient’s airway completely obstructed and was later found to be due to the presence of a vallecular cyst. This case report will detail the management of an unexpected difficult airway in a remote location. 


Unexpected Difficult Airway in a Remote Location: Vallecular Cyst

Background: Myocarditis has been a rare, but well-documented side effect of the mRNA-based vaccines against SARS-CoV-2 as well as a complication of viral infections including SARS-CoV-2. However, myopericarditis as a complication of monoclonal antibody infusion or as complication of allergic reaction to antibody infusions might be and underreported.

Case presentation: In this case, we report a 30-year-old man with a previous diagnosis of COVID infection 1 week prior to presentation, unvaccinated for SARS-CoV-2 who was referred from a monoclonal infusion center where he received casirivimab and imdevimab and 15 minutes after infusion began to complain of chills, chest pain, shortness of breath and was hypotensive. While in the infusion center he received epinephrine, Benadryl and was directed to the emergency room. While in the ER, patient was febrile, tachycardic, and hypotensive. Initial troponin was 1.91 which peaked at 11.73 with the CK-MB that peaked at 21.2. EKG had no ischemic changes. First two-dimensional echocardiogram showed an ejection fraction of about 45%, with a left ventricular dysfunction and trivial posterior pericardial effusion. Diagnosed as myopericarditis. On admission he was started on full dose lovenox, aspirin, fluid resuscitation, steroids, remdesevir and bipap due to his respiratory compromise. 3 Days later with clinical improvement, repeat 2-d echocardiogram with EF of 65%, with normal ventricular contractility and no pericardial effusion. Patient was discharged home with close cardiology follow up.

Conclusions: Though this could be simple case of viral myopericarditis with troponinemia secondary to demand-ischemia, the differential should be broadened to complication of monoclonal antibody, given the sudden symptom onset after infusion completion and/or a possible Kounis syndrome. Though there have not been any reported cases of casirivimab and imdevimab causing myopericarditis, adverse cardiac events after monoclonal therapy have been reported mainly in cancer patients receiving monoclonal infusions.


A Case of Possible Casirivimab/Imdevimab Induced Myopericarditis in a COVID Positive Young Male

A Case of Romano-Ward Congenital Long QT Syndrome in Mother and Daughter

Cardiac malignancies are exceedingly rare, with metastatic disease more common than primary tumors. Regardless of origin, cardiac tumors have insidious clinical presentations, from mild chest pain to florid heart failure depending on the size and location of the tumor. We present an unusual case of cardiac disease. 
A 56-year-old woman with a history of stage I uterine leiomyosarcoma status post chemotherapy and hysterectomy presented with epigastric discomfort eight years after cancer remission. CT chest demonstrated a heterogeneously enhancing mass in the right atrium accompanied by a large pericardial effusion and regional lymphadenopathy. She underwent pericardiocentesis due to tamponade physiology, but no malignant cells were seen on cytologic analysis. Subsequent CTA of the heart and cardiac MRI confirmed an 8.2 cm x 6.3 cm hypervascular tumor in the anterior pericardial space that straddled the right heart, compressing the atrium and ventricle, and encasing the right coronary artery. Pericardial mass core biopsy was consistent with leiomyosarcoma. One month after diagnosis, she was admitted to the hospital to start the first cycle of neoadjuvant chemotherapy with mesna, adriamycin, ifosfamide, and dacarbazine. During the first cycle, she developed episodes of non-sustained ventricular tachycardia concerning for an arrhythmogenic effect of the mass versus the cardiotoxic effects of chemotherapy. She was started on a low dose beta-blocker. Following completion of three cycles of chemotherapy, she is planned for tumor restaging and potential surgery. 
Although rare, cardiac tumors should be considered in patients with a history of malignancy, and given the anatomic location of the heart, cardiac pathology should remain on the differential for epigastric pain. Having a high index of clinical suspicion can lead to more rapid identification, especially with advancements in imaging techniques such as cardiac MRI and CTA. Leiomyosarcomas are aggressive tumors, and symptoms may only arise once the tumor is at an advanced stage, as in this patient. Prompt initiation of chemotherapy and surgical resection can improve overall prognosis. Ultimately, this patient requires close coordination between the cardiology, oncology, and cardiothoracic teams to provide the most optimal care.

A Rare Cardiac Tumor Presenting as Epigastric Pain

Background: Takotsubo cardiomyopathy (TCM), also known as an apical ballooning syndrome or stress cardiomyopathy, is a rare clinical syndrome defined as a profound but reversible left ventricular dysfunction in the absence of coronary artery disease. TCM is commonly associated with physical and emotional stress, but the pathophysiology is unknown. Here we present a 42-year-old female who develops TCM due to prolonged emotional stress causing the progression of motor neuron dysfunction. 
Case Presentation: A 42-year-old female presented to the ER with loss of consciousness after developing several bouts of cough followed by acute onset of breathlessness triggered by a prolonged stressful event for several months. She developed cardiac arrest, for which CPR was successfully performed. The family denied that she had chest pain, palpitations, and lower extremity edema. Echocardiography showed apical ballooning of the left ventricle (LV), distal
septum, and apex appeared hypokinetic, with a reduced EF of 40%. Repeated screening echo showed improvement in EF alone. Prior to this event, the patient had a history of severe muscle weakness, fatigue, and bulbar symptoms for 8 months, breathing difficulty with weakness in both upper and lower limbs, loss of appetite, and weight loss of about 9 kgs in 3 months. She noticed progressive thinning of leg and arm muscles. There were no visual symptoms, sensory symptoms of bowel and bladder, or systematic symptoms. Her nerve conduction velocity was normal. Antibody testing for AchR, as well as MUSK antibodies, was negative. The patient died approximately 3 months after the first cardiac arrest.
Discussion: 
Prolonged stress, myasthenic crisis, and hyperthyroidism can lead to TCM. Here
we report a patient with clinical signs and symptoms of bulbar onset ALS who had prolonged emotional and physical stress that led to TCM, thereby progressing neuromuscular weakness. We advocate screening, careful cardiac monitoring, and management of Motor neuron disease (MND) patients, especially ALS, regularly and, more importantly, during acute emotional or physical stress, as there is a potential risk of developing TCM and rapid progression of MND/ALS symptoms leading to fatal respiratory failure.

A rare case of Takotsubo cardiomyopathy associated with amyotrophic lateral sclerosis

Abstract Title:
A Triple Threat: Triple-Valve Endocarditis Case Report and Literature Review
Background
Triple Valve Endocarditis (TVE) is uncommon and infrequently described in the current medical literature. Large-scale studies studying its incidence, characteristics, and management are difficult to conduct. Here we describe a case of TVE in an intravenous (IV) drug user and review the literature of TVE identification, treatment, and prognosis. 

Case Presentation
A 37-year-old man was brought to our hospital due to behavior changes since being tossed from an unknown person’s car onto a driveway four days earlier. He had completed treatment for methicillin-resistant Staphylococcus aureus infective endocarditis (IE) two months earlier but continued to use IV heroin. He was intubated for airway protection and started on broad-spectrum antibiotics. A transthoracic echocardiogram identified vegetations on the aortic, mitral, and tricuspid valves. Magnetic resonance imaging (MRI) of the lumbar spine revealed evidence of osteomyelitis and paraspinal abscesses at the L5 - S1 levels. MRI brain showed multiple areas of acute infarction. Blood cultures obtained on arrival were positive for methicillin-sensitive Staphylococcus aureus and the patient was changed to IV Nafcillin. His hospital course was complicated by the development of acute renal failure. Cardiac surgery deemed the patient a poor surgical candidate. Due to the extensive metastatic disease and his lack of response off sedation, his family had the patient terminally extubated on day three of hospitalization. 

Discussion

Streptococcus and Staphylococcus species are the most frequent causes of TVE. Case series of patients with TVE are lacking due to its uncommon nature. Multi-valve endocarditis (two and three valve involvement) represented 18% of cases in one study of 14 patients, 31% in a series with 383 patients, and 12% in a study of 48 patients. The mitral, tricuspid, and aortic valves were most commonly involved in TVE. A transesophageal echocardiogram is the imaging modality of choice as it increases the sensitivity of identifying vegetations to about 90%. Antibiotic treatment is similar to single-valve IE but surgical intervention remains challenging due to a high incidence of postoperative complications. However, a multidisciplinary team-based approach can decrease the expected one-year mortality rate by nearly fifty percent. Because TVE is associated with higher risks of complications and poorer outcomes, prompt identification and treatment are especially important.


A Triple Threat: Triple-Valve Endocarditis Case Report and Literature Review

Amiodarone Induced Bone Marrow Granulomas: A literature Review of Case reports

Background
Amiodarone is classified as a class III antiarrhythmic drug primarily indicated in the treatment of ventricular arrhythmias. The agent has a wide array of adverse effects that may affect multiple organ systems, including the pulmonary, central nervous system, cardiovascular, thyroid, liver, eyes, and skin. A rare and uncommon occurrence from amiodarone use is the development of bone marrow granulomas. This review article summarizes the mechanism of action, clinical uses of amiodarone therapy and reviews the unique presentation of amiodarone-associated bone marrow granulomas using ten case reports. It also includes a discussion section summarizing literature that can further aid healthcare professionals and patients in the diagnosis, management, and education of this adverse effect.

Methods
An electronic database search of scientific platforms such as PubMed, Pubmed Central (PMC), and Google Scholar was conducted. Respective data was collected and reviewed to conduct this
manuscript. 10 previously published case reports regarding amiodarone induced bone marrow granulomas were analyzed and reported. Articles were selected based on the keywords mentioned above.

Results
As per the reported cases of amiodarone- associated granulomas, subjects taking a dosage of 100 mg – 200 mg for a duration of 6 to 18 months was enough to ensure bone marrow granulomas. Repeated bone marrow biopsy 3 to 6 months after discontinuation of the offending agent demonstrated an improvement. All ten case reports revealed noncaseating granulomas confirmed by bone marrow biopsy. Subjects that did develop cases of anemia, EPO showed great improvements to raise Hb levels. 4 of the 10 cases continued treatment despite the confirmed bone marrow granulomas due to the intractable nature of their cardiac condition. In such cases, routine monitoring is recommended, and repeated bone marrow biopsies may be required.

Conclusion
Bone marrow granulomas are seen commonly in infectious and malignant etiologies. A thorough
history and workup can reveal uncommon causes such as medication-induced and prevent misdiagnosis. Bone marrow granulomas in subjects previously receiving treatment with amiodarone should arouse clinical suspicion for possible drug-induced etiology.


Amiodarone Induced Bone Marrow Granulomas: A Literature Review of Case Reports

Background: 
Catheter ablation-induced visual symptoms are reported to be rare. Catheters can cause an iatrogenic atrial septal defect (IASD) that can remain open for up to nine months.  A similar dynamic  change in the patent foramen ovale (PFO) has also been hypothesized to be caused by IASD. This can result in paradoxical emboli containing particulate material from the equipment and/or microemboli, both of which result in cortical spreading without infarction in the brain, resulting in visual complications. 

Case Presentation :
A 72-year-old Caucasian woman with paroxysmal atrial fibrillation (AF) but no migraines presented to our hospital with acute visual changes.  She had an uneventful AF catheter ablation four days prior to her presentation.  
Her medications were flecainide 50 mg twice/day and apixaban 5 mg twice/day.  
She experienced nausea, constipation, and headaches after catheter ablation, which subsided after three days. On the fourth day after the procedure, she noticed blurriness, a right visual field defect, and blind spots in both eyes. These visual changes were episodic and lasted for 15-20 minutes without any known precipitating or relieving factors. She denied fever, chest pain, palpitations, confusion, dizziness, loss of consciousness, and motor or sensory deficits. Her general physical exam, including a thorough neurological examination, was normal. Vitals were stable. The patient's initial 12-lead ECG and telemetry strips showed normal sinus rhythm. Normal blood and metabolic lab results. MRI and CT brain angiography showed no abnormalities. Her symptoms resolved after 24 hours while she remained under observation.  
She was discharged home and has reported no recurrence of symptoms to her cardiologist. 

Discussion:
With the increased use of catheter ablation procedures for symptomatic arrhythmias, rare but significant post-procedural symptoms such as visual disturbances have been reported. Monitoring for IASD, which can potentially cause complications after catheter ablation, with transthoracic echo (TTE) or transesophageal echo (TEE), can be helpful in determining the etiology; more research is needed in this area to avoid unnecessary, lengthy, and costly investigations that burden patients and institutions. We hypothesize that this patient's visual symptoms resulted from IASD-related complications as a result of her catheter ablation.

Approach to Visual Manifestations Following Catheter Ablation

Introduction:

Epsilon waves on electrocardiogram (ECG) are low voltage potentials following the QRS complex representing delayed right ventricular conduction. These are characteristic of arrhythmogenic right ventricular cardiomyopathy (ARVC). This condition has been associated with sudden cardiac death.

Case Presentation:

We present a 59-year-old female with a family history of sudden cardiac death who was admitted to the hospital for syncope and found to have a subdural hematoma managed with Burr hole drainage. On day 2, the patient had a sudden episode of sustained monomorphic ventricular tachycardia and loss of consciousness requiring cardioversion to sinus rhythm and initiated on amiodarone. Subsequent ECG showed sinus rhythm with incomplete right bundle branch block, T wave inversions and epsilon waves in precordial leads. Transthoracic echocardiogram showed severely dilated right ventricle and reduced ejection right ventricular ejection fraction (RVEF) of 45%. Cardiac MRI showed worsening RVEF of 27% and regional right-ventricular hypokinesia. Additional genetic testing for ARVC was negative. As per Task Force 2010 criteria the patient met at least two major and two minor criteria which confirmed a “definite” diagnosis of ARVC. The patient was continued on mexiletine and metoprolol and advised to avoid endurance exercise. She received a secondary prevention cardioverter-defibrillator and was referred to advanced heart failure specialist for cardiac transplant evaluation.

Discussion

ARVC involves mutations in the genes coding desmosomes thus causing replacement of the myocardium with fibrofatty tissue. This results in delayed intraventricular conduction allowing for fatal reentrant ventricular arrhythmias. We emphasize the importance of recognizing epsilon waves on ECG which are highly specific to this disease process. The incidence of epsilon waves in ARVC accounts for 30%. Moreover, recognition of LV involvement in patients with ARVC can help identify patients at higher risk of mortality that may go on to benefit with heart transplant evaluation.

Next from AMA Research Challenge 2022

Chronic Abdominal Wall Pain Relieved by Sphenopalatine Ganglion Block

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