A case of atypical type of Sturge-Weber syndrome demonstrated reversible change by MRI FLAIR method in ictus and in postictal state]

We report a patient of atypical type of Sturge-Weber syndrome who demonstrated a reversible change by MRI FLAIR method in ictus and postictal state. A 5-year-old boy was admitted to our hospital because of severe headache, vomiting and loss of consciousness with his eyes conjugated to left for a few minutes. He had no facial nevus and other abnormal findings in physical examination. CT scan showed two small calcifications in the right occipital lobe. Postcontrast T 1-weighted image of MRI demonstrated a right parieto-occipital leptomeningeal enhancement. We diagnosed this case as an atypical type of Sturge-Weber syndrome. Although, on admission, FLAIR method showed the area of high signal intensity, after anticonvulsant therapy, those abnormal area disappeared. It is presumably detected by FLAIR method slight extravasation of plasma element in the surface of the brain due to regional hyperperfusion in ictus.     

Cardiovascular responses during laryngeal mask airway insertion in normotensive, hypertensive and chronic renal failure patients]

The hemodynamic response to the insertion of the laryngeal mask airway (LM) following induction with propofol 2 mg.kg-1 was assessed and compared in normotensive (Normal), hypertensive (HT) and chronic renal failure (CRF) patients (n = 23 in each group). Before induction, in HT and CRF groups blood pressure and rate pressure products (RPP) were higher than in Normal group (P < 0.05). Although blood pressure and RPP were decreased in every patient by induction with propofol, no patients needed vasopressor drugs. The decreases of blood pressure and RPP were larger in HT and CRF groups than in Normal group (P < 0.05). There were no differences between groups in heart rate and rate of successful LM insertion. We concluded that LM insertion with propofol 2 mg.kg-1 was an effective induction method preventing the adverse circulatory responses in normotensive, hypertensive and chronic renal failure patients.     

Retroperitoneal venous hemangioma

Retroperitoneal venous hemangioma is a very rare condition. Only two cases have been reported in the English literature. We report the case of a 28-yr-old man with a retroperitoneal venous hemangioma who underwent surgical resection. Preoperative magnetic resonance imaging revealed a retroperitoneal tumor with low signal intensity on the T₁-weighted image and high signal intensity on the T₂-weighted image, typical of hemangiomas. In addition, it showed a partial interruption of the muscle layer surrounding the tumor. Intraoperative findings showed a large, reddish brown retroperitoneal mass with an ill defined capsule firmly attached to the muscular tissue surrounding the tumor. Retroperitoneal hemangioma is rare and is almost always of the cavernous type. Although accurate pre- and intraoperative diagnosis of retroperitoneal venous hemangiomas is difficult, magnetic resonance imaging and the presence of a firm attachment to the muscles may be helpful for the important diagnostic findings of this disease.     

Candesartan prevents arteriopathy progression in cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy model

Cerebral small vessel disease (CSVD) causes dementia and gait disturbance due to arteriopathy. Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is a hereditary form of CSVD caused by loss of high-temperature requirement A1 (HTRA1) serine protease activity. In CARASIL, arteriopathy causes intimal thickening, smooth muscle cell (SMC) degeneration, elastic lamina splitting, and vasodilation. The molecular mechanisms were proposed to involve the accumulation of matrisome proteins as substrates or abnormalities in transforming growth factor β (TGF-β) signaling. Here, we show that HTRA1^−/− mice exhibited features of CARASIL-associated arteriopathy: intimal thickening, abnormal elastic lamina, and vasodilation. In addition, the mice exhibited reduced distensibility of the cerebral arteries and blood flow in the cerebral cortex. In the thickened intima, matrisome proteins, including the hub protein fibronectin (FN) and latent TGF-β binding protein 4 (LTBP-4), which are substrates of HTRA1, accumulated. Candesartan treatment alleviated matrisome protein accumulation and normalized the vascular distensibility and cerebral blood flow. Furthermore, candesartan reduced the mRNA expression of Fn1, Ltbp-4, and Adamtsl2, which are involved in forming the extracellular matrix network. Our results indicate that these accumulated matrisome proteins may be potential therapeutic targets for arteriopathy in CARASIL.     

Subvalvular tissue mimicking valve detachment-like pathology by vertical aneurysm in Takayasu’s arteritis

A 74-year-old woman with Takayasu’s arteritis previously underwent aortic valve replacement at 59 years old. She was initially diagnosed with aortic valve stenosis and valve detachment. Moreover, preoperative computed tomography revealed ∼40 mm distance between the coronary artery ostium and the prosthetic valve (PV) and an aneurysm at the sinus of Valsalva. A Bentall procedure was subsequently performed. Intraoperative findings revealed no detachment of the PV. Following PV removal, the subvalvular tissue was noted to protrude into the left ventricular outflow tract. Subsequently, it was revealed that the tissue could have interfered with the PV; however, the PV appeared to have been detached considering the imaging findings.     

Association of serum creatinine-to-cystatin C ratio with skeletal muscle mass and strength in nonalcoholic fatty liver disease in the Iwaki Health Promotion Project

We evaluated the feasibility of using serum creatinine-to-cystatin C ratio in the assessments of muscle mass and strength in nonalcoholic fatty liver disease. In a community-based cross-sectional study, skeletal muscle mass and handgrip strength were assessed in 641 Japanese adults. Low skeletal muscle mass index and low handgrip strength were defined as indicated in the sarcopenia diagnostic criteria of the Japan Society of Hepatology. Nonalcoholic fatty liver disease was defined as fatty liver on ultrasonography in the absence of other causes of steatosis. The creatinine-to-cystatin C ratio was useful for identifying the participants with low skeletal muscle mass index, with an area under the receiver-operating characteristic curve of 0.84 [95% confidence interval (CI), 0.77–0.91] in men and 0.72 in women (95% CI, 0.65–0.78), and those with low handgrip strength, with an area under the receiver-operating characteristic curve of 0.96 (95% CI, 0.93–0.99) in men and 0.79 (95% CI, 0.66–0.92) in women. Moreover, the creatinine-to-cystatin C ratio correlated with skeletal muscle mass index (r = 0.511, p<0.001) and handgrip strength (r = 0.657, p<0.001), whereas it did not correlate with exacerbation of hepatic steatosis. In this study, creatinine-to-cystatin C ratio correlated with muscle mass and strength in nonalcoholic fatty liver disease regardless of hepatic steatosis.     

Multiple Gastric Metastases after Distal Pancreatectomy for Pancreatic Cancer

A 67-year-old woman underwent distal pancreatectomy for pancreatic cancer. Recurrence in the form of lung metastasis was discovered eight months after surgery, and chemotherapy was initiated. Two years after the surgery, she was admitted for the evaluation of melena. Esophagogastroduodenoscopy revealed multiple subepithelial lesions with ulceration from the gastric body to the fornix. The histopathology of biopsy specimens was consistent with ductal adenocarcinoma, which appeared similar to the resected pancreatic cancer. The patient was diagnosed with multiple gastric metastases of pancreatic cancer. We herein report a case of pancreatic cancer with multiple gastric metastases that occurred after surgery for pancreatic tail cancer.     

gamma-Tocopherol Accelerated Sodium Excretion in a Dose-Dependent Manner in Rats with a High Sodium Intake

We have previously reported that gamma-tocopherol (gamma-Toc) displays a natriuretic potency in rats fed a NaCl diet and administered 20 mg gamma-Toc. In this study, we investigated whether gamma-Toc has natriuretic potency at a dose lower or higher than 20 mg in rats given a NaCl diet. Male rats were fed a control diet or a NaCl diet and administered either placebo or 10, 20 or 40 mg of gamma-Toc. The rat urine was collected for 24 hours (divided into 6 hour periods) and the 2,7,8-trimethyl-2-(2'-carboxyethyl)-6-hydroxychroman (gamma-CEHC) level, the sodium excretion content, and the urine volume were determined. The 24-hour gamma-CEHC and sodium levels in the urine of the NaCl groups given 20 mg or 40 mg gamma-Toc were significantly higher than those in the placebo group. The peak levels of urine sodium and gamma-CEHC in the NaCl group given 40 mg gamma-Toc appeared at 0-6 h, which was a more rapid increase than that seen in the group given 20 mg gamma-Toc. The 24-hour urine volumes of the NaCl groups given 10 and 20 mg gamma-Toc were significantly higher than the urine volume of the placebo group. Our findings suggested that gamma-Toc increased sodium excretion in a dose-dependent manner in rats fed a NaCl diet. Moreover, a high dose of gamma-Toc may accelerate its metabolism and cause an increase in the rate of sodium excretion.     

Effects of the pregnant uterus on the extradural venous plexus in the supine and lateral positions, as determined by magnetic resonance imaging

To clarify the effects of the pregnant uterus on the extradural venous plexus in the supine and lateral positions, we studied magnetic resonance (MR) images of the lumbar spine in three parturients. T2- weighted axial MR images were obtained with the parturient in the supine and lateral positions. On each slice level in the same subject, the MR images were compared with the control MR images obtained before pregnancy. When the parturient lay supine, the pregnant uterus compressed the inferior vena cava and almost totally obstructed it; the extradural venous plexus was engorged. On turning the parturient into the lateral position, the inferior vena cava was free from compression, and the engorged extradural venous plexus was found to shrink to the level of the non-pregnant state.