Multiple cerebellar infarction due to vertebral artery obstruction and bulbar symptoms associated with vertical subluxation and atlanto-occipital subluxation in ankylosing spondylitis

Ankylosing spondylitis (AS) results in disease-specific inflammation at the site of ligamentous insertion into the bone. Atlantoaxial joint subluxation and vertical subluxation of the axis may occur as a consequence of instability resulting from the inflammatory process. Spontaneous anterior atlantoaxial subluxation is a well recognized complication in about 2% of patients with AS, and presents with or without signs of spinal cord compression. Vertical subluxation may follow anterior or posterior subluxation. It was noted in 3-8% of patients with rheumatoid arthritis, but is an exceedingly rare complication of AS. Moreover, it has never been reported that multiple cerebellar infarction and bulbar symptoms developed spontaneously due to atlanto-occipital subluxation and vertical subluxation in a patient with a long [corrected] history of AS. We describe a man with AS who developed multiple cerebellar infarction due to vertebral artery obstruction and bulbar symptoms associated with atlanto-occipital subluxation and vertical subluxation.     

Refining Genotypes and Phenotypes in KCNA2-Related Neurological Disorders

Pathogenic variants in KCNA2, encoding for the voltage-gated potassium channel K_v1.2, have been identified as the cause for an evolving spectrum of neurological disorders. Affected individuals show early-onset developmental and epileptic encephalopathy, intellectual disability, and movement disorders resulting from cerebellar dysfunction. In addition, individuals with a milder course of epilepsy, complicated hereditary spastic paraplegia, and episodic ataxia have been reported. By analyzing phenotypic, functional, and genetic data from published reports and novel cases, we refine and further delineate phenotypic as well as functional subgroups of KCNA2-associated disorders. Carriers of variants, leading to complex and mixed channel dysfunction that are associated with a gain- and loss-of-potassium conductance, more often show early developmental abnormalities and an earlier onset of epilepsy compared to individuals with variants resulting in loss- or gain-of-function. We describe seven additional individuals harboring three known and the novel KCNA2 variants p.(Pro407Ala) and p.(Tyr417Cys). The location of variants reported here highlights the importance of the proline(405)–valine(406)–proline(407) (PVP) motif in transmembrane domain S6 as a mutational hotspot. A novel case of self-limited infantile seizures suggests a continuous clinical spectrum of KCNA2-related disorders. Our study provides further insights into the clinical spectrum, genotype–phenotype correlation, variability, and predicted functional impact of KCNA2 variants.     

Solubility of Si3N4 in Liquid SiO2

The nitrogen solubility in the SiO₂-rich liquid in the metastable binary SiO₂-Si₃N₄ system has been determined by analytical TEM to be 1%–4% of N/(O + N) at 1973–2223 K. Analysis of the near edge structure of the electron energy loss peak indicates that nitrogen is incorporated into the silicate network rather than being present as molecular N₂. A regular solution model with a positive enthalpy of mixing for the liquid was used to match the data for the metastable solubility of N in the presence of crystalline Si₃N₄ and to adjust the computed phase diagram. The solubility of Si₃N₄ in fused SiO₂ is far less than reported in liquid silicates also containing Al, Mg, and/or Y. Apparently, these cations act as modifiers that break anion bridges in the silicate network and, thereby, allow further incorporation of Si₃N₄ without prohibitive amounts of network cross-linking. Finally, indications emerged regarding the diffuse nature of the Si₃N₄-SiO₂ interface that leads to amorphous regions of higher N content.     

High-Throughput Experimentation in Oxidation Catalysis

High-throughput experimentation in catalysis comprises the following components: (i) automated high-throughput synthesis, (ii) testing in Stage I and Stage II, for which to some extent novel assays are necessary, (iii) data handling and experimental design tools, and (iv) robotics. This contribution covers these topics, using examples from the research of the authors, but also from the literature, in order to illustrate the problems and opportunities associated with high-throughput experimentation in catalysis, focusing particularly on heterogeneous catalysis.     

Silphinene Sesquiterpenes as Model Insect Antifeedants

Silphinene sesquiterpenes are established chrysomelid antifeedants. In this work, nine silphinene analogs, 11-acetoxy-5-angeloyloxysilphinen-3-one (1), 11-acetoxy-5-tigloyloxysilphinen-3-one (2), 11-acetoxy-5-iso- butyryloxysilphinen-3-one (3), 11-hydroxy-5-angeloyloxysilphinen-3-one (4), 11,5-dihydroxysilphinen-3-one (5), 11,5-diacetoxysilphinen-3-one (6), 5,11-diisobutyryloxysilphinen-3-one (7), silphinen-3,5,11-trione (8), and O-methyl-5-epicantabrenolic acid methyl ester (10), and a presilphiperfolane sesquiterpene (9) were tested against several divergent insect species, including the lepidopteran Spodoptera littoralis, the chrysomelid Leptinotarsa decemlineata, and five aphid species, and their antifeedant effects were compared with those of picrotoxinin, a GABA-antagonist, and thymol, an allosteric modulator for insect GABA receptors. All insects tested responded to at least one silphinene analog and/or GABA antagonist. Compound 3 and thymol were effective antifeedants against all species tested except S. littoralis, with varying potencies according to their feeding ecologies. The toxicity of these compounds was species-dependent and did not correlate with their antifeedant effect.     

Practical Significance,Theory and Determination of Compatibility of Dyes on Synthetic-polymer Fibres

The compatibility of dyes is particularly important in regard to the time, reproducibility and levelness of dyeing. For basic dyes on acrylic fibres and anionic dyes on nylon, a characteristic of the dye—the compatibility value K— is valuable in determining whether it can be used in the same bath as others. Dyes having the same K-value are compatible under most dyeing conditions. For disperse dyes, K-values do not exist. In selecting such dyes for use together, other properties of the dyes and the concentrations to be used in the particular combination must be considered.