[Rippling muscle disease with myasthenia gravis].

In February 2020, a 51-year-old woman experienced leg myalgia and noticed calf muscle movements that resembled a rippling wave while crouching down. In June 2020, she complained of bilateral arm myalgia. In August 2020, she developed left ptosis, had difficulty raising her bilateral arms, and developed diplopia and was admitted to our hospital. Anti-acetylcholine receptor antibodies turned out to be positive. We made a diagnosis of myasthenia gravis and acquired rippling muscle disease (RMD). Her myasthenia gravis symptoms and myalgia decreased with oral prednisolone. Contrast-enhanced computed tomography revealed thymoma. She underwent extended thymectomy and was discharged from the hospital. Her myalgia worsened, but it was responsive to methylprednisolone pulse therapy. CAV3 gene mutations are recognized as causes of congenial RMD whereas acquired RMD is associated with myasthenia gravis. Acquired RMD is rarely reported in Japan, but should be kept in mind as a condition treatable with immunotherapy.

Direct observation of electrically induced Pauli paramagnetism in single-layer graphene using ESR spectroscopy.

Graphene has been actively investigated as an electronic material owing to many excellent physical properties, such as high charge mobility and quantum Hall effect, due to the characteristics of a linear band structure and an ideal two-dimensional electron system. However, the correlations between the transport characteristics and the spin states of charge carriers or atomic vacancies in graphene have not yet been fully elucidated. Here, we show the spin states of single-layer graphene to clarify the correlations using electron spin resonance (ESR) spectroscopy as a function of accumulated charge density using transistor structures. Two different electrically induced ESR signals were observed. One is originated from a Fermi-degenerate two-dimensional electron system, demonstrating the first observation of electrically induced Pauli paramagnetism from a microscopic viewpoint, showing a clear contrast to no ESR observation of Pauli paramagnetism in carbon nanotubes (CNTs) due to a one-dimensional electron system. The other is originated from the electrically induced ambipolar spin vanishments due to atomic vacancies in graphene, showing a universal phenomenon for carbon materials including CNTs. The degenerate electron system with the ambipolar spin vanishments would contribute to high charge mobility due to the decrease in spin scatterings in graphene.

TAS-102 Safety in Metastatic Colorectal Cancer: Results From the First Postmarketing Surveillance Study.

BACKGROUND: Unexpected toxicities of newly approved drugs might be revealed in clinical practice after market launch. This postmarketing surveillance study investigated expected and unexpected adverse drug reactions (ADRs) of TAS-102 in clinical practice in the first 6 months after market launch. PATIENTS AND METHODS: All metastatic colorectal cancer (mCRC) patients (pts) received TAS-102 35 mg/m2 as a starting dose orally twice daily for 5 consecutive days, with 2 days of rest per week for 2 weeks, followed by a 14-day rest period. ADRs during treatment courses were reported spontaneously by attending physicians. RESULTS: From May 26 to November 25, 2014, 3420 mCRC pts were treated with TAS-102 at 1134 institutes in Japan. In total, 370 ADRs (185 ADRs of myelosuppression and related infection in 125 pts, of which 58 ADRs in 31 pts were serious ADRs [SADRs] and 127 ADRs in 98 pts were non-SADRs) were observed in 219 pts and included 89 SADRs in 51 pts and 281 non-SADRs in 183 pts (a pt was counted twice if SADR and non-SADR were experienced). The most frequent ADRs were: myelosuppression comprising neutropenia (n = 77), leukopenia (n = 28), thrombocytopenia (n = 23), anemia (n = 20), and febrile neutropenia (FN; n = 19). Serious neutropenia and FN tended to occur from days 15 to 21 in the first cycle in 12 (75%) of 16 pts. CONCLUSION: The ADRs and safety profile of TAS-102 in this study was similar to that in recent TAS-102 clinical trials, without unexpected safety signals. Careful monitoring should be undertaken in pts with serious neutropenia around day 15 in the first cycle of treatment to prevent FN.

Open pelvic ring fracture and multiple fractures of the lower extremities: a case report.

A 73-year-old female was hit by a car, and transferred to our hospital. On examination, her consciousness was alert, but her vital signs were unstable. There are three 10-cm open wounds on her right buttock. X-rays showed an unstable pelvic ring fracture, a right femoral shaft, a right proximal tibia and a right tibial plafond fractures. One hour after the injury, transarterial embolization (TAE) followed by external fixation (EF), and retroperitoneal pelvic packing (RPP) was performed. Two days and five days after the injury, thorough debridement of the open wounds was performed. The skin defect on the right buttock and the lower abdomen had enlarged to 40 x 35 cm, therefore, negative pressure wound therapy was applied. On the same day, right femur was fixed using a retrograde intramedullary nailing. 12 days after the injury, the proximal tibial fracture was fixed using a plate, and the tibial plafond fracture was fixed using screws and external fixators. 28 days after the injury, the split-thickness skin graft was performed on the right buttock and the lower abdomen. Seven months after the injury, the open wounds were completely healed without infection. She was able to walk smoothly with a T-cane. For the management of open pelvic ring fractures, it is essential to perform TAE, EF and RPP as soon as possible. Providing aggressive management, including thorough debridement, is mandatory to prevent severe infection and sepsis. We achieved a good clinical outcome by using a combination of TAE, EF, RPP and staged surgery, including thorough debridement.

Inhalation of 10% carbon dioxide rapidly terminates Scn1a mutation-related hyperthermia-induced seizures.

The aim of this study was to assess the anticonvulsant effect of carbon dioxide (CO2) on Scn1a mutation-related febrile seizures. We examined physiological changes in the blood gas levels after the induction of hyperthermia-induced seizures (HISs), which were associated with the Scn1a missense mutation. We determined the efficacy of inhalation of 5% or 10% CO2 to treat HISs. HISs were evoked in Scn1a mutant and wild-type (WT) rats by hot water baths. To determine the anticonvulsant effect of CO2 inhalation, rats were placed in a chamber filled with air or mixed gas containing 5% CO2 or 10% CO2 for 3 min, immediately after the induction of HISs. We also analyzed the blood gas levels at the end of inhalation of CO2. Hot water bathing induced a significant reduction in the partial pressure of CO2 (pCO2) and respiratory alkalosis in the WT and Scn1a mutant rats. HISs were evoked in 100% of the Scn1a mutant rats within 5 min, but in none of the WT rats. The Scn1a mutant rats demonstrated a higher HISs susceptibility associated with respiratory alkalosis than the WT rats. Inhalation of 10% CO2 shortened the seizure duration from 62.6±12.1 s to 15.5±1.0 s. Blood gas analysis after the inhalation of 10% CO2 demonstrated an elevated pCO2 level and respiratory acidosis. Inhalation of 10% CO2 demonstrated a potent and fast-acting anticonvulsant effect against HISs.

Clinical features of Vibrio vulnificus infections in the coastal areas of the Ariake Sea, Japan.

Vibrio vulnificus infection can result in necrotizing fasciitis and sepsis and is associated with high mortality. Most patients infected with this microbe have liver dysfunction as an underlying disease. However, because of the sporadic nature of outbreaks and unidentified cases, extensive evaluation of clinical features and identification of factors affecting prognosis have not been performed. We retrospectively analyzed 37 cases in Japan from 1984 to 2008 to review clinical features and to identify risk factors associated with prognosis. Statistical differences between clinical features (patient's characteristics, initial clinical laboratory data, symptoms upon admission, and other risk indicators) and prognosis were analyzed by use of the chi(2) test or the Mann-Whitney U test. Multivariate logistic regression analysis was also performed to assess factors which potentially affect hospital mortality. The mortality rate was 64.9%. An underlying liver disease was observed in 91.6% of the patients. The presence of liver cirrhosis tended to be related to hospital mortality; however, statistical significance was not achieved. Advanced age, lower platelet counts, and the presence of extensive skin lesions at onset affected outcomes with statistical significance. The prognosis of this disease is poor, because septic shock and necrotizing fasciitis often develop within a few days. Early diagnosis and treatment are needed to improve the prognosis of V. vulnificus infection.

Changes in circulating levels of calcitonin gene-related peptide and nitric oxide metabolites in septic patients during direct hemoperfusion with polymyxin B-immobilized fiber.

BACKGROUND/AIMS: This study evaluated the mechanism of hemodynamic improvement in polymyxin B-immobilized fiber (PMX) treatment. METHODS: Fifteen septic patients with endotoxemia and/or gram negative infection were treated with PMX. Plasma concentrations of calcitonin gene-related peptide (CGRP) and nitric oxide metabolites were measured. RESULTS: CGRP and nitric oxide metabolites before treatment were significantly higher than in healthy controls. CGRP had a significantly inverse correlation with diastolic blood pressure and a positive correlation with the Septic Severity Score and the number of failed organs. Systolic and diastolic blood pressure and systemic vascular resistance index increased significantly both immediately after and 24 h after PMX treatment. CGRP decreased significantly 24 h after the treatment. In contrast, there were no significant changes in nitric oxide metabolites. CONCLUSION: PMX treatment improved hemodynamic parameters in septic patients, and CGRP increased with increasing severity of sepsis. We conclude that a decrease in CGRP levels may be related to hemodynamic improvement resulting from PMX treatments.