The Dystonia Coalition: A Multicenter Network for Clinical and Translational Studies.

Dystonia is a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal postures, repetitive movements, or both. Research in dystonia has been challenged by several factors. First, dystonia is uncommon.

Performance of axillary and rectal temperature measurement in private pediatric practice.

To better understand the role and reliability of axillary temperature measurements in clinical real life, axillary and rectal measurements in infants presenting in a private pediatric practice because of fever were compared. Prospectively, 169 infants (81 girls), median 9 (interquartile range 6-13) months of age, were examined at room temperature (20-24 °C). Two left and two right axillary, as well as two rectal measurements were taken with a digital thermometer and subsequently averaged.

Recent developments in dystonia.

Purpose Of Review: The dystonias are a family of related disorders with many different clinical manifestations and causes. This review summarizes recent developments regarding these disorders, focusing mainly on advances with direct clinical relevance from the past 2 years.

Recent Findings: The dystonias are generally defined by their clinical characteristics, rather than by their underlying genetic or neuropathological defects.

Designing clinical trials for dystonia.

With advances in the understanding of the pathophysiology of dystonia, novel therapeutics are being developed. Such therapies will require clinical investigation ranging from exploratory studies to examine safety, tolerability, dosage selection, and preliminary efficacy to confirmatory studies to evaluate efficacy definitively. As dystonia is a rare and complex disorder with clinical and etiological heterogeneity, clinical trials will require careful consideration of the trial design, including enrollment criteria, concomitant medication use, and outcome measures.

Phenomenology and classification of dystonia: a consensus update.

This report describes the consensus outcome of an international panel consisting of investigators with years of experience in this field that reviewed the definition and classification of dystonia. Agreement was obtained based on a consensus development methodology during 3 in-person meetings and manuscript review by mail. Dystonia is defined as a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both.

Amyloid-beta peptide Abetap3-42 affects early aggregation of full-length Abeta1-42.

The major amyloid-beta (Abeta) peptides found in the brain of familial and late onset Alzheimer's disease include the full-length Abeta1-42 and N-terminally truncated, pyroglutamylated peptides Abetap3-42 and Abetap11-42. The biophysical properties of Abeta1-42 have been extensively studied, yet little is known about the other modified peptides. We investigated the aggregation kinetics of brain-specific Abeta peptides to better understand their potential roles in plaque formation.

Diethylamine extraction of proteins and peptides isolated with a mono-phasic solution of phenol and guanidine isothiocyanate.

Representative extraction of both RNA and protein from a single biological sample is required for reliable assessment of coordinated changes in gene and protein expression. Such a simultaneous extraction can be performed by using Trizol Reagent. Here, we demonstrate that, as an alternative to SDS, 2% diethylamine is an effective solvent, which can be conveniently used in extraction of Trizol-isolated proteins from various tissues.

The influence of a clear layer on near infrared spectrophotometry: comparison of measurements in a liquid neonatal head phantom to infants in vivo.

Near infrared spectrophotometric (NIRS) algorithms to determine the tissue oxygen saturation (TOI) assume a semi-infinite, homogenous tissue geometry. At the head, the clear cerebrospinal fluid (CSF) layer may violate this assumption. The aim was to estimate the error in the TOI values caused by the CSF layer in vitro and to confirm the results in vivo.

Liver tissue oxygenation as measured by near-infrared spectroscopy in the critically ill child in correlation with central venous oxygen saturation.

Objective: To evaluate the clinical usefulness of near-infrared spatially resolved spectroscopic quantitative assessment of liver tissue oxygenation for simple, non-invasive estimation of global tissue oxygenation in critically ill neonates and children.

Design: Prospective observational clinical study.

Setting: A tertiary multidisciplinary neonatal and paediatric intensive care unit (23 beds).