Lamotrigine improves aggression in patients with temporal lobe epilepsy

Aggression in patients with temporal lobe epilepsy (TLE) may have phenomenological and neurobiological heterogeneity. In the present study, we targeted patients with TLE who showed aggression and evaluated the effects of lamotrigine on this symptom using the Buss-Perry Aggression Questionnaire (BAQ), which is based on a four-factor model that includes Physical Aggression, Verbal Aggression, Anger, and Hostility. As compared with the healthy control subjects (n=115), patients with TLE (n=21) had significantly higher BAQ Total, Physical Aggression, Anger, and Hostility scores. Ten weeks after initiation of lamotrigine, the BAQ Total and Anger scores of the patients with TLE were significantly improved. However, the patients with TLE in this study did not exhibit depressive symptoms. Our results suggest that lamotrigine mitigates aggression, especially anger, which represents the emotional factor of aggression in the BAQ.     

Association between long-term smoking and hypertension and early-onset nodular glomerulosclerosis

Diabetic nodular glomerulosclerosis, also known as Kimmelstiel-Wilson syndrome, is a specific pathological variant of diabetic nephropathy ; however, histological findings similar to diabetic nephropathy are observed occasionally without glucose intolerance. Therefore, such nodular glomerulosclerosis is called idiopathic nodular glomerulosclerosis. Several case reports that have been published recently indicate that smoking and hypertension, which are classical renal risk factors, may be attributed to this form of glomerular degeneration. Accordingly smoking- and hypertension-associated nodular glomerulosclerosis has been considered to be different from the idiopathic form. This novel form of nodular glomerulosclerosis is associated with a history of long-term smoking and hypertension, and the age of onset of this disease is more than 60 years. We present the case of a 27-year-old Japanese male who was admitted to our hospital with nephrotic syndrome, hypertension, and renal impairment. He had a smoking history of at least 13 years, and had been exposed to passive smoking for several years because his parents were smokers. Renal biopsy revealed diffuse and global nodular glomerulosclerosis, although the patient did not have any primary diseases such as diabetes mellitus or paraproteinemia, that can cause this condition. We diagnosed smoking- and hypertension-associated nodular glomerulosclerosis. Cessation of smoking and the administration of an angiotensin II receptor blocker decreased his proteinuria and showed recovery of kidney function. This case report suggests that long-term smoking is closely associated with nodular glomerulosclerosis. Further, in our case, the age of the patient was lower than that of patients with the same disease among cases that have been reported previously.     

Staged Fontan operation for children with heterotaxy syndrome,bilateral ductus arteriosus,and nonconfluent pulmonary artery

We report two cases of successful Fontan operation in children with heterotaxy syndrome associated with univentricular physiology and absent and nonconfluent central pulmonary arteries with both distal pulmonary arteries directly connected to the ipsilateral ductus arteriosus. After unilateral systemic-pulmonary shunt, the central pulmonary artery was reconstructed with a polytetrafluoroethylene prosthetic graft concomitantly with bidirectional cavopulmonary shunt. Finally, extracardiac total cavopulmonary connection was performed as an off-pump procedure. Children with bilateral ductus arteriosus and a nonconfluent pulmonary artery with univentricular physiology present a particular challenge in regard to completing Fontan operations. Careful attention should be directed at ensuring balanced growth of the bilateral distal pulmonary arteries. When planning reconstruction of the central pulmonary artery with a prosthesis, late reconstruction may be beneficial, as it enables utilization of a larger-caliber graft, obviating the need for replacement during a subsequent Fontan operation.     

Attenuation of folic acid-induced renal inflammatory injury in platelet-activating factor receptor-deficient mice

Platelet-activating factor (PAF), a potent lipid mediator with various biological activities, plays an important role in inflammation by recruiting leukocytes. In this study we used platelet-activating factor receptor (PAFR)-deficient mice to elucidate the role of PAF in inflammatory renal injury induced by folic acid administration. PAFR-deficient mice showed significant amelioration of renal dysfunction and pathological findings such as acute tubular damage with neutrophil infiltration, lipid peroxidation observed with antibody to 4-hydroxy-2-hexenal (day 2), and interstitial fibrosis with macrophage infiltration associated with expression of monocyte chemoattractant protein-1 and tumor necrosis factor-alpha in the kidney (day 14). Acute tubular damage was attenuated by neutrophil depletion using a monoclonal antibody (RB6-8C5), demonstrating the contribution of neutrophils to acute phase injury. Macrophage infiltration was also decreased when treatment with a PAF antagonist (WEB2086) was started after acute phase. In vitro chemotaxis assay using a Boyden chamber demonstrated that PAF exhibits a strong chemotactic activity for macrophages. These results indicate that PAF is involved in pathogenesis of folic acid-induced renal injury by activating neutrophils in acute phase and macrophages in chronic interstitial fibrosis. Inhibiting the PAF pathway might be therapeutic to kidney injury from inflammatory cells.     

Proton beam therapy for inoperable recurrence of bronchial high-grade mucoepidermoid carcinoma

We report the case of a 17-year-old patient who received four courses of proton beam therapy for inoperable recurrent high-grade bronchial mucoepidermoid carcinoma of the chest wall and lymph nodes. The equivalent doses in conventional fractionation of 79.2-80.6 Gy were applied to the tumor from the first to third courses of proton beam therapy; the hemi-chest wall was also irradiated prophylactically in the third course. The irradiated tumor recurred marginally and liver metastasis developed, but tumor size within the irradiated field was suppressed. Proton beam therapy was also applied to the marginally recurrent tumor in the fourth course. The patient died of cancer about 5 years after the first course of proton beam therapy-about 9 years after the initial diagnosis and surgery. Repeated irradiation of the mediastinum and chest wall with photon radiotherapy is often limited by side-effects in the heart, esophagus and spinal cord. However, no severe late complications in critical organs were detected in this case. Only a Grade 2 skin reaction and lymphatic edema were observed. Therefore, high-dose proton beam therapy may be an option as a salvage therapy with less toxicity to normal tissues compared with photon radiotherapy and provide an alternative to repeated surgery.     

Potassium octatitanate fibers (TISMO) induce pleural mesothelial cell reactions with iron accumulation in female A/J mice

It is crucial to develop therapeutic approaches for malignant mesothelioma, as well as to obtain information involving the possible mechanism involved in the development of mesothelioma. Subsequently, thoracotomy was performed to infuse test particles directly into the thoracic cavity of A/J mice. Fiber-shaped particles of potassium octatitanate (TISMO) and granular-shaped micro- and nano-size order particles of titanium dioxide (TiO(2)) were employed (1.5 mg in 0.2 ml saline/mouse). The experiment was terminated after 21 weeks to assess responses. Only the fiber-shaped TISMO, morphologically similar to asbestos, induced a severe reaction of the pleura. A number of TISMO fibers were observed in the alveoli, indicating penetration through the pleura. Following Berlin blue staining, positive spots were observed around the TISMO fibers, indicative of iron. These positive spots corresponded with cells that immunostained positively for calretinin, a marker of mesothelial cells. Similar observations were reported for asbestos-induced mesothelioma. The present study showed that only fiber-shaped TISMO induced severe reactions of the mesothelium in the pleura, and these involved iron accumulation derived from endogenous sources. The results indicate that the risk of mesothelial cell reaction does not depend on particle size, but may depend on shape.     

Multiple Endocrine Neoplasms and Serous Cysts of the Pancreas in a Patient with von Hippel-Lindau Disease

Introduction

Patients with the von Hippel–Lindau (VHL) syndrome have an increased risk of developing pancreatic endocrine neoplasms.

Case Report

A 40-year-old female with a past history of bilateral adrenal pheochromocytomas, whose brother had a history of a malignant pheochromocytoma, was referred to our institution with symptoms of adrenal crisis including general fatigue and insomnia in spite of increasing predonisolone therapy. Screening by ultrasonogram and computed tomography demonstrated multiple well-demarcated, enhancing nodules (maximum, 3 cm) in the head and tail of pancreas and multiple cystic lesions throughout the entire pancreas. Although this patient met the criteria for VHL, magnetic resonance imaging for her brain did not reveal any lesions. Pancreatoduodenectomy with partial distal pancreatectomy was performed. Histologic examination of the resected pancreas revealed multiple well-differentiated endocrine neoplasms accompanied with multiple serous cysts. The patient remains free of disease 5 years after surgery.     

Two cases of chronic active Epstein-Barr virus infection in which EBV-specific cytotoxic T lymphocyte was induced after allogeneic bone marrow transplantation

Abstract:  CAEBV is a high mortality and morbidity disease with life-threatening complications. Nevertheless, the treatment regimens for CAEBV have not yet been established. Although some reports have described CAEBV therapy involving treatments such as antiviral drugs, immunomodulatory agents, and immunochemotherapy, none of these treatments have been demonstrated to be effective. The only treatment reported to be effective is allogeneic SCT. However, the complications of SCT are severe, so treatment results have been poor. Recently, immunotherapy has been devised, but this is still in the developmental stage. In this report, two cases of CAEBV in which allogeneic SCT was performed soon after diagnosis are reported. In both cases, a high EBV genome titer in the peripheral blood was detected at onset. After SCT, the EBV genome titer decreased as CTL activity gradually increased. This fact suggested that not only high-dose chemotherapy as a preconditioning treatment of SCT but also increased CTL activity which could eliminate virus-infected cells might be effective, although additional cases should be studied in order to establish effective treatments.     

A long-surviving case of gastric cancer with main portal vein tumor thrombus after surgical resection and postoperative S-1 therapy

Gastric cancer patients with main portal vein tumor thrombus usually have a short survival time, owing to its aggressive behavior. Herein, we report a long-surviving case of gastric cancer with main portal vein tumor thrombus. A 78-year-old man presenting with anorexia and body weight loss was diagnosed with gastric cancer. The patient was referred to our hospital for further examination and treatment. Endoscopy revealed a type 3 tumor (8.0 cm in length) in the body of the stomach. Biopsy led to the diagnosis of moderately differentiated adenocarcinoma. Enhanced computed tomography revealed a large tumor thrombus extending from the gastric coronary vein to the portal trunk. A total gastrectomy with lymphadenectomy, splenectomy, and thrombectomy was performed. Postoperative chemotherapy with S-1 was administered for 18 months. The patient died a natural death without recurrence at 49 postoperative months. To the best of our knowledge, the patient was the oldest to be diagnosed with gastric cancer with main portal vein tumor thrombus at diagnosis, who survived >36 months. Although gastric cancer with main portal vein tumor thrombus is a rare occurrence, its prognosis is extremely poor. Intensive surgery and long-term chemotherapy may be effective at improving survival time in these patients.