CSF lactate dehydrogenase activity in patients with Creutzfeldt-Jakob disease exceeds that in other dementias

The diagnosis of Creutzfeldt- Jakob disease (CJD) is still made by exclusion of other dementias. We now evaluated lactate dehydrogenase (LDH) in the cerebrospinal fluid (CSF) as a possible additional diagnostic tool. CSF LDH levels of patients with CJD ( n = 26) were compared with those in other dementias ( n = 28). LDH isoenzymes were determined in a subset ( n = 9). Total LDH and isoenzyme LDH-1 were significantly higher, whereas the fractions of LDH-2 and LDH-3 were significantly lower in CJD patients. We conclude that in addition to established CSF parameters, LDH and its isoenzymes might serve as a further help to discriminate between CJD and other dementias. Copyright (C) 2004 S. Karger AG, Basel

SPG10 is a rare cause of spastic paraplegia in European families

Background: SPG10 is an autosomal dominant form of hereditary spastic paraplegia (HSP), which is caused by mutations in the neural kinesin heavy chain KIF5A gene, the neuronal motor of fast anterograde axonal transport. Only four mutations have been identified to date.Objective: To determine the frequency of SPG10 in European families with HSP and to specify the SPG10 phenotype.Patients and methods: 80 index patients from families with autosomal dominant HSP were investigated for SPG10 mutations by direct sequencing of the KIF5A motor domain. Additionally, the whole gene was sequenced in 20 of these families.Results: Three novel KIF5A mutations were detected in German families, including one missense mutation (c.759G>T, p.K253N), one in frame deletion (c.768_770delCAA, p.N256del) and one splice site mutation (c.217G>A). Onset of gait disturbance varied from infancy to 30 years of age. All patients presented clinically with pure HSP, but a subclinical sensory--motor neuropathy was detected by neurophysiology studies.Conclusions: SPG10 accounts for approximately 3% of European autosomal dominant HSP families. All mutations affect the motor domain of kinesin and thus most likely impair axonal transport. Clinically, SPG10 is characterised by spastic paraplegia with mostly subclinical peripheral neuropathy

Field induced phase transition in the few photon regime

Some features of the field induced phase transition accompanied by the vacuum creation of an electron-positron plasma (EPP) in strong time-dependent electric fields have been discussed in the work [1] in the domain of the tunneling mechanism ($\omega \ll m$, where $\omega$ is the characteristic frequency of the external field and $m$ is the electron mass). In the present contribution the features of the this process will be considered in the few photon domain where $\omega \sim m$. We observe a narrowing of the transient domain of the fast oscillations and, mainly, a considerable growth of the effectiveness of the EPP production. Under these circumstances, we see an increase of the effectiveness of the EPP creation in the particular case of a bifrequent excitation, where both mechanisms (tunneling and few photon) act simultaneously [2,3].Comment: 6 pages, 4 figures, contribution to the Proceedings of the XXIII International Baldin Seminar on "High Energy Physics Problems", Dubna, Russia, September 19-24, 201

Automatic structures for semigroup constructions

We survey results concerning automatic structures for semigroup constructions, providing references and describing the corresponding automatic structures. The constructions we consider are: free products, direct products, Rees matrix semigroups, Bruck-Reilly extensions and wreath products.Comment: 22 page

Capture probability of a survey trawl for red king crab (Paralithodes camtschaticus)

The relative abundance of Bristol Bay red king crab (Paralithodes camtschaticus) is estimated each year for stock assessment by using catch-per-swept-area data collected on the Alaska Fisheries Science Center’s annual eastern Bering Sea bottom trawl survey. To estimate survey trawl capture efficiency for red king crab, an experiment was conducted with an auxiliary net (fitted with its own heavy chain-link footrope) that was attached beneath the trawl to capture crabs escaping under the survey trawl footrope. Capture probability was then estimated by fitting a model to the proportion of crabs captured and crab size data. For males, mean capture probability was 72% at 95 mm (carapace length), the size at which full vulnerability to the survey trawl is assigned in the current management model; 84.1% at 135 mm, the legal size for the fishery; and 93% at 184 mm, the maximum size observed in this study. For females, mean capture probability was 70% at 90 mm, the size at which full vulnerability to the survey trawl is assigned in the current management model, and 77% at 162 mm, the maximum size observed in this study. The precision of our estimates for each sex decreased for juveniles under 60 mm and for the largest crab because of small sample sizes. In situ data collected from trawl-mounted video cameras were used to determine the importance of various factors associated with the capture of individual crabs. Capture probability was significantly higher when a crab was standing when struck by the footrope, rather than crouching, and higher when a crab was hit along its body axis, rather than from the side. Capture probability also increased as a function of increasing crab size but decreased with increasing footrope distance from the bottom and when artificial light was provided for the video camera

Hemodynamic progression of aortic stenosis in adults assessed by doppler echocardiography

AbstractDoppler echocardiography was used to follow the hemodynamic severity of aortic stenosis. First, the reproducibility of repeat recordings (mean interval 28 ± 36 days) of aortic jet velocity, made by two independent observers, was tested in 38 adults with aortic stenosis and unchanged clinical status. The two recordings of maximal velocity correlated well (r = 0.96, y = 0.88x + 0.46m/s, SEE = 0.21 m/s) with a mean coefficient of variation of 3.2%. Repeat recording of left ventricular outflow tract velocity by two independent observers in 10 other patients with aortic stenosis also correlated well (r = 0.94, y = 1.06x + 0.0 m/s, SEE = 0.06 m/s) with a mean coefficient of variation of 4.6%.Next, Doppler echocardiography was used to study 42 patients with aortic stenosis (mean age 66 years) over a follow-up interval of 6 to 43 months (mean 20). Maximal aortic jet velocity increased by 0.36 m/s per year (range −0.3 to +1.0 m/s per year). Mean transaortic pressure gradient changed by −7 to +23 (mean 8) mm Hg/year. Aortic valve area by the continuity equation (n = 25) decreased by 0 to 0.5 cm2/year (mean decrease 0.1 cm2/year). year patients had a worsening of stenosis (decrease in valve area) even though they had no change or a decrease in pressure gradient, because of concurrent decreases in transaortic volume flow.Twenty-one patients (50%) developed new or progressive symptoms of aortic stenosis necessitating valve replacement. These patients had a higher maximal aortic jet velocity at follow-up (4.5 versus 3.9 m/s, p < 0.01) and a greater rate of increase in mean pressure gradient (15 versus 7 mm Hg/year, p < 0.01) than did those who remained asymptomatic; however, there were no significant differences in age, follow-up interval or maximal aortic jet velocity at entry.It is concluded that Doppler echocardiographic measures of aortic stenosis severity are reproducible. The rate of change of transaortic pressure gradient varies among patients and the gradient may not increase even when stenosis severity worsens. Although stenosis severity progresses more rapidly in patients who develop symptoms requiring valve replacement, these patients cannot be identified at the initial study

Local Equation of State and Velocity Distributions of a Driven Granular Gas

We present event-driven simulations of a granular gas of inelastic hard disks with incomplete normal restitution in two dimensions between vibrating walls (without gravity). We measure hydrodynamic quantities such as the stress tensor, density and temperature profiles, as well as velocity distributions. Relating the local pressure to the local temperature and local density, we construct a local constitutive equation. For strong inelasticities the local constitutive relation depends on global system parameters, like the volume fraction and the aspect ratio. For moderate inelasticities the constitutive relation is approximately independent of the system parameters and can hence be regarded as a local equation of state, even though the system is highly inhomogeneous with heterogeneous temperature and density profiles arising as a consequence of the energy injection. Concerning the local velocity distributions we find that they do not scale with the square root of the local granular temperature. Moreover the high-velocity tails are different for the distribution of the x- and the y-component of the velocity, and even depend on the position in the sample, the global volume fraction, and the coefficient of restitution.Comment: 14 pages, 14 figures of which Figs. 13a-f and Fig. 14 are archived as separate .gif files due to upload-size limitations. A version of the paper including all figures in better quality can be downloaded at http://www.theorie.physik.uni-goettingen.de/~herbst/download/LocEqSt.ps.gz (3.8 MB, ps.gz) or at http://www.theorie.physik.uni-goettingen.de/~herbst/download/LocEqSt.pdf (4.9 MB, pdf