An Exceptional Case of Diplopia and Ptosis: Extramedullary Plasmacytoma of the Clivus With Multiple Myeloma.

Intracranial plasmacytoma is an exceedingly rare presentation of plasma cell neoplasms. Usually presenting late in the course of the disease, progression from the presentation can be abrupt. Hence, a low threshold to biopsy the lesion should be maintained during diagnostic evaluation. Multiple myeloma workup should also be sent and treated concomitantly along with local treatment. Here, we present a case of extramedullary plasmacytoma of the clivus leading to progressive visual deficits with undiagnosed multiple myeloma requiring pulse steroids, intracranial irradiation, and high-dose chemotherapy with improvement in symptoms.

An Unusual Case of Isolated Acute Aphasia in Multiple Sclerosis.

Acute flare of multiple sclerosis usually presents with sensorimotor deficits in limbs or one side of the face, optic neuritis, internuclear ophthalmoplegia, and/or cerebellar signs and symptoms. Isolated aphasia is observed only in a handful of cases. Herein, we present a case of a patient who presented with isolated transcortical motor aphasia. Initial thought was that the patient was having a cerebrovascular accident as he had a history of uncontrolled hypertension. It was only later found on magnetic resonance imaging (MRI) of the brain that the patient had demyelinating lesions compatible with his new symptoms. He exhibited an excellent response to intravenous methylprednisolone therapy and was discharged with outpatient evaluation for immunotherapy.

Catastrophic Antiphospholipid Syndrome Associated With Systemic Lupus Erythematosus Flare With Multiorgan Failure: A Chronicle of Cardiogenic Shock, Renal Failure, Vasculitis-Like Lesions, and Disseminated Intravascular Coagulation.

Catastrophic antiphospholipid syndrome (CAPS) is a rare disorder characterized by a storm of thrombosis leading to rapidly progressive multiple organ damage and thus needs to be picked earlier in the course of the disease. A higher index of suspicion is therefore mandated to initiate triple therapy to save end-organ damage. Antiphospholipid syndrome (APS) is a known association of systemic lupus erythematosus (SLE) and, when present with lupus, has the worst outcome and mainly afflicts younger cohorts. We report the case of a 33-year-old male with an extensive medical history, most notable of lupus with positive antiphospholipid antibodies complicated by nephropathy, and myocarditis presents with cardiogenic shock and progressive renal failure. The course was complicated by diffuse intra-abdominal thrombosis involving bowel, spleen, and kidneys; skin discoloration; and later disseminated intravascular coagulation (DIC). Triple therapy was initiated, which resolved the crisis, although the patient succumbed to late sequelae of infection and died of megacolon perforation. Here, we discuss the association of CAPS with SLE and a plethora of presentations, which involved but were not limited to cardiogenic shock, worsening nephropathy, mimicked vasculitis, digital cyanosis, and DIC.

Coronavirus Disease 2019 Pneumonia and Acute Pancreatitis in a Young Girl.

Severe acute respiratory syndrome coronavirus 2 pneumonia and acute pancreatitis are rarely reported in patients with coronavirus disease 2019 (COVID-19). We present the case of a 13-year-old girl who presented with nausea, vomiting, and abdominal pain for the last two days, along with a cough for the last week. She had a fever and tachycardia. Lung examination revealed reduced breath sounds, and abdominal examination showed tenderness in the epigastrium. COVID-19 polymerase chain reaction was positive, and her serum chemistry revealed elevated serum amylase and lipase. Abdominal computed tomography revealed diffuse inflammation of the pancreas with peripancreatic edema, and chest X-ray demonstrated diffuse infiltrates and pneumonic patches in both lungs. Her initial management included bowel rest, intravenous fluids, intravenous remdesivir, and azithromycin with supplemental oxygen based on the provisional diagnosis of COVID-19 pneumonia and acute pancreatitis. Her abdominal symptoms started improving, and dexamethasone was added to her regimen due to her worsened respiratory condition. She was symptom-free on day seven except for a mild cough. She was discharged on day eight with follow-up.

Glioblastome Multiforme: A Bibliometric Analysis.

Bibliometric analyses are widely used to gauge the scholarly impact of any scientific publication. We conducted a bibliometric analysis of the 100 most influential articles on glioblastoma multiforme (GBM). We searched Scopus using the keywords "Glioblastoma multiforme," "GBM," Glioblastoma," and "Grade IV glioma." A list of the top 100 articles was prepared. The articles were sorted according to the number of citations. A detailed analysis was carried out to identify the characteristics of the most influential studies. The 100 most cited articles in the field were published over 38 years between 1978 and 2018, with the maximum number of articles published in the 10-year period from 2001 to 2010. The total number of citations for 100 articles was 148,594 and 4.8% were self-citations. Citations ranged from 9624 to 617, with a median of 935 (interquartile range, 906). The top cited articles originated from 22 countries, with the greatest contributions from the United States. Nature made the greatest contribution to the research on GBM, with a total of 14 articles, and Cancer Cell and New England Journal of Medicine were the second biggest contributors. Fifty-seven studies focused on the pathogenesis of GBM. There were 12 authors who had ≥5 articles in the top 100 citation list. Only 31% of the articles were funded by public and private sector organizations. Our analysis highlights the characteristics of the most influential articles on GBM and provides valuable insight into the research that has been conducted in this field.

Solitary Splenic Tuberculosis in an Immunocompetent Child: A Case Report.

Tuberculosis (TB) is a lethal infectious disease that still remains a major threat in developing countries. Solitary splenic tuberculosis is a rare entity and there have been very few cases of it reported in literature. It is mostly encountered in patients who have an immunocompromised state. It may occur with a myriad of non-specific presentations, making it complex to diagnose. Here, we report a case of an eight-year-old female, immunocompetent, who had complaints of fever, abdominal pain and chronic diarrhea. Laboratory data failed to provide any information about the final diagnosis. On physical examination, splenomegaly was present. Imaging studies were conducted with an abdominal ultrasound showcasing mild ascites, splenomegaly, with a homogeneous echo pattern and no focal mass. Computed tomography (CT) of the abdomen showed two hypodense areas in the subcapsular region of the spleen and extending into the capsule, suggestive of a tuberculous abscess with mesenteric lymphadenopathy. The diagnosis was further corroborated when the patient showed remarkable improvement on anti-tuberculous therapy. This is a very uncommon phenomenon, especially in an immunocompetent patient and hence, it is very important to keep this on the list of differentials especially in an area where TB is endemic.

From Gut to Heart: Havoc in a Young Patient with Typhoid-associated Cardiomyopathy.

Typhoid is an endemic hassle, especially in third-world countries like Pakistan. It is an enteric fever characterized by systemic manifestations that include high temperature and abdominal pain. If not properly treated, at times, it can transgress into complications predominantly involving the gut, where the site of pathology lies. Sometimes, however, it can involve other organ systems and pose diagnostic challenges owing to unfamiliar modes of presentation. Here in, we present a case of a 14-year-old male, previously afflicted and treated for typhoid who presented to the medical consult service with abdominal pain, high-grade fever, and mild chest discomfort. His hemodynamic parameters deteriorated within weeks as he developed pulmonary edema and hypoxemia. He was later diagnosed with echocardiography which earlier on, showed signs of acute myocarditis and eventually dilated cardiomyopathy. The patient was treated with antibiotics coupled with supportive and intensive care which yielded relief in his symptoms. He was later followed up with serial echocardiograms and showed improvement in the cardiac parameters.

Paraneoplastic Pemphigus with Underlying Castleman's Disorder: A Rare Report with Literature Review.

Paraneoplastic pemphigus (PNP), also known as paraneoplastic autoimmune multisystem syndrome (PAMS), is an autoimmune blistering disorder of the skin associated with various hematological and nonhematological malignancies. In most of the cases, it can be a harbinger of a concealed benign or malignant neoplasm. We report the case of a 23-year-old female patient who presented to the dermatology consult service with a previously known diagnosis of refractory pemphigus vulgaris but she failed to reach remission for her oral and skin lesions on steroid and intravenous immunoglobulin (IVIG). She was later investigated for underlying malignancy as the concern of PNP was raised. She was found to be diagnosed with a pelvic mass which was found to be Castleman's disease. Our patient responded well to surgery and postoperative course of pulsed methylprednisolone and IVIG. Here, we discuss the diagnosis and clinical course of this unique case and strive to create awareness about PNP that can present as a refractory polymorphous blistering dermatological disorder and can hinder the diagnosis and management of patients.

Congenital Cytomegalovirus Infection and Tetralogy of Fallot: An Unusual Association in a Three-month-old Baby.

Congenital cytomegalovirus (cCMV) infection is the leading cause of infant morbidity and mortality worldwide. Despite being associated with significant neurological sequelae in infected infants, it remains an under-recognized public health entity. Symptomatic newborns most frequently display hepatosplenomegaly, petechiae, jaundice, microcephaly, intrauterine growth restriction, chorioretinitis, purpura, and seizures. Progressive sensorineural hearing loss is the most prominent adverse outcome of both symptomatic and asymptomatic CMV infections in infants. We report the case of a three-month-old baby who presented with complaints of progressive jaundice for three months and a two days history of fever associated with one episode of fits. The baby was diagnosed with congenital CMV infection on the basis of positive CMV IgM and IgG and positive maternal serum CMV IgG. Finding a murmur on physical examination prompted echocardiography which revealed Tetralogy of Fallot (TOF). The child was managed with a 6-week course of ganciclovir after which his symptoms improved and he was referred to cardiology for the evaluation of his heart defect. Follow-ups at the clinic have shown normal growth and development. This is the first reported association of cCMV infection with TOF. This case highlights the need to consider the possibility of the presence of heart defects in all infants with cCMV infection in addition to neurodevelopmental abnormalities. Clinicians should maintain a high degree of suspicion for cCMV infection in all neonates to ensure timely intervention and to prevent long-term neurological sequelae.

Postprandial Head-drops: Insight into Systemic Effects of Ocular Timolol Preparation in Elderly.

Timolol is a nonselective beta-adrenergic antagonist with no intrinsic sympathomimetic activity. Conditions like intraocular hypertension (IOH) and primary open angle glaucoma (POAG) warrant the use of a topical ocular preparation of timolol. It works to effectively lower the intraocular pressure (IOP) in patients with glaucoma but due to its unique pharmacodynamics, it also poses some very striking systemic side-effects. These include heart block, arrhythmias, and syncopal episodes. Herein, we present a challenging case of a previously known healthy elderly 67-year-old female, known case of POAG, who presented to the clinic with a very peculiar complaint, i.e. episodes of her head dropping down on to the table multiple times after she was done eating her food. This led to a cascade of diagnostic tests being employed encompassing cardiovascular, endocrinal, neurological, and gastrointestinal systems; all of which came out to be negative. Finally, after a very thorough literature review, it was established that timolol was the true culprit. The cessation of the drug immediately resulted in lasting relief.

Foregut Anguish: A Rare Case of Median Arcuate Ligament Syndrome in Common Variable Immunodeficiency.

Median arcuate ligament syndrome (MALS), also known as celiac artery compression syndrome (CACS), is a rare clinical entity that is characterized by extrinsic compression of the celiac artery by the median arcuate ligament of the diaphragm. It mostly presents as a triad of post-prandial abdominal pain, weight loss, and bruit in the epigastrium. Given its variable and atypical presentation, it is a diagnostic challenge for physicians. MALS is supposed to be a diagnosis of exclusion and, thus, many investigations need to be done before coining it as a definitive diagnosis. Herein, we present a case of a 17-year-old female patient, a known case of common variable immunodeficiency (CVID) who presented to our setup with progressive, excruciating epigastric pain and bilious vomiting after undergoing excision of CVID-associated gastric adenocarcinoma (CAGA). Multiple sets of tests were conducted to rule out possible cardiopulmonary, musculoskeletal, and abdominal etiology. The diagnosis was made on the basis of findings depicted on three-dimensional computed tomographic angiography (3D-CTA) and ultrasound Doppler studies of the celiac artery. She underwent laparoscopic dissection of the median arcuate ligament with a celiac plexus block, which yielded immediate relief in symptomatology and was later followed up with duplex scans and showed complete remission of symptoms.

Methane and Constipation-predominant Irritable Bowel Syndrome: Entwining Pillars of Emerging Neurogastroenterology.

Functional gut disorders have long known to cause depravity in quality of life. Among the group of these heterogeneous disorders, irritable bowel syndrome (IBS) has been known to affect a large chunk of our population. IBS is not as simple as it sounds. Caused by a multitude of factors, the heterogeneity of this disorder has laid the foundation for research and the new principles of neurogastroenterology. Dysbiosis and methane production are one of the forthcoming factors that are currently under investigation. Down the road of exclusive enteric anaerobic fermentation of polysaccharides, methane is produced. It was considered to be an inert gas in the past, with little to no role in gut activity but now it is established that it has an impressive role in the etiology of constipation-predominant IBS (C-IBS). Acting as a neurotransmitter, it is known to affect ileal and colonic transit time, which has currently been shown in animal studies. Many laxatives, ionophore antibiotics, drugs like rifamixin and neomycin have been targeted against this very principle. Lately, lovastatin has emerged as a potential pharmacologic therapy to devoid the gut of methane without disrupting the gut niche in itself and has shown promise in relieving the symptoms of C-IBS. The goal of this article is to compile and assemble the literature available on IBS and the neuromodulation of methane to teach physicians and research scientists about the current age of gastroenterology and the growing need to emphasize the role of methane in the symptomatology of functional gut disorders like C-IBS.

Knowledge and Attitude Towards Cardiopulmonary Resuscitation Among Doctors of a Tertiary Care Hospital in Karachi.

Objective Cardiac arrest is an emergency, which can be managed effectively by sound knowledge and practice of basic life support (BLS) skills. However, it has been globally reported that the knowledge of doctors regarding cardiopulmonary resuscitation (CPR) and BLS is sub-standard. We conducted this study with the aim to assess the knowledge and attitude of doctors toward CPR in Dr. Ruth K.M. Pfau Civil Hospital, one of the largest tertiary care hospitals, in Pakistan. Methods We conducted a cross-sectional study, in Dr. Ruth K.M. Pfau Civil Hospital located in Karachi, Pakistan, using cluster sampling. A total of 285 doctors were interviewed. Results A majority of the doctors were unaware of the revised rate and depth of chest compressions (65.6% and 75.8% respectively). While many know the abbreviations of BLS and CPR (96.55% and 95.4%, respectively), 56.5% did not know what automated external defibrillator (AED) stood for. CPR was preferred over chest compression-only resuscitation (CCR) by 91.6% of the doctors. Half of the participants rated their knowledge as average. Most stated that they will not be reluctant to perform CPR in an emergency situation. The majority also agreed that BLS training should be an integral part of the medical curriculum. Conclusion There is an evident lack of knowledge of CPR among healthcare professionals, particularly regarding the updates made in the 2015 American Heart Association (AHA) guidelines. However, an overall positive attitude was observed.

Top 100 Most Influential Articles in the Field of Myeloid Neoplasms: A Bibliometric Study.

BACKGROUND: Bibliometric analyses are a tool employed by researchers and funding agencies to establish the most important areas of research in a particular field, and to determine which foci need increased research attention. Such analyses have been published in a variety of clinical specialties; however, a detailed literature search showed that no such study has been done for "myeloid neoplasms." In order to bridge this gap, we conducted a citation analysis of the 100 most influential articles on myeloid neoplasms. METHODS: Two independent researchers extracted relevant articles from the Scopus database. These articles were then ranked in descending order of citations and a list of the top 100 original articles was made. A further, more detailed list was created containing significant discriminating characteristics. RESULTS: The top cited articles were published over a period of 47 years, with most of them being published in the 5-year interval of 2001-2005. The citations ranged from 636 to 4,039. The articles originated from 28 different countries. Most of the articles were published in high-impact journals. CONCLUSION: Our analysis sheds light on the quality of work and driving trends, listing the most cited and impactful guideline articles within this field and aiding clinicians.

Xeroderma Pigmentosum - Cockayne Syndrome Complex (XP-CS) - Another case.

We present the case of a 3-year old girl with clinical manifestations typical of XP-CS, an extremely rare combination of Xeroderma Pigmentosum and Cockayne Syndrome. She had a swelling above the upper lip and multiple brown spots on her face, neck, arms and back. She was globally delayed, deaf, dumb and photophobic. MRI brain showed mild cerebral atrophy and bilateral demyelination. De Sanctis Cacchione variant (dSCS) and Rothmund Thomson syndrome (RTS), which were among the differential diagnosis were ruled out upon careful evaluation. Supportive treatment was given and regular checkups were recommended to monitor the progression of the disease but our patient did not show up for the follow up. This report shows that the diagnosis of XP-CS can be based on clinical features and MRI findings when the genetic testing is not available.

Efficacy of serum blood urea nitrogen, creatinine and electrolytes in the diagnosis and mortality risk assessment of patients with acute coronary syndrome.

BACKGROUND: Although blood urea nitrogen (BUN), creatinine (Cr) and electrolytes are not the mainstay of diagnosis in acute coronary syndrome (ACS) patients but they may have a role in providing a more detailed view of the complications and mortality rates. The aim of this study was to determine the efficacy of these parameters in the diagnosis and mortality risk-assessment of patients with ACS. METHODOLOGY: A total of 200 patients with ACS were recruited in this prospective study. The relationship of serum BUN, Cr and electrolytes with cardiac enzymes, Global Registry of Acute Coronary Events (GRACE) and mortality was assessed during a 6-months follow-up. Statistical test like multivariate linear regression and binary logistic regression analysis were applied. RESULTS: On multivariate linear regression analysis, serum potassium (K) (Unstandardized Coefficient B=-3.77; p=0.04) showed significant negative association with Creatine Kinease and serum BUN (Unstandardized Coefficient B=0.52; p=0.001) showed significant positive association with Troponin I. The patients with GRACE>105 had significantly higher levels of serum BUN and Cr. Receiver operating characteristic curves showed that area under curve (AUC) of BUN (0.7) was higher than AUC of Cr (0.5). Multiple adjusted model showed that patients with BUN>32.5mg/dl were almost 20 times more likely to be associated with mortality as compared to reference group. CONCLUSION: In addition to cardiac enzymes, K along with BUN and Cr may serve as important aid in diagnosis of ACS. BUN and Cr may also serve as important tools in mortality-risk assessment of ACS patients.

Prognostic value of blood count parameters in patients with acute coronary syndrome.

BACKGROUND: Recent studies have shown that complete blood count (CBC) parameters can effectively predict long-term mortality and re-infarction rates in acute coronary syndrome (ACS). However, the role of these parameters in predicting short term mortality has not been studied extensively. The main objective of this study was to determine whether CBC parameters can predict 30-days mortality and the incidence of major adverse cardiac event (MACE) in ACS patients. METHODOLOGY: A total of 297 patients with ACS were recruited in this prospective study. The relationship of baseline white blood cell (WBC) to mean platelet volume ratio (WMR) with MACE and mortality was assessed during a 30-days follow up. The patients were divided into two groups: Group A [WMR<1000] and Group B [WMR>1000]. Multivariate COX regression was performed to calculate hazard ratios (HR). RESULTS: WMR had the highest area under receiver operating characteristics curve and highest discriminative ability amongst all CBC parameters in predicting mortality. Patients in Group B had a higher mortality rate (p<0.001) than patients in Group A. WBC count (p=0.02), platelet count (p=0.04), WMR (p=0.008), platelet to lymphocyte ratio (p<0.001) and neutrophil to lymphocyte ratio (p=0.03) were significantly higher in the MACE-positive group as compared to MACE-negative. In multivariate cox regression analysis, WMR>1000 (HR=2.9, 95% confidence interval 1.3-6.5, p=0.01) was found to be strongest biochemical marker in predicting mortality. CONCLUSION: WMR is an easily accessible and an inexpensive indicator, which may be used as a prognostic marker in patients with ACS.

Efficacy of Hematological and Coagulation Parameters in the Diagnosis and Prognosis of Patients with Acute Coronary Syndrome.

Background : The diagnosis and management of acute coronary syndrome (ACS) have improved significantly over the past few decades; however, the recognition of myocardial ischemia still proves to be a dilemma for cardiologists. The aim of this study was to determine the role of hematological and coagulation parameters in the diagnosis and prognosis of patients with ACS. Methods: This prospective study recruited 250 patients with ACS and 250 healthy controls. The diagnostic role of hematological and coagulation parameters was assessed by comparing the patients with ACS with the control group. The relationships between these parameters and mortality were determined by dividing the patients into 2 groups: Group A (discharged) and Group B (patients who died within 30 days of follow-up). Multivariate Cox regression analysis was performed to calculate the hazard ratio (HR). Results: The mean age of the patients was 55.14±10.71 years, and 65.2% of them were male. Prothrombin time (P<0.001), activated partial thromboplastin time (P<0.001), mean platelet volume (MPV) (P<0.001), white blood cell (WBC) count (P<0.001), and red blood cell distribution width (RDW) (P<0.001) were significantly higher in the case group than in the control group. WBC count (P<0.001), RDW (P<0.001), and MPV (P<0.001) were significantly higher in the controls than in the case group. The Cox regression model showed that RDW above 16.55% (HR=6.8), MPV greater than 11.25 fL (HR=2.6), and WBC higher than 10.55×103/µL (HR=6.3) were the independent predictors of mortality. Conclusion: In addition to being the independent predictors of short-term mortality, RDW, WBC, and MPV when used together with the coagulation profile may aid in the diagnosis of ACS in patients presenting with chest pain.