Episodic neurologic disorders: syndromes, genes, and mechanisms.

Many neurologic diseases cause discrete episodic impairment in contrast with progressive deterioration. The symptoms of these episodic disorders exhibit striking variety. Herein we review what is known of the phenotypes, genetics, and pathophysiology of episodic neurologic disorders. Of these, most are genetically complex, with unknown or polygenic inheritance. In contrast, a fascinating panoply of episodic disorders exhibit Mendelian inheritance. We classify episodic Mendelian disorders according to the primary neuroanatomical location affected: skeletal muscle, cardiac muscle, neuromuscular junction, peripheral nerve, or central nervous system (CNS). Most known Mendelian mutations alter genes that encode membrane-bound ion channels. These mutations cause ion channel dysfunction, which ultimately leads to altered membrane excitability as manifested by episodic disease. Other Mendelian disease genes encode proteins essential for ion channel trafficking or stability. These observations have cemented the channelopathy paradigm, in which episodic disorders are conceptualized as disorders of ion channels. However, we expand on this paradigm to propose that dysfunction at the synaptic and neuronal circuit levels may underlie some episodic neurologic entities

Addressing effective construction logistics through the lens of vehicle movements

Purpose – Construction logistics is an essential part of construction supply chain management (CSCM). However, limited attention has been paid to this issue in the New Zealand construction industry. The purpose of this paper is to contribute to the knowledge about what hampers efficiency in transporting construction materials and plants to a construction site. The intention is to gain detailed understanding of the practice and obstacles in efficient construction logistics and thus identify interventions to improve logistics efficiency, especially using the numbers of vehicle movements to the construction site as an indicator. Design/methodology/approach – A case study approach was adopted with on-site observations and interviews. Observations were performed during constructions on-site from the start of construction to “hand-over” to the building owner. A selection of construction suppliers and subcontractors involved in the studied project were interviewed. Findings – Data analysis suggested that cost-related factors affecting the construction logistics, both monetary and non-monetary factors were not measured and largely ignored, especially the possible environmental and/or social impact occurred by the truck movement. Factors in the service-related sector were insufficiently managed in the observed site. The main contribution to inefficient construction logistics are related to understanding and implementing CSCM. It is noticed that there is inadequate awareness of CSCM and logistics efficiency largely due to lack of commitment from the management level and skills at the operational level. Originality/value – Significant intrinsic and extrinsic interventions necessary to enhance construction logistics were acknowledged from the data analysis. These include both qualitative and quantitative data. These intrinsic and extrinsic interventions, such as implementing appropriate logistics tools that suits individual site and introducing traffic management costs, offer plausible explanations regarding how to improve the efficiency in construction logistics through optimising transportation movements to the construction site

Phase diagram as a function of doping level and pressure in Eu1−x_{1-x}Lax_xFe2_2As2_2 system

We establish the phase diagram of Eu$_{1-x}$La$_x$Fe$_2$As$_2$ system as a function of doping level x and the pressure by measuring the resistivity and magnetic susceptibility. The pressure can suppress the spin density wave (SDW) and structural transition very efficiently, while enhance the antiferromagnetic (AFM) transition temperature T$_N$ of Eu$^{2+}$. The superconductivity coexists with SDW order at the low pressure, while always coexists with the Eu$^{2+}$ AFM order. The results suggests that Eu$^{2+}$ spin dynamics is disentangeld with superconducting (SC) pairing taken place in the two-dimensional \emph{Fe-As} plane, but it can strongly affect superconducting coherence along c-axis

Strange nonchaotic attractors in noise driven systems

Strange nonchaotic attractors (SNAs) in noise driven systems are investigated. Before the transition to chaos, due to the effect of noise, a typical trajectory will wander between the periodic attractor and its nearby chaotic saddle in an intermittent way, forms a strange attractor gradually. The existence of SNAs is confirmed by simulation results of various critera both in map and continuous systems. Dimension transition is found and intermittent behavior is studied by peoperties of local Lyapunov exponent. The universality and generalization of this kind of SNAs are discussed and common features are concluded