The National ALS Registry: a recruitment tool for research.

Introduction: Subject recruitment is critical for understanding fatal diseases like ALS, however linking patients with researchers can be challenging. The U.S.

Clinical complications of urinary catheters caused by crystalline biofilms: something needs to be done.

This review is largely based on a previous paper published in the journal Spinal Cord. The care of many patients undergoing long-term bladder catheterization is complicated by encrustation and blockage of their Foley catheters. This problem stems from infection by urease-producing bacteria, particularly Proteus mirabilis.

Parental attitudes toward newborn screening for Duchenne/Becker muscular dystrophy and spinal muscular atrophy.

Introduction: Disease inclusion in the newborn screening (NBS) panel should consider the opinions of those most affected by the outcome of screening. We assessed the level and factors that affect parent attitudes regarding NBS panel inclusion of Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), and spinal muscular atrophy (SMA).

Methods: The attitudes toward NBS for DMD, BMD, and SMA were surveyed and compared for 2 categories of parents, those with children affected with DMD, BMD, or SMA and expectant parents unselected for known family medical history.

Biocide activity against urinary catheter pathogens.

Antimicrobial effects of essential oils against bacteria associated with urinary catheter infection was assessed. Tests were performed on 14 different bacterial species cultured either planktonically or as biofilms. Biofilms were found to be up to 8-fold more tolerant of the test agents.

Environmental and occupational risk factors for amyotrophic lateral sclerosis: a case-control study.

Background/aims: Environmental and occupational exposures are implicated as risk factors for amyotrophic lateral sclerosis (ALS), the etiology of which is largely unknown, although no causal relationships have been established.

Objective: The aim of the study was to evaluate the associations of personal risk factors and self-reported environmental and occupational exposures with risk of ALS.

Methods: The cases involved ALS patients (n = 66) identified from major neurological centers in Pittsburgh and Philadelphia, Pa.

Facial diplegia, pharyngeal paralysis, and ophthalmoplegia after a timber rattlesnake envenomation.

The timber rattlesnake, also known as Crotalus horridus, is well known to cause significant injury from toxins stored within its venom. During envenomation, toxic systemic effects immediately begin to cause damage to many organ systems including cardiovascular, hematologic, musculoskeletal, respiratory, and neurologic. One defining characteristic of the timber rattlesnake is a specific neurotoxin called crotoxin, or the "canebrake toxin," which is a potent β-neurotoxin affecting presynaptic nerves that can cause paralysis by inhibiting appropriate neuromuscular transmission.

Endstation for ultrafast magnetic scattering experiments at the free-electron laser in Hamburg.

An endstation for pump-probe small-angle X-ray scattering (SAXS) experiments at the free-electron laser in Hamburg (FLASH) is presented. The endstation houses a solid-state absorber, optical incoupling for pump-probe experiments, time zero measurement, sample chamber, and detection unit. It can be used at all FLASH beamlines in the whole photon energy range offered by FLASH.

Ultrafast optical demagnetization manipulates nanoscale spin structure in domain walls.

During ultrafast demagnetization of a magnetically ordered solid, angular momentum has to be transferred between the spins, electrons, and phonons in the system on femto- and picosecond timescales. Although the intrinsic spin-transfer mechanisms are intensely debated, additional extrinsic mechanisms arising due to nanoscale heterogeneity have only recently entered the discussion. Here we use femtosecond X-ray pulses from a free-electron laser to study thin film samples with magnetic domain patterns.

An indwelling urinary catheter for the 21st century.

What's known on the subject? and What does the study add? A vast literature has been published on the prevalence, morbidity and microbiology of catheter-associated urinary tract infections. Research and development in recent years has focused on producing antibacterial coatings for the indwelling Foley catheter with insufficient attention to its design. This article provides a critical examination of the design of the indwelling Foley catheter.

Validity of hospital discharge data for identifying cases of amyotrophic lateral sclerosis.

Inpatient hospital encounters and emergency department visits were examined to identify cases of amyotrophic lateral sclerosis (ALS).The ninth edition of the International Classification of Disease, clinical modification (ICD-9-CM) for ALS was confirmed for ALS was confirmed in 93% of inpatient discharges and in 91% of emergency department visits by the diagnostic standard (chart review). Yearly prevalence rates ranged from 2.

Development of an "early warning" sensor for encrustation of urinary catheters following Proteus infection.

Biofilm formation in long-term urinary catheterized patients can lead to encrustation and blockage of urinary catheters with serious clinical complication. Catheter encrustation stems from infection with urease-producing bacteria, particularly Proteus mirabilis. Urease generates ammonia from urea, and the elevated pH of the urine results in crystallization of calcium and magnesium phosphates, which block the flow of urine.

Accuracy and usefulness of ICD-10 death certificate coding for the identification of patients with ALS: results from the South Carolina ALS Surveillance Pilot Project.

The purpose if this study was to investigate the positive predictive value and sensitivity of the ICD-10 code G12.2, which is used to identify patients who have possibly died from ALS. All patients with a motor neuron disease diagnosis code during the study period (2001-2005) were identified using administrative data.

Neurological deficits during treatment with tumor necrosis factor-alpha antagonists.

Introduction: Neurological deficits that occur during treatment with tumor necrosis factor (TNF)-α antagonists are rare, and their clinical features have not been fully elucidated.

Methods: Retrospective review of medical records of 9 patients who were given TNF-α antagonists, subsequently developed neurological deficits and were cared for at the Medical University of South Carolina between January 2002 and May 2010. Adverse drug reaction probability scale was used for the assessment of their causal connection.

Myasthenic syndrome caused by plectinopathy.

Background: Plectin crosslinks intermediate filaments to their targets in different tissues. Defects in plectin cause epidermolysis bullosa simplex (EBS), muscular dystrophy (MD), and sometimes pyloric atresia. Association of EBS with a myasthenic syndrome (MyS) was documented in a single patient in 1999.

Imaging the in-plane magnetization in a Co microstructure by Fourier transform holography.

We report on experiments using Fourier transform holography to image the in-plane magnetization of a magnetic microstructure. Magnetic sensitivity is achieved via the x-ray magnetic circular dichroism effect by recording holograms in transmission at off-normal incidence. The reference beam is defined by a narrow hole milled at an inclined angle into the opaque mask.

The encrustation and blockage of long-term indwelling bladder catheters: a way forward in prevention and control.

Objectives: To review the literature showing that understanding how Foley catheters become encrusted and blocked by crystalline bacterial biofilms has led to strategies for the control of this complication in the care of patients undergoing long-term indwelling bladder catheterization.

Methods: A comprehensive PubMed search of the literature published between 1980 and December 2009 was made for relevant articles using the Medical Subject Heading terms 'biofilms', 'urinary catheterization', 'catheter-associated urinary tract infection' and 'urolithiasis'. Papers on catheter-associated urinary tract infections and bacterial biofilms collected during 40 years of working in the field were also reviewed.

Strategy to control catheter encrustation with citrated drinks: a randomized crossover study.

Purpose: Nucleation pH is the pH at which Ca and Mg come out of urine to form crystals. If the safety margin between voiding pH and nucleation pH could be increased, it would increase the possibility of an alternative to controlling the activity of urease producing bacteria as a strategy to control catheter encrustation.

Materials And Methods: We performed a 6-week randomized crossover study in 24 patients with catheter blockage who were randomly allocated to a specific sequence of 3 consecutive available treatments, including increased fluid intake, lemon juice and potassium citrate.

Voltage gated potassium channel antibodies in amyotrophic lateral sclerosis.

Although overlap exists between the clinical manifestations in patients with acquired peripheral nerve hyperexcitibility and amyotrophic lateral sclerosis (ALS), the presence of VGKC antibodies has not been associated with the development or clinical manifestations of motor neuron disease. We present the finding of elevated potassium channel antibodies in a cohort of patients with ALS and demonstrate a higher number of abnormal titer levels and higher mean titer levels in ALS patients compared to a cohort of patients with peripheral nervous system disorders. These results suggest the possibility of a subset of ALS patients where autoimmunity could play a role in disease development and progression.

Magnetic ground state of single and coupled permalloy rectangles.

We have studied the magnetic domain structure in Permalloy rectangles that reveal flux-closure domain configurations. Arrays with varying spacing between the rectangles are investigated by scanning electron microscopy with polarization analysis as well as by micromagnetic simulation. In contrast to general expectation, rectangles in the flux-closure Landau state show significant coupling and form a magnetic pattern of common chirality.

Crystalline bacterial biofilm formation on urinary catheters by urease-producing urinary tract pathogens: a simple method of control.

The problem of catheter encrustation stems from infection by urease-producing bacteria. These organisms generate ammonia from urea, elevate the pH of urine and cause crystals of calcium and magnesium phosphates to form in the urine and the biofilm that develops on the catheter. In this study, a laboratory model was used to compare the ability of 12 urease-positive species of urinary tract pathogens to encrust and block catheters.

Rolled-up three-dimensional metamaterials with a tunable plasma frequency in the visible regime.

We propose and realize a novel concept of a self-organized three-dimensional metamaterial with a plasma frequency in the visible regime. We utilize the concept of self-rolling strained layers to roll up InGaAs/GaAs/Ag multilayers with multiple rotations. The walls of the resulting tubes represent a radial superlattice with a tunable layer thickness ratio and lattice constant.

The effect of EDTA instillations on the rate of development of encrustation and biofilms in Foley catheters.

The aim of this research was to examine whether a daily instillation of tetra sodium ethylenediaminetetraacetic acid (EDTA) solution could reduce the rate at which encrustation by crystalline Proteus mirabilis biofilms blocks urinary catheters. Sets of three bladder models were fitted with size 14 all-silicone catheters. Tetra sodium EDTA solution was instilled into the catheter following biofilm development.

Coherent-pulse 2D crystallography using a free-electron laser x-ray source.

Coherent diffractive imaging for the reconstruction of a two-dimensional (2D) finite crystal structure with a single pulse train of free-electron laser radiation at 7.97 nm wavelength is demonstrated. This measurement shows an advance on traditional coherent imaging techniques by applying it to a periodic structure.

A study of the structure of the crystalline bacterial biofilms that can encrust and block silver Foley catheters.

The aim of this study was to examine the structure of the crystalline bacterial biofilms that encrust and block silver/hydrogel-coated latex catheters. Scanning electron microscopy was used to examine the crystalline deposits that were found encrusting catheters obtained from six patients undergoing long-term catheterization in a community setting. Large populations of bacilli and cocci were seen on all catheters developing on a basal foundation layer of crystalline material.

Integrated setup for the fabrication and measurement of magnetoresistive nanoconstrictions in ultrahigh vacuum.

A UHV instrument is presented for in situ fabrication of nanostructures and in situ investigation of their magnetoresistance. Nanostructures of diverse shape and size are created from thin films utilizing a focused ion beam. The magnetic nanostructures are contacted via a micromanipulator, which makes it possible to address the individual structures.