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Systolic durations, estimated by each of the end systole estimates, are then compared to the validation systolic duration provided by the PTT based end systole point. Data comes from ten pigs, across two protocols testing the algorithms under different hemodynamic states. The resulting mean difference ± limits of agreement between measured and estimated systolic duration, of [Formula see text] versus [Formula see text], for the new and existing algorithms respectively, indicate the new algorithms superiority.BACKGROUND Cockayne syndrome is a rare autosomal recessive neurodegenerative disorder caused by mutations of either the ERCC6/CSB or ERCC8/CSA genes. Here, we describe two sisters with Cockayne syndrome caused by compound heterozygous mutations in the ERCC8 gene using multimodal imaging. Significant ophthalmic and systemic phenotypic variability is discussed. MATERIALS AND METHODS Multimodal imaging was performed in two affected sisters and included electroretinography, optical coherence tomography, ultra-wide-field confocal scanning laser ophthalmoscopy, fundus autofluorescence and fluorescein angiography, and magnetic resonance imaging. Genetic analyses were performed on the affected sisters, both parents, and three unaffected siblings. RESULTS The older sister (Patient 1) had mental retardation, bilateral hearing loss, ataxia, and decreased visual acuity with retinal dystrophy. Radiographic studies revealed microcephaly, cerebral and cerebellar atrophy, ventriculomegaly, and a diffusely thickened skull. Fur imaging and systemic findings revealed wide phenotypic variability between the affected siblings.BACKGROUND A number of clinical trials have been published assessing the role of iliac crest bone grafting for the management of recurrent anterior instability with glenoid bone loss in contemporary practice. We therefore performed a systematic review of contemporary literature to examine the effect of iliac crest bone grafting on postoperative outcomes of these patients. Our hypothesis is that contemporary iliac crest bone block techniques are associated with low reoperation and complication rates combined with satisfactory functional results. METHODS The US National Library of Medicine (PubMed/MEDLINE), the Cochrane Database of Systematic Reviews, and EMBASE were searched between January 2008 and December 2019 for relevant publications. RESULTS Following the application of the inclusion-exclusion criteria, nine articles were found eligible for our analysis. In total, 261 patients (mean age range, 25.5-37.5 years; mean follow-up range, 20.6-42 months) were included in the studies of the current review. The ms.The original version of this article unfortunately contained a mistake. Title was incorrect.OBJECTIVE To measure T2 values for magnetic resonance neurography (MRN) of the healthy distal sciatic nerve and compare those to T2 changes in patients with nerve compression. MATERIALS AND METHODS Twenty-one healthy subjects and five patients with sciatica due to disc herniation underwent MRN using a T2-prepared turbo spin echo (TSE) sequence of the distal sciatic nerve bilaterally. Six and one of those healthy subjects further underwent a commonly used multi-echo spin-echo (MESE) sequence and magnetic resonance spectroscopy (MRS), respectively. RESULTS T2 values derived from the T2-prepared TSE sequence were 44.6 ± 3.0 ms (left) and 44.5 ± 2.6 ms (right) in healthy subjects and showed good inter-reader reliability. Firsocostat In patients, T2 values of 61.5 ± 6.2 ms (affected side) versus 43.3 ± 2.4 ms (unaffected side) were obtained. T2 values of MRS were in good agreement with measurements from the T2-prepared TSE, but not with those of the MESE sequence. DISCUSSION A T2-prepared TSE sequence enables precise determination of T2 values of the distal sciatic nerve in agreement with MRS. A MESE sequence tends to overestimate nerve T2 compared to T2 from MRS due to the influence of residual fat on T2 quantification. Our approach may enable to quantitatively assess direct nerve affection related to nerve compression.Alzheimer's disease (AD) is a debilitating neurodegenerative disease that causes a progressive decline in memory, language and problem solving. For decades mechanism-based therapies have primarily focused on amyloid β (Aβ) processing and pathways that govern neurofibrillary tangle generation. With the potential exception to Aducanumab, a monotherapy to target Aβ, clinical trials in these areas have been challenging and have failed to demonstrate efficacy. Currently, the prescribed therapies for AD are those that target the cholinesterase and glutamatergic systems that can moderately reduce cognitive decline, dependent on the individual. In the brain, over 40% of neuronal synapses are glutamatergic, where the glutamate level is tightly regulated through metabolite exchange in neuronal, astrocytic and endothelial cells. In AD brain, Aβ can interrupt effective glutamate uptake by astrocytes, which evokes a cascade of events that leads to neuronal swelling, destruction of membrane integrity and ultimately cell death. Much work has focussed on the post-synaptic response with little insight into how glutamate is regulated more broadly in the brain and the influence of anaplerotic pathways that finely tune these mechanisms. The role of blood branched chain amino acids (BCAA) in regulating neurotransmitter profiles under disease conditions also warrant discussion. Here, we review the importance of the branched chain aminotransferase proteins in regulating brain glutamate and the potential consequence of dysregulated metabolism in the context of BCAA or glutamate accumulation. We explore how the reported benefits of BCAA supplementation or restriction in improving cognitive function in other neurological diseases may have potential application in AD. Given that memantine, the glutamate receptor agonist, shows clinical relevance it is now timely to research related pathways, an understanding of which could identify novel approaches to treatment of AD.BACKGROUND Quantitative SPECT imaging in targeted radionuclide therapy with lutetium-177 holds great potential for individualized treatment based on dose assessment. The establishment of dose-effect relations requires a standardized method for SPECT quantification. The purpose of this multi-center study is to evaluate quantitative accuracy and inter-system variations of different SPECT/CT systems with corresponding commercially available quantitative reconstruction algorithms. This is an important step towards a vendor-independent standard for quantitative lutetium-177 SPECT. METHODS Four state-of-the-art SPECT/CT systems were included Discovery™ NM/CT 670Pro (GE Healthcare), Symbia Intevo™, and two Symbia™ T16 (Siemens Healthineers). Quantitative accuracy and inter-system variations were evaluated by repeatedly scanning a cylindrical phantom with 6 spherical inserts (0.5 - 113 ml). A sphere-to-background activity concentration ratio of 101 was used. Acquisition settings were standardized medium energy collimator, body contour trajectory, photon energy window of 208 keV (± 10%), adjacent 20% lower scatter window, 2 × 64 projections, 128 × 128 matrix size, and 40 s projection time.
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