Varicella-zoster virus (VZV) multifocal vasculopathy in a patient with systemic lupus erythematosus - a diagnostic and treatment dilemma.

Cerebral vasculopathy due to varicella-zoster virus (VZV) infection is well-documented. We report a fatal case of VZV multifocal vasculopathy in a patient with systemic lupus erythematosus (SLE) who presented with subacute changes in mental status and had multiple areas of hemorrhagic infarcts on brain imaging. However, the correct diagnosis was delayed by several confounding factors including the absence of zoster rash, normal cerebral angiography, persistently low cerebrospinal fluid (CSF) glucose and negative initial polymerase chain reaction (PCR) for VZV DNA in the CSF. Our case and literature review suggests that the sensitivity of PCR for VZV DNA in the CSF is low in VZV vasculopathy and clinical suspicion of this disease in the setting of characteristic imaging findings could be crucial to timely diagnosis.

A case of Strongyloides hyperinfection syndrome in the setting of persistent eosinophilia but negative serology.

Strongyloides stercoralis is a unique intestinal nematode with the ability to replicate and complete its life cycle without leaving the host. We report a fatal case of Strongyloides hyperinfection syndrome in a patient who had persistent eosinophilia for several years but negative Strongyloides serology. Our case suggests that ELISA serologies cannot solely be relied upon to diagnose Strongyloides stercoralis infection; history and clinical judgment remain crucial to this diagnosis.

The neurologist's dilemma: a comprehensive clinical review of Bell's palsy, with emphasis on current management trends.

BACKGROUND: Recent advances in Bell's palsy (BP) were reviewed to assess the current trends in its management and prognosis. MATERIAL/METHODS: We retrieved the literature on BP using the Cochrane Database of Systematic Reviews, PubMed, and Google Scholar. Key words and phrases used during the search included 'Bell's palsy', 'Bell's phenomenon', 'facial palsy', and 'idiopathic facial paralysis'. Emphasis was placed on articles and randomized controlled trails (RCTs) published within the last 5 years. RESULTS: BP is currently considered the leading disorder affecting the facial nerve. The literature is replete with theories of its etiology, but the reactivation of herpes simplex virus isoform 1 (HSV-1) and/or herpes zoster virus (HZV) from the geniculate ganglia is now the most strongly suspected cause. Despite the advancements in neuroimaging techniques, the diagnosis of BP remains one of exclusion. In addition, most patients with BP recover spontaneously within 3 weeks. CONCLUSIONS: Corticosteroids are currently the drug of choice when medical therapy is needed. Antivirals, in contrast, are not superior to placebo according to most reliable studies. At the time of publication, there is no consensus as to the benefit of acupuncture or surgical decompression of the facial nerve. Long-term therapeutic agents and adjuvant medications for BP are necessary due to recurrence and intractable cases. In the future, large RCTs will be required to determine whether BP is associated with an increased risk of stroke.

Superior mesenteric artery syndrome and its associated gastrointestinal implications.

The superior mesenteric artery (SMA) syndrome is a rare but potentially life-threatening gastrointestinal condition. Over the years, it has been referenced by several names, the most common of which is Wilkie's syndrome. These numerous terminologies have made it difficult to estimate its true frequency in the general population. Common symptoms associated with this syndrome include intermittent postprandial abdominal pain, nausea, and bilious vomiting. Our review revealed that although it is currently well-defined in the literature, the diagnosis of SMA syndrome remains challenging as other disorders can mimic its presentation. However, CT angiography is currently favored in the literature for diagnosis as it can not only show the narrowed aorto-mesenteric angle and distance, but also the extent of duodenal obstruction. In addition, we found no consensus on the preferred mode of therapy once SMA syndrome is diagnosed. The agreement among authors is that the treatment options should be based on severity of the disease, using conservative measures as the first line of therapy in mild SMA syndrome. Duodenojejunostomy is the preferred surgical approach when conservative management fails, or in severe cases.

Are pediatric brain tumors on the rise in the USA? Significant incidence and survival findings from the SEER database analysis.

INTRODUCTION: Central nervous system tumors are the second most common form of cancer in children between the ages of 1 and 19 years. We aimed to provide the most recent data on the incidence and survival of these tumors in the USA and to assess the literature. METHODS: Frequency, rates, and survival sessions were calculated using the November 2008 submission for the US Surveillance Epidemiology and End Results Program. Data were collected and analyzed for children and adolescents aged 1 to 19 years with primary brain tumors. RESULTS: We found that the incidence rate of all pediatric brain tumors has been on a gradual but steady increase from 1973 to 2008 (p < 0.001). The average annual increase was 1.37 %. Our survival analysis of the individual tumors revealed that the 5-year overall survival for children diagnosed between 1974 and 1978 with medulloblastoma was 43.7 %. However, this increased to 62.8 % for children diagnosed between 1999 and 2003. A similar survival trend was also observed when all the other pediatric brain cancer histologies were collectively analyzed (p < 0.001). CONCLUSIONS: From our study, we can conclude that contrary to previous reports indicating a plateau in the incidence rates of pediatric brain tumors since the mid-1980s, there has been an increase from 1973 to 2008. Potential causes include environmental carcinogens, but more research is needed to investigate the factors behind this sustained rise in incidence over the years.

One is the loneliest number: a review of the ganglion impar and its relation to pelvic pain syndromes.

The ganglion impar is often overlooked as a component of the sympathetic nervous system. Despite its obscurity, this ganglion provides a pathway for neurons by accommodating postganglionic sympathetics, visceral afferents, and somatic fibers traveling to and from the pelvis. Its classic anatomic location as described in the 1720's held up until recently, with the current literature now revealing a great deal of anatomical variability. This variation becomes important when the ganglion impar is used as a treatment target for patients with chronic pelvic pain - its primary clinical implication. The aim of this review was to provide a better understanding of the anatomy of ganglion impar, accounting for variation in size, shape, and location. In addition, the clinical importance and treatment modalities associated with the ganglion impar are outlined.

Is preoperative diagnosis possible? A clinical and radiological review of lymphoepithelial cysts of the pancreas.

Lymphoepithelial cysts of the pancreas are rare lesions found mainly in middle-aged men. They are usually benign and have no clear natural history except one study linking their occurrence with HIV infection. Nevertheless, they often cause enormous psychological stress to patients as they tend to mimic pancreatic neoplasms which are known to carry poor prognosis. The authors have therefore assessed the published literature from PubMed in order to determine whether lymphoepithelial cysts can be diagnosed preoperatively using novel imaging techniques. Based on our findings, it is evident that three-dimensional computed tomography scans, in-phase and out-of phase magnetic resonance imaging studies, and endosonography have enabled better characterization of pancreatic lymphoepithelial cysts than a decade ago. Endoscopic ultrasound-guided fine needle aspiration has also added considerably to the promise of preoperative diagnosis. Thus, the authors can affirm that despite surgical excision of the cyst with pathological examination being the gold standard for diagnosis, it is possible that a combination of the modern imaging techniques and/or minimally-invasive approach can enable patients avoid unnecessary surgery in the future.

When lithium hurts: a look at Ebstein anomaly.

Ebstein anomaly (EA) is a congenital defect of the tricuspid valve (TV) and the right ventricle (RV) in which the attachments of the septal and posterior valve leaflets are apically displaced. The latter creates 3 morphologic components inside the right heart, namely the right atrium proper, the atrialized RV, and the functional RV. This rare anomaly accounts for <1.5% of all congenital heart diseases. The current opinion among authors is that it is a genetically heterogeneous condition caused by failure of delamination of the TV leaflets from the underlying myocardium and the interventricular septum. Its characteristic electrocardiographic findings include tall, broad, right atrial P waves, prolonged PR intervals, and deep Q waves in the right precordial leads. Echocardiography is currently the best technique for diagnosing this anomaly, although cardiac magnetic resonance imaging is also gaining traction as an alternative modality. The management strategies for EA correlate with the age of the patient, severity of the heart disease, and/or associated cardiac abnormalities. TV repair, rather than valve replacement, is preferred because of its favorable long-term prognosis. Nevertheless, a large, randomized study is still needed to compare the different valve repair techniques used in patients with EA.

Acute necrotizing pancreatitis in the setting of CMV viremia and AIDS: a case report and review of literature from 1980 to 2012.

CONTEXT: Cytomegalovirus (CMV)-induced pancreatitis in patients with AIDS is a known entity with poor prognosis. CASE REPORT: We report a case of a 43-year-old woman with AIDS and CMV viremia who was evaluated for hypotension and found to have severe necrotizing pancreatitis. The authors have also conducted a MEDLINE search for CMV-induced pancreatitis from 1980 to 2012 and reviewed the pertinent results. DISCUSSION: Until mid-1990s in the United States, pancreatitis due to CMV was mainly diagnosed at autopsy in AIDS patients. However, presumably due to the advent of antiretroviral therapy, there has since been a significant decline in the number of reported cases among these individuals. Rather, our review revealed that the occurrence of CMV-induced pancreatitis has since been described in a variety of clinical settings, ranging from patients on corticosteroid therapy to immunocompetent persons. CONCLUSIONS: Clinicians need a high index of suspicion to timely diagnose CMV-induced pancreatitis as patients often present with non-specific signs and symptoms. As it occurred in our case, early intervention is crucial and may alter the outcome in such patients.

A friend to the airways: a review of the emerging clinical importance of the bronchial arterial circulation.

Lungs receive the bulk of their blood supply through the pulmonary arteries. The bronchial arteries, on the other hand, vascularize the bronchi and their surroundings. These two arteries anastomose near the alveolar ducts. Contrary to the pulmonary circulation which is fairly well studied, the bronchial arteries have been appreciated more by their absence, and in some cases, by an interruption in the pulmonary arterial flow. Therefore, a more accurate anatomical and functional knowledge of these atherosclerosis-resistant vessels is needed to help surgeons and clinicians to avoid iatrogenic injuries during pulmonary interventions. In this review, we have revisited the anatomy and pathophysiology of the bronchial arteries in humans, considering the recent advances in imaging techniques. We have also elaborated on the known clinical applications of these arteries in both the pathogenesis and management of common pulmonary conditions.

A review of subclavian steal syndrome with clinical correlation.

Subclavian 'steal' phenomenon is a function of the proximal subclavian artery (SA) steno-occlusive disease, with subsequent retrograde blood flow in the ipsilateral vertebral artery (VA). The symptoms from the compromised vertebrobasilar and brachial blood flows constitute the subclavian steal syndrome (SSS), and include paroxysmal vertigo, drop attacks and/or arm claudication. Once thought to be rare, the emergence of new imaging techniques has drastically improved its diagnosis and prevalence. The syndrome, however, remains characteristically asymptomatic and solely poses no serious danger to the brain. Recent studies have shown a linear correlation between increasing arm blood pressure difference with the occurrence of symptoms. Atherosclerosis of the SA remains the most common cause. Doppler ultrasound is a useful screening tool, but the diagnosis must be confirmed by CT or MR angiography. Conservative treatment is the initial best therapy for this syndrome, with surgery reserved for refractory symptomatic cases. Percutaneous angioplasty and stenting, rather than bypass grafts of the subclavian artery, is the widely favored surgical approach. Nevertheless, large, prospective, randomized, controlled trials are needed to compare the long-term patency rates between the endovascular and open surgical techniques.

The twisted colon: a review of sigmoid volvulus.

Sigmoid volvulus (SV) is the third leading cause of colon obstruction in adults. In infants and children, it is exceedingly rare with only sporadic cases reported so far. SVs from secondary causes, with congenital megacolon being the most important, are nevertheless more common in young people. The etiology of this disorder is not completely understood. It is known to occur in the setting of redundant sigmoid loop, which rotates around its narrow and elongated mesentery. Although the latter occurs in the setting of constipation, a congenitally elongated colon, and other predisposing factors, there is no consensus on the precipitating factor leading to SV formation. The symptoms are suggestive of small bowel obstruction, but the presentations can be acute or indolent. Plain abdominal radiography is used to diagnose SV in most cases with computed tomography scan or magnetic resonance imaging as the confirmatory tests when necessary. After it has been untwisted, the definitive and standard therapy for SV is sigmoid resection and primary anastomosis. The nonresective alternatives have also been widely used with mixed success, but a large, randomized controlled trial is needed to compare their efficacy with resection and primary anastomosis. Laparoscopic surgery in SV management is unwarranted and costly. Complications of SV include hemorrhagic infarction, perforation, septic shock, and death. The mortality data from SV vary, but the latest literature cites an overall range of 14 to 45 per cent.

Francis Sibson (1814-1876): the unsung hero of modern surgery and anesthesia.

Francis Sibson (1814-1876) was a British physician and anatomist widely regarded as a true pioneer of the medical profession. He overcame a tragic and difficult childhood to become an avid educator at several medical schools and dedicated his life to research. For modern scientists, he is most remembered for describing Sibson's fascia, his experimental use of curare in the treatment of hydrophobia and tetanus, and his detailed description of the positions and movements of internal organs. He died on September 7, 1876 at the age of 62 from complications of an aortic aneurysm.

August Forel (1848-1931): a look at his life and work.

Dr. August Henri Forel was a world-renowned psychiatrist, neuroanatomist, myrmecologist, social reformer, and promoter of world peace. His collected works (1907) included a description of what is now known as the tegmental fields of Forel and the zona incerta. In 1887, he described the cellular functional units within the brain, setting the foundation for what would later become known as the neuron theory. He also studied the thalamus and hypothalamus and gave detailed descriptions of the trigeminal, vagus, and hypoglossal nerves. His work was honored with the naming of the ventral tegmental decussation after him. Forel battled alcoholism and dedicated his work to numerous social reforms as well as advocating for world peace. As a devoted pacifist, he attempted to bring the First World War to a close. A decade before he died, Forel became a member of the Baha'i Faith and was a founding influence for the National Spiritual Assembly of Baha'is of Switzerland. His image has appeared on Swiss postage stamps and 1,000 Franc bank notes.

Computational studies of Fe(III) binding to bryostatins, bryostatin analogs, siderophores and marine natural products: arguments for ferric complexes in medicinal applications.

In this computational study, geometric factors are calculated by applying semi-empirical methods (PM3) that support experimental evidence from this lab where bryostatins can bind trivalent iron with six Fe-O bonds forming an octahedral geometry. The geometric factors are calculated for all 20 structures (Fe3+ bound to bryostatin 1-20) as a neutral, monovalent, and divalent species. The average Fe-O bond distances and bond angles are compared to those of known marine and terrestrial siderophores. From these two data sets, we then examined other known marine natural products (MNPs) that can form a hexavalent complex with six Fe-O bonds and draw conclusions about their potential biological role as marine siderophores. This computational data indicates that Fe(III) strongly bonds to a host of MNPs, increasing their water solubility, contracting their structure, hence allowing transport through cell membranes more readily, and in some cases, stabilizing ester bonds that are susceptible to hydrolysis. It is argued that administering medicinally bryostatin, its analogs or other MNPs as a ferric complex, holds some fundamental chemical advantages compared to its administration as a neutral uncomplexed species.