Treatment with flunitrazepam of continuous spikes and waves during slow wave sleep (CSWS) in children

SummaryWe describe our treatment of two boys with continuous spikes and waves during slow wave sleep (CSWS). One of the boys was suffering from non-convulsive status epilepticus and the other from conscious disturbance with automatism. Their ictal EEG readings showed continuous diffuse spike and wave complexes, which were considered to show electrical status. The boys were diagnosed as having CSWS, and were later diagnosed with Landau–Kleffner syndrome (LKS). EEG readings returned to normal on intravenous injection of flunitazepam (FZP) at a dose of 0.02mg/kg, suggesting that FZP is an effective treatment for CSWS

Therapeutic potential of clinical-grade human induced pluripotent stem cell-derived cardiac tissues

Objectives: To establish a protocol to prepare and transplant clinical-grade human induced pluripotent stem cell (hiPSC)-derived cardiac tissues (HiCTs) and to evaluate the therapeutic potential in an animal myocardial infarction (MI) model. Methods: We simultaneously differentiated clinical-grade hiPSCs into cardiovascular cell lineages with or without the administration of canonical Wnt inhibitors, generated 5- layer cell sheets with insertion of gelatin hydrogel microspheres (GHMs) (HiCTs), and transplanted them onto an athymic rat MI model. Cardiac function was evaluated by echocardiography and cardiac magnetic resonance imaging and compared with that in animals with sham and transplantation of 5-layer cell sheets without GHMs. Graft survival, ventricular remodeling, and neovascularization were evaluated histopathologically. Results: The administration of Wnt inhibitors significantly promoted cardiomyocyte (CM) (P < .0001) and vascular endothelial cell (EC) (P = .006) induction, which resulted in cellular components of 52.0 ± 6.1% CMs and 9.9 ± 3.0% ECs. Functional analyses revealed the significantly lowest left ventricular end-diastolic volume and highest ejection fraction in the HiCT group. Histopathologic evaluation revealed that the HiCT group had a significantly larger median engrafted area (4 weeks, GHM(-) vs HiCT: 0.4 [range, 0.2-0.7] mm² vs 2.2 [range, 1.8-3.1] mm²; P = .005; 12 weeks, 0 [range, 0-0.2] mm² vs 1.9 [range, 0.1-3.2] mm2; P = .026), accompanied by the smallest scar area and highest vascular density at the MI border zone. Conclusions: Transplantation of HiCTs generated from clinical-grade hiPSCs exhibited a prominent therapeutic potential in a rat MI model and may provide a promising therapeutic strategy in cardiac regenerative medicine

One-Directional Antenna Systems: Energy Transfer from Monomers to JAggregates within 1D Nanoporous Aluminophosphates

A cyanine dye (PIC) was occluded into two 1D-nanopoporus Mg-containing aluminophosphates with different pore size (MgAPO-5 and MgAPO-36 with AFI and ATS zeolitic structure types, with cylindrical channels of 7.3 Å diameter and elliptical channels of 6.7 Å × 7.5 Å, respectively) by crystallization inclusion method. Different J-aggregates are photophysically characterized as a consequence of the different pore size of the MgAPO frameworks, with emission bands at 565 nm and at 610 nm in MgAPO-5 and MgAPO-36, respectively. Computational results indicate a more linear geometry of the J-aggregates inside the nanochannels of the MgAPO-36 sample than those in MgAPO-5, which is as a consequence of the more constrained environment in the former. For the same reason, the fluorescence of the PIC monomers at 550 nm is also activated within the MgAPO-36 channels. Owing to the strategic distribution of the fluorescent PIC species in MgAPO-36 crystals (monomers at one edge and J-aggregates with intriguing emission properties at the other edge) an efficient and one-directional antenna system is obtained. The unidirectional energy transfer process from monomers to J-aggregates is demonstrated by remote excitation experiments along tens of microns of distance.Financial support from Gobierno Vasco (IT912-16) and Ministerio de Economía y Competitividad “MINECO” (through Projects MAT2014-51937-C3-3-P, MAT2016-77496-R and MAT-2015-65767-P) is acknowledged. R.S.L. and V.M.M. acknowledge niversidad del PaísVasco (UPV-EHU) for a postdoctoral fellowship and MINECO for a “Ramón y Cajal” Contract RYC-2011-09505), respectively. H.U. gratefully acknowledges the financial support of the European Research Council (#280064), the FWO (G056314N, G0B5514N, G081916N), and JSPS KAKENHI (JP17H03003, JP17H05244, JP17H05458). Centro Técnico de Informática (CSIC) is acknowledged for running the calculations and Accelrys for providing the computational softwar

Lattice instability and elastic dispersion due to the rattling motion in the type-I clathrate Ba_8Ga_<16>Sn_<30>

To investigate the off-center rattling motion and its charge-carrier dependence in type-I clathrate compounds, we carried out ultrasonic measurements on type-I Ba8Ga16Sn30 and a reference compound, K8Ga8Sn38. We found elastic softening of C44 originating from a lattice instability due to the off-center rattling motion of Ba atom in Ba8Ga16Sn30. Elastic softening below 1 K suggests that the lattice instability remains at very low temperatures. We also found ultrasonic dispersion which has no mode selectivity. No-mode-selective ultrasonic dispersion in Ba8Ga16Sn30 would be caused by a strong electron-phonon coupling. No charge-carrier dependence is observed between n-type and p-type Ba8Ga16Sn30. The significant softening on the bulk modulus in Ba8Ga16Sn30 contrasts to the continuous hardening in K8Ga8Sn38, indicating the central role of the rattling motion in the softening

シンケイコン ショウガイ デ ハッショウシタ シンケイ サルコイドーシス ノ イチレイ

A５２-year-old woman was referred to our hospital for further examination of thoracolumbar pain. As dysesthesia at Th４level was seen in neurological examination, thoracic radiculopathy or myelopathy was suspected. Blood examination showed elevated level of serum ACE and lysozyme. Lymphadenopathy was evident in bilateral hila and mediastina with marked FDG and Gallium accumulation in FDG-PET-CT and Gallium scintigraphy, respectively. The number of lymphocytes and the CD４／CD８ratio were increased in the BALF. Histological findings of specimens obtained from the lung and the skin lesion revealed noncaseating epithelioid granuloma, which yielded the diagnosis of sarcoidosis. The cerebrospinal fluid examinations showed elevated level of cell counts, proteins and β２-microglobulin. Taken together, she was diagnosed as neurosarcoidosis with thoracic radiculopathy. Her symptoms were improved with oral administration of prednisolone, but they were exacerbated when prednisolone dose was tapered to２０mg／day. Combined therapy of methotrexate and prednisolone was initiated, thereafter her symptoms disappeared completely