Niemann-Pick type C disease - the tip of the iceberg? A review of neuropsychiatric presentation, diagnosis and treatment

Niemann-Pick type C (NP-C) disease is a rare neurodegenerative lysosomal storage disorder. It is highly heterogeneous, and there is limited awareness of a substantial subgroup that has an attenuated adolescent/adult-onset disease. In these patients psychiatric features, often a psychosis, may dominate the initial impression, although often there is an associated ataxia and cognitive impairment. Typically, patients experience a substantial diagnostic delay. In this review we highlight the importance of early recognition and discuss the pathophysiology, neuropsychiatric presentation and recent changes in the investigation and work-up of these patients, and treatment options.http://www.apa.org/pubs/journals/bulam2018Paediatrics and Child Healt

DNA electrophoresis studied with the cage model

The cage model for polymer reptation, proposed by Evans and Edwards, and its recent extension to model DNA electrophoresis, are studied by numerically exact computation of the drift velocities for polymers with a length L of up to 15 monomers. The computations show the Nernst-Einstein regime (v ~ E) followed by a regime where the velocity decreases exponentially with the applied electric field strength. In agreement with de Gennes' reptation arguments, we find that asymptotically for large polymers the diffusion coefficient D decreases quadratically with polymer length; for the cage model, the proportionality coefficient is DL^2=0.175(2). Additionally we find that the leading correction term for finite polymer lengths scales as N^{-1/2}, where N=L-1 is the number of bonds.Comment: LaTeX (cjour.cls), 15 pages, 6 figures, added correctness proof of kink representation approac

Levi-Civita Effect in the polarizable vacuum (PV) representation of general relativity

The polarizable vacuum (PV) representation of general relativity (GR), derived from a model by Dicke and related to the "TH-epsilon-mu" formalism used in comparative studies of gravitational theories, provides for a compact derivation of the Levi-Civita Effect (both magnetic and electric), herein demonstrated.Comment: 8 page

Tunneling and Metastability of continuous time Markov chains

We propose a new definition of metastability of Markov processes on countable state spaces. We obtain sufficient conditions for a sequence of processes to be metastable. In the reversible case these conditions are expressed in terms of the capacity and of the stationary measure of the metastable states

Gulf Stream remote forcing of shelfbreak currents in the Mid‐Atlantic Bight

The “Slope Sea” is a narrow band of ocean that lies between the Gulf Stream and the continental shelf edge in the Mid‐Atlantic Bight. It is composed primarily of slopewater, and a closed cyclonic gyre circulates within the upper few hundred meters of its western end. A two‐year time series of current measurements in this region has shown that the southwestward flowing shelfbreak currents along the shoreward flank of the gyre are directly related to the position of the Gulf Stream, which is typically some 150 to 300 km seaward of the shelfbreak. The mean monthly shelfbreak currents are strongest, towards the southwest at about 30–40 cm s−1, when the Stream is within 150 km of the shelf edge, whereas those currents are close to zero when the Stream is about 300 km away

Extraction of the πNN\pi NN coupling constant from NN scattering data

We reexamine Chew's method for extracting the $\pi NN$ coupling constant from np differential cross section measurements. Values for this coupling are extracted below 350 MeV, in the potential model region, and up to 1 GeV. The analyses to 1~GeV have utilized 55 data sets. We compare these results to those obtained via $\chi^2$ mapping techniques. We find that these two methods give consistent results which are in agreement with previous Nijmegen determinations.Comment: 12 pages of text plus 2 figures. Revtex file and postscript figures available via anonymous FTP at ftp://clsaid.phys.vt.edu/pub/n

Identity of the imaginary-time and real-time thermal propagators for scalar bound states in a one-generation Nambu-Jona-Lasinio model

By rigorous reanalysis of the results, we have proven that the propagators at finite temperature for scalar bound states in one-generation fermion condensate scheme of electroweak symmetry breaking are in fact identical in the imaginary-time and the real-time formalism. This dismisses the doubt about possible discrepancy between the two formalisms in this problem. Identity of the derived thermal transformation matrices of the real-time matrix propagators for scalar bound states without and with chemical potential and the ones for corresponding elementary scalar particles shows similarity of thermodynamic property between the two types of particles. Only one former inference is modified, i.e. when the two flavors of fermions have unequal nonzero masses, the amplitude of the composite Higgs particle will decay instead grow in time.Comment: 5 pages, revtex4, no figure