Complicated multi-drug resistant typhoid fever with cerebral oedema, diffuse encephalitis and fungal infection of the oral cavity: A case report.

Enteric fever is a highly fatal infectious disease that can present with extensive symptoms that renders diagnosis quite risky. Multi-drug resistant Salmonella typhi infection has become endemic in third world countries and has been routinely associated with catastrophic complications and even death, with diagnostic and therapeutic impedance. Typhoid fever is known to cause life-threatening cerebral complications. We report the case of a 16-year-old male who presented to us with a high-grade fever, watery diarrhoea, altered level of consciousness, and a mixed dark-coloured crusted oral lesion. Blood workup showed neutropenia, lymphocytopenia, thrombocytopenia, transaminitis, and hyponatraemia. Blood culture grew multi-drug resistant Salmonella Typhi. CT scan of the brain showed diffuse cerebral oedema, while EEG was consistent with the diagnosis of diffuse encephalitis. The patient responded well to culture-sensitive antibiotics, while the oral lesion showed a dramatic response to presumptive antifungal treatment. We discuss the compositions available to date on typhoid-associated encephalitis and the connection of fungal infection in this specific case attempting to promote awareness regarding possible unorthodox presentations of enteric fever.

Primary Pulmonary Leiomyosarcoma: An Unusual Cause of Pleural Effusion.

Primary pulmonary leiomyosarcomas (PPLs) are extremely rare tumors of the lungs. They can present with non-specific symptoms or can also be asymptomatic with clues towards diagnosis being found on routine examination or radiographs. We present a case of a 54-year-old woman who presented with worsening shortness of breath and spells of dizziness. Her chest radiographs showed right-sided pleural effusion and CT revealed a large enhancing pleural mass with compression atelectasis and mediastinal shift. She underwent a thoracoscopy and right pleural biopsy. Histopathology and immunohistochemistry were most consistent with leiomyosarcoma. An extensive search for a possible primary in other sites was unrevealing, thus diagnosing the patient with PPL. She was managed with surgery and radiotherapy.

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.

A Perplexing Case of Recurrent Abscess-Like Lesions: Insights Into Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type.

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) is one of the rarest forms of primary cutaneous lymphomas (PCLs) and it confers a poor prognosis. Diagnosis of PCDLBCL-LT can be challenging and complex as it can manifest with a myriad of dermatological presentations. However, early treatment with chemo-radiation leads to an appropriate response. We present the case of a 66-year-old female with a history of polymyositis and interstitial lung disease on immunosuppression who presented to our institution with recurrent abscess-like lesions localized to buttocks that were later biopsied and diagnosed as the leg-type variant of PCL. She received chemotherapy with the rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) regimen and subsequent involved-site radiation therapy (ISRT), which resulted in complete remission. The patient was later followed up and remained in remission for years.

Acquired Hemophilia A in Peripartum Period: Diagnostic and Therapeutic Dilemma.

Acquired hemophilia A (AHA) is a bleeding diathesis caused by auto-antibody generation against factor VIII, an essential component of the coagulation cascade. Although having many etiologies, pregnancy is also one of the conditions associated with AHA. It mostly presents as a raised activated partial thromboplastin time (aPTT), and during the peripartum and postpartum period, concern for AHA should be raised as delays in diagnosis can be detrimental. Herein, we present a case of a 31-year-old female with sickle cell trait who developed venous bleeding and, later, neuraxial, musculoskeletal, and subcutaneous bleeding. She underwent an extensive course of treatment before getting into remission.

Advanced Squamous Cell Carcinoma of Gall Bladder Masquerading as Liver Abscess With Review of Literature Review on Advanced Biliary Tract Cancer.

Advanced biliary tract carcinoma (ABC) tends to have a poor prognosis, with trials done having limited data from oncologists' perspectives. Squamous cell variant of gallbladder cancer (GBC) is one of the rarest forms of cancer known in the literature, with a very aggressive course and dismal prospects. Herein, we present a case of a 67-year-old man who got diagnosed with squamous cell carcinoma, which initially masqueraded as liver abscess and was associated with severe hypercalcemia, pyrexia, jaundice, and submassive pulmonary embolism.

Metachronous Renal Cell Carcinoma: A Rare Presentation of Adrenal Crisis.

Responsible for 2% of global cancer diagnoses, renal cell carcinoma (RCC) can metastasize to almost every organ system; however, metastasis to the contralateral adrenal gland is extremely rare. We report the case of a 59-year-old male who presented with atypical chest pain and altered mental status. The patient developed hypotension, with hyponatremia raising concern for adrenal insufficiency (AI). We confirmed a diagnosis of AI secondary to adrenal metastasis in the setting of radical nephrectomy with ipsilateral adrenalectomy, and the patient's symptoms resolved with adequate treatment. This report emphasizes the importance of complications caused by metastatic disease to the remaining adrenal gland in patients with RCC who have undergone ipsilateral radical nephrectomy.

Frequency of Hyperlactatemia in Intensive Care Patients within Tertiary Care Hospital in Pakistan.

Objective The aim of this study is to determine the frequency of hyperlactatemia in intensive care patients with sepsis in Civil Hospital Karachi. Method The cross-sectional study was conducted at a tertiary care hospital for six months and comprised all those participants who were older than 12 years of age, had sepsis, and were admitted in the intensive care unit (ICU) within 24 hours. Participants with suspected secondary acidemia from acute liver failure, end-stage renal disease, on anti-retroviral therapy, an overdose of salicylates, alcohols, acetaminophen, metformin user, intestinal resection, or bacterial overgrowth were excluded from the study.  Arterial blood samples were collected for lactate levels by the researcher himself, within 24 hours of admission to the ICU. The samples were stored in fluoride tubes and were kept at around four-degree centigrade temperature to avoid any alteration in lactate levels. Demographic details, diagnoses, vitals (blood pressure, heart rate, and respiratory rate) along with lactate levels and criteria for sepsis (including SIRS) of all patients were recorded in the proforma. Lactate level above 1.6 millimoles per liter (mmol/L) was taken as elevated (i.e. hyperlactatemia). SPSS version 16.0 was used for data analysis. Frequency and percentage were calculated for age categories, gender, and hyperlactatemia. Mean, and the standard deviation was calculated for the age of the patient and lactate levels. Effect modifiers were controlled through stratification for age, gender, co-morbidities, and duration of illness. p-value ≤ 0.05 was taken as significant. Results A total of 354 patients with sepsis were included in the study. The mean age of the patients was 42.93 (±19.03) years. Out of 354 patients, 169 (47.7%) were males, and 185 (52.3%) were females. Overall, the frequency of hyperlactatemia in intensive care patients with sepsis was 36.4%. The mean value of lactate was 1.963 mmol/L. With respect to stratification for age, hyperlactatemia was more frequent in the elderly patient population (47%) as compared to the young (31%) and middle (30%) aged patients with sepsis (p-value = 0.013). For gender, hyperlactatemia was more frequent in females (39%) than in males (33%). Concerning the duration of illness, hyperlactatemia was more frequent in those patients who were ill for more than one month (80%) than in those who were ill for less than seven days (30%) or 7 to 30-day period (36%) (p-value <0.001). Concerning co-morbidities, hypertension was the most common co-morbid condition in the study population (30%). Hyperlactatemia was significantly frequent in those patients who already had cardiac problem or stroke (66% [p value = 0.003] and 51% [p value = 0.013], respectively). Conclusion Hyperlactatemia is a common finding in patients with sepsis in an ICU. Being a marker of hypoxia, lactate level measurements can be of vital importance in a critical care setup, where they can be utilized to determine various factors such as mortality, morbidity, and duration of intensive care. Lactate levels thus should be evaluated in clinical studies, in correlation with various parameters more frequently. Lactate levels can also rise in various conditions apart from sepsis which merits further investigation.

Neuroendocrine Tumour Of Unknown Primary Origin: Unusual Case Of Metastatic Hepatic Foci In A Female Patient.

Under the banner of cancers of unknown primary origin (CUPs), neuroendocrine tumours account for less than five percent of the neoplasms. The clinical manifestations and management depend upon the tumour's grade and differentiation and its site of growth. At times, despite of aggressive search for primary origin, cancer remains hidden. Herein, we present a case of a middle-aged woman who presented to our tertiary set-up with complaints of abdominal pain and distension. After a series of radiologic and interventional investigations including positron emission tomography with liver biopsy and immunohistochemical analysis, a diagnosis of the welldifferentiated neuroendocrine tumour was made, located in the right lobe of the liver. However, the primary origin could not be identified. The patient was managed with trans-arterial chemoembolization (TACE) followed by hepatic resection and was followed biennially afterwards. In our case, hepatic metastasis was treated with chemoembolization and stagedresection and provided a good prognosis to the patient. Our case is unique as only a few case reports have been published with following presentation and documentation of efficacious treatment is needed to contribute to the literature. Proper trials with exteriorization of bowel and radiological imaging is necessary to stage the primary tumour, even if end result is in vain. This will help to further improve the prognosis.

L-looped Transposition of the Great Arteries in a Patient with Marfanoid Habitus: First Reported Case in Literature.

L-looped transposition of great arteries (L-TGA) is an extremely rare heart condition. It is associated with physiologically corrected transposition of great vessels, leading to the normal return of deoxygenated systemic venous blood to the heart and transport of oxygenated pulmonary venous blood to the main systemic circulation. Anatomic discordancy and anomalous coronary artery distribution predispose the right ventricle to excessive workload and eventual heart failure. This mandates anatomic correction or proper medical management of heart failure. Herein, we present a case of a 14-year-old girl who presented to our cardiology consults with a false impression of pre-made clinical diagnosis of tetralogy of Fallot. She had increasing dyspnea and cyanosis on presentation. Striking marfanoid habitus and unusual echocardiographic findings of tripartite geometry of heart with parallel and discordant positioning of atria, ventricles, and great arteries led us to cardiac computed tomography which confirmed the diagnosis of L-TGA. Our patient also had associated patent ductus arteriosus, dextrocardia, ventricular septal defect (VSD), and pulmonary atresia. Due to the complex nature of heart disease and unavailability of resources, she was treated with a comprehensive heart failure protocol and followed up clinically and radiologically at regular intervals and showed massive improvement. This is the first-ever documented case of L-TGA with complex shunting and marfanoid habitus.

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.

Perplexing Case of Wilkie's Syndrome: A Rare Presentation in a Young Patient.

Wilkie's syndrome, also commonly known as superior mesenteric artery (SMA) syndrome, is an infrequent and rare cause of small bowel obstruction. It is caused by extrinsic compression of the duodenal segment of the intestine between the aorta and SMA, causing significant post-prandial abdominal pain and vomiting. The literature suggests the incidence of 0.01% to 0.3%. We present here an atypical presentation of SMA syndrome in which a young patient presented to our tertiary set-up with unusually constant abdominal pain and weight loss requiring invasive interventions.

Gut Entrapped in the Thorax: A Rare Presentation of Abdominal Pain in Chilaiditi Syndrome.

Chilaiditi syndrome is a rare disorder comprising of the interposition of the gut between the diaphragm and liver. This can lead to a spectrum of gastrointestinal and respiratory presentations, primarily in the elderly population in whom the disorder is relatively more prevalent. We present a case of a 63-year-old man who presented to our setup with abdominal pain and shortness of breath and later got diagnosed with Chilaiditi syndrome. He was managed conservatively and showed complete resolution of the symptoms.

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.

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.