Rheumatoid meningitis sine arthritis.

Rheumatoid meningitis is a rare and very serious extra-articular manifestation of rheumatoid arthritis. We present a case of a 7()year-old female with no history of arthritis who developed stroke-like symptoms, seizures, psychosis and compulsive behavior. Serial brain magnetic resonance images (MRI) over four months demonstrated progressive interhemispheric meningeal thickening. She had mild lymphocytic pleocytosis on the cerebrospinal fluid analysis and serum anti-cyclic citrullinated peptide antibodies resulted positive in high titers. She underwent a brain biopsy showing necrotizing granulomas consistent with rheumatoid meningitis. Her symptoms resolved with treatment with glucocorticoids and cyclophosphamide. She has not been diagnosed with rheumatoid arthritis even after 1 year of follow up. Clinicians should be aware of the possibility of rheumatoid meningitis without rheumatoid arthritis and keep it on the differential for patients with aseptic meningitis and otherwise negative work up

Metastatic Lobular Breast Carcinoma in a Meningioma: A Case Study

Hormonal relationships between breast carcinoma and meningioma have long been reported in the literature. This association may be related to the widespread expression of progesterone receptors in meningiomas. Indeed, meningiomas are more common in women and may demonstrate increased growth during pregnancy. Women have an increased risk of meningioma following breast carcinoma diagnosis and vice versa. However, much more uncommon is a tumor to tumor metastases of breast carcinoma into a meningioma. We report a case of a 56 year old female with a past medical history of breast cancer, found to have a right sphenoorbital meningioma with metastatic lobular carcinoma. Pathological findings include a WHO grade 1, transitional meningioma with IHC staining for EMA, marking not only a meningioma but also linearly arranged epithelioid cells. Determined to be metastases of invasive lobular carcinoma, these epithelioid cells were E-cadherin negative and GATA3, GCDFPmammoglobin, and ER positive. Progesterone receptor staining was inconclusive due to the strongly progesterone staining meningioma. Radiographic studies lack the sensitivity or specificity to diagnose intrameningioma metastases. Furthermore, because meningiomas and breast carcinomas are often both steroid receptor and EMA positive, careful histopathologic analysis of the entire meningioma may help to overcome challenges in diagnosing such metastases

Desmin common mutation is associated with multi-systemic disease manifestations and depletion of mitochondria and mitochondrial DNA.

Desmin (DES) is a major muscle scaffolding protein that also functions to anchor mitochondria. Pathogenic DES mutations, however, have not previously been recognized as a cause of multi-systemic mitochondrial disease. Here, we describe a 45-year-old man who presented to The Children\u27s Hospital of Philadelphia Mitochondrial-Genetics Diagnostic Clinic for evaluation of progressive cardiac, neuromuscular, gastrointestinal, and mood disorders. Muscle biopsy at age 45 was remarkable for cytoplasmic bodies, as well as ragged red fibers and SDH positive/COX negative fibers that were suggestive of a mitochondrial myopathy. Muscle also showed significant reductions in mitochondrial content (16% of control mean for citrate synthase activity) and mitochondrial DNA (35% of control mean). His family history was significant for cardiac conduction defects and myopathy in multiple maternal relatives. Multiple single gene and panel-based sequencing studies were unrevealing. Whole exome sequencing identified a known pathogenic p.S13F mutation in DES that had previously been associated with desmin-related myopathy. Desmin-related myopathy is an autosomal dominant disorder characterized by right ventricular hypertrophic cardiomyopathy, myopathy, and arrhythmias. However, neuropathy, gastrointestinal dysfunction, and depletion of both mitochondria and mitochondrial DNA have not previously been widely recognized in this disorder. Recognition that mitochondrial dysfunction occurs in desmin-related myopathy clarifies the basis for the multi-systemic manifestations, as are typical of primary mitochondrial disorders. Understanding the mitochondrial pathophysiology of desmin-related myopathy highlights the possibility of new therapies for this otherwise untreatable and often fatal class of disease. We postulate that drug treatments aimed at improving mitochondrial biogenesis or reducing oxidative stress may be effective therapies to ameliorate the effects of desmin-related disease

A Field Evaluation of the LuciTrap and the Western Australian Trap with Three Different Baits Types for Monitoring Lucilia cuprina and Lucilia sericata in New Zealand

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Severe eosinophilic syndrome associated with the use of probiotic supplements: a new entity?

Severe eosinophilic syndromes related to the administration or use of unsuspected immunogenic substances have been described previously. Many of these diseases presented initially as clusters or isolated cases. The spanish toxic oil syndrome, the eosinophilia myalgia syndrome, and nephrogenic systemic fibrosis are examples of such diseases. We describe 2 cases of a severe eosinophilic syndrome characterized by marked peripheral blood eosinophilia (\u3e15,000 cells/ml), mononeuritis multiplex, and necrotizing vasculitis which developed in a close temporal association with the recent onset use of nonprescription probiotics. There was no history of a prior autoimmune disease. Although both cases had prompt response to immunosuppression with rapid resolution of peripheral blood eosinophilia and accompanying constitutional symptoms, they remained with permanent neurological deficits

Characterization of the chicken inward rectifier K(+ )channel IRK1/Kir2.1 gene

BACKGROUND: Inward rectifier potassium channels (IRK) contribute to the normal function of skeletal and cardiac muscle cells. The chick inward rectifier K(+ )channel cIRK1/Kir2.1 is expressed in skeletal muscle, heart, brain, but not in liver; a distribution similar but not identical to that of mouse Kir2.1. We set out to explore regulatory domains of the cIRK1 promoter that enhance or inhibit expression of the gene in different cell types. RESULTS: We cloned and characterized the 5'-flanking region of cIRK1. cIRK1 contains two exons with splice sites in the 5'-untranslated region, a structure similar to mouse and human orthologs. cIRK1 has multiple transcription initiation sites, a feature also seen in mouse. However, while the chicken and mouse promoter regions share many regulatory motifs, cIRK1 possesses a GC-richer promoter and a putative TATA box, which appears to positively regulate gene expression. We report here the identification of several candidate cell/tissue specific cIRK1 regulatory domains by comparing promoter activities in expressing (Qm7) and non-expressing (DF1) cells using in vitro transcription assays. CONCLUSION: While multiple transcription initiation sites and the combinatorial function of several domains in activating cIRK1 expression are similar to those seen in mKir2.1, the cIRK1 promoter differs by the presence of a putative TATA box. In addition, several domains that regulate the gene's expression differentially in muscle (Qm7) and fibroblast cells (DF1) were identified. These results provide fundamental data to analyze cIRK1 transcriptional mechanisms. The control elements identified here may provide clues to the tissue-specific expression of this K(+ )channel

Lymphocytic Hypophysitis

A 59 year-old female presented with visual problems in 2006, and was initially diagnosed with a pituitary macroadenoma on MRI (Figure 1). Preoperatively, the pituitary lesion decreased in size after the patient was started on steroids, raising the possibility of lymphoma and inflammatory conditions. Ultimately, endoscopic trans-nasal biopsy of the pituitary lesion revealed lymphocytic hypophysitis. She was treated with steroids for two years and developed several side effects from chronic steroid use, including Cushingoid features, diabetes, cataracts, weight-gain and osteopenia. As a result, she was subsequently treated with methotrexate and gradual weaning of the steroids

A Metastatic Middle Cerebral Artery Aneurysm Caused by an Intraluminal Bronchogenic Tumor Embolus

Objective and Importance: To describe the clinical presentation and neuropathological findings of metastatic cerebral aneurysms of bronchogenic origin. Metastatic bronchogenic cerebral aneurysms are exceptionally rare and have only been reported in association with high grade hemorrhage. Clinical Presentation: One patient presenting with a history of headache, speech difficulty, left-sided “numbness”, left seventh nerve palsy and left hemiparesis was found to have intraparenchymal and subarachnoid hemorrhage with an aneurysm of the right distal Sylvian middle cerebral artery. Intervention: Pterional craniotomy with excision of mycotic segment. Surgical specimen sent to pathology for routine histology and immunohistochemistry. Conclusion: Patients with metastatic cerebral aneuryms of bronchogenic origin usually present with subarachnoid hemorrhage, contrary to prior observations that “hemorrhage from neoplasia-induced aneurysms is paradoxically rare.” A tumor embolus should be considered in the differential diagnosis of a mycotic aneurysm

Solar Physics and the Solar-Stellar Connection at Dome C

Solar magnetic fields evolve on many time-scales, e.g., the generation, migration, and dissipation of magnetic flux during the 22-year magnetic cycle of the Sun. Active regions develop and decay over periods of weeks. The build-up of magnetic shear in active regions can occur within less than a day. At the shortest time-scales, the magnetic field topology can change rapidly within a few minutes as the result of eruptive events such as flares, filament eruptions, and coronal mass ejections. The unique daytime seeing characteristics at Dome C, i.e., continuous periods of very good to excellent seeing during almost the entire Antarctic summer, allow us to address many of the top science cases related to the evolution of solar magnetic fields. We introduce the Advanced Solar Photometric Imager and Radiation Experiment and present the science cases for synoptic solar observations at Dome C. Furthermore, common science cases concerning the solar-stellar connection are discussed in the context of the proposed International Concordia Explorer Telescope.Comment: 8 pages, 2 b/w figures, submitted to 2nd ARENA Conference on "The Astrophysical Science Cases at Dome C'', H. Zinnecker, H. Rauer, and N. Epchtein (eds.), EAS Publications Serie