271 research outputs found

    Teacher, Father, King. Royal representations of Louis-Philippe I

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    I am indebted to the current owners of Castle Reichenau in Tamins, Graubünden, especially Johann-Baptista von Tscharner, who kindly invited me to walk in the footsteps of 19th-century tourists and visit the Louis-Philippe room and Adolf von Planta’s laboratory. I am also grateful to the House of Orléans for granting me access to archival material from the fonds de la Maison de France held in the Archives Nationales, Paris.Peer reviewedPublisher PD

    Early Globus Pallidus Internus Stimulation in Pediatric Patients With Generalized Primary Dystonia: Long-Term Efficacy and Safety

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    Primary generalized dystonia presents mainly at a young age and commonly is severely disabling. The authors report the long-term follow-up (mean, 73 months; range, 50-101 months) of 5 pediatric patients (mean age at surgery 13 years; range, 8-16 years) undergoing globus pallidus internus deep brain stimulation. Mean improvement in the Burke-Fahn-Marsden movement score was 67.4% (range, 47.0%-87.5%), 75.4% (range, 61.5%-91.7%), and 83.5% (range, 72.0%-93.3%) at 3 months, 12 months, and long-term follow-up (>36 months), respectively. Hardware problems (electrode dislocation/breakage of extension cable, and imminent perforation of extension cable) were observed in 2 patients (operative revision without sequelae). Except for mild dysarthria in 2 patients, no other therapy-related morbidity was observed. The authors found globus pallidus internus stimulation to offer a very effective and safe therapy in pediatric patients with primary dystonia. Early neurosurgical intervention seems to be crucial to prevent irreversible impairment of motor function

    Bilateral double beta peaks in a PD patient with STN electrodes

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    Subthalamic local field potentials in the beta band are considered as potential biomarkers for closed-loop deep brain stimulation. To investigate the subthalamic beta band peak amplitudes in a Parkinson's disease patient over an extended period of time by using a novel and commercially available neurostimulator with permanent sensing capability. We recorded local field potentials of the subthalamic nucleus using the Medtronic Percept™ implantable neurostimulator at rest and during physical activity (gait) with and in response to deep brain stimulation. We found a double-peaked beta activity on both sides. Increasing stimulation and physical activity resulted in a decreased beta band amplitude, but was accompanied by the appearance of a second, and previously unrecognized peak at 13~Hz in the right hemisphere. Our results will support the investigation of distinct different peaks in the beta band and their relevance and usefulness as closed-loop biomarkers

    Long-Term Efficacy and Safety of Chronic Globus Pallidus Internus Stimulation in Different Types of Primary Dystonia

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    Background: Deep brain stimulation (DBS) of the globus pallidus internus (GPi) offers a very promising therapy for medically intractable dystonia. However, little is known about the long-term benefit and safety of this procedure. We therefore performed a retrospective long-term analysis of 18 patients (age 12-78 years) suffering from primary generalized (9), segmental (6) or focal (3) dystonia (minimum follow-up: 36 months). Methods: Outcome was assessed using the Burke-Fahn-Marsden (BFM) scores (generalized dystonia) and the Tsui score (focal/segmental dystonia). Follow-up ranged between 37 and 90 months (mean 60 months). Results: Patients with generalized dystonia showed a mean improvement in the BFM movement score of 39.4% (range 0 68.8%), 42.5% (range -16.0 to 81.3%) and 46.8% (range-2.7 to 83.1%) at the 3- and 12-month, and long-term follow-up, respectively. In focal/ segmental dystonia, the mean reduction in the Tsui score was 36.8% (range 0-100%), 65.1% (range 16.7-100%) and 59.8% (range 16.7-100%) at the 3- and 12-month, and long-term follow-up, respectively. Local infections were noted in 2 patients and hardware problems (electrode dislocation and breakage of the extension cable) in 1 patient. Conclusion: Our data showed Gpi-DBS to offer a very effective and safe therapy for different kinds of primary dystonia, with a significant long-term benefit in the majority of cases. Copyright (c) 2008 S. Karger AG, Base

    Efficacy and safety of VEPTR instrumentation for progressive spine deformities in young children without rib fusions

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    This retrospective study analyses 23 children treated with vertical expandable prosthetic titanium rib (VEPTR) for correction of non-congenital early onset spine deformities. After the index procedure (IP), the device was lengthened at 6-month intervals. The average (av) age at the time of IP was 6.5years (1.11-10.5). The av follow-up time was 3.6years (2-5.8). Diagnosis included 1 early onset idiopathic scoliosis, 11 neuromuscular, 2 post-thoracotomy scoliosis, 1 Sprengel deformity, 2 hyperkyphosis, 1 myopathy and 5 syndromic. Surgeries (187) included 23 IPs, av 6.5 (4-10) device expansions per patient (149) and 15 unplanned surgeries. 23 complications (0.13 per surgery) included 10 skin sloughs, 5 implant dislocations, 2 rod breakages and 6 infections. Coronal Cobb angle was av 68° (11°-111°), at follow-up av 54° (0°-105°). Pelvic obliquity was av 33° (13°-60°), at follow-up av 16° (0°-42°). T1 tilt was av 29° (5°-84°), two remained unchanged, the remainder improved 10°-68°. Sagittal plane: All but two had stable profiles, two hyperkyphosis of 110°/124° improved to 56°/86°. Space available for lung ratio was less than 90% in ten before the IP, improved in nine and deteriorated in one. Originally designed for thoracic insufficiency syndromes related to rib and vertebral anomalies, VEPTR proved to be a valuable alternative to dual growing rods for non-congenital early onset spine deformities. The complication rate was lower, the control of the sagittal plane and the pelvic obliquity was as good, but the correction of the coronal plane deformity was less than growing rods. However, VEPTR's spine-sparing approach might provoke less spontaneous spinal fusion and ease the final correction at maturit

    Utilizing the NANT Core Competencies to Guide the Occupational Therapy Doctoral Capstone Experience and Project in the NICU

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    The occupational therapy doctorate degree requires a capstone experience and project to be completed within a specific occupational therapy (OT)-related setting. The doctoral capstone experience and project can be difficult to complete in the highly specialized Neonatal Intensive Care Unit (NICU). This paper provides a sample outline of the doctoral capstone experience and project in the NICU. The NANT Core Competencies, written by the National Association of Neonatal Therapists Professional Collaborative, were utilized as a guideline to track student progress and ensure understanding of neonatal therapy topics. This paper follows one student’s doctoral capstone experience and project in the NICU while mentored by an occupational therapist. Learning objectives related to utilizing the NANT Core Competencies, gaining clinical skills, and participating in program development were created. The site mentor trained, supported, and educated the student in the NICU while monitoring her progress during the fourteen weeks. Overall, the student successfully completed all objectives. This paper provides an example of the doctoral capstone experience and project in the NICU utilizing the NANT Core Competencies to support the mentor-student relationship and student learning with educational topics provided in the competencies guiding the capstone experience. With details including student progress, weekly mentor-student meetings, and roles/responsibilities of the student and mentor, this paper serves as a resource and guideline for OT faculty as well as potential mentors of occupational therapy doctorate students in the NICU setting. Additionally, this paper highlights the feasibility of the completion of the OT doctoral capstone in the NICU

    Movement kinematic after deep brain stimulation associated microlesions

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    Deep brain stimulation is widely used for the treatment of movement disorders such as Parkinson's disease and dystonia. After the implantation of electrodes an immediate improvement of clinical symptoms has been described. It is unclear, whether movement kinematics are also changed by this 'microlesion effect'

    Microvascular decompression for trigeminal neuralgia in the elderly: efficacy and safety

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    OBJECTIVE The safety and efficacy of surgical microvascular decompression (MVD) in elderly patients with trigeminal neuralgia (TN) is controversially discussed in the literature. A widespread reluctance to expose this cohort to major intracranial surgery persists. Our aim was to compare the efficacy and safety between older and younger patients with TN. METHODS In this cross-sectional study, 139 MVD procedures (103 patients < 70 and 36 patients ≥ 70) were included. Surgical fitness was assessed by the American Society of Anesthesiology (ASA) grade. The pain-free interval was evaluated using Kaplan-Meier analysis only in patients with a recent follow-up visit. Independent risk factors for recurrence in patients with a minimum 12-month follow-up were determined. RESULTS Patients ≥ 70 showed a significantly higher number of comorbidities. Pain intensity, affection of trigeminal branches and symptom duration was similar between groups. No significant difference in treatment associated complications and permanent neurological deficits was shown. There was no treatment-related mortality. A tendency towards a lower recurrence rate in patients < 70 did not reach statistical significance (17.6% vs. 28.6%, P = 0.274). Pain-free interval was not different between both cohorts (78.7 vs. 73.5 months, P = 0.391). CONCLUSION Despite a higher prevalence of comorbidities in elderly patients, complication rates and neurological deficits after MVD were comparable to younger patients. Rates of immediate and long-term pain relief compared favorably to previous studies and were similar between elderly and younger patients. These data endorse MVD as a safe and effective first-line surgical procedure for elderly patients with TN and neurovascular conflict on MRI

    Phosphatase Activity of Homo- and Heterodinuclear Transition Metal Complexes of Patellamide Derivatives

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    Naturally occuring cyclic peptides exist in the ascidians Lissoclinum Patella of the Pacific and Indian Oceans. The biological role of these structural interesting marine secondary metabolites is still unclear. The patellamides are able to bind a variety of transition metal ions. Some of the copper(II) complexes of cyclic pseudo-peptides are known to form carbonato-bridged complexes when exposed to CO2. Thus, in previous studies a library of patellamide derivatives has been designed and prepared. The copper(II) coordination properties of these patellamide derivatives is widely understood. Interestingly, the corresponding dinuclear copper(II) complexes are able to efficiently, catalytically hydrolyze phosphoesters and hydrate CO2. The natural peptides are produced by the cyanobacteria prochloron didemnid, a photosynthetic symbiont of L.Patella. Since the patellamides are extracted from hydrophyllic cytoplasmic environment, together with notably high concentrations of some transition metal ions like copper(II) and zinc(II). It is likely that a natural function of probably existing transition metal complexes in this environment is hydrolase activity. The biologic background as well as the state of art regarding the copper(II) coordination chemistry is summarized in Chapter 1. The synthesis of the patellamide derivatives is shortly discussed. In Chapter 3, the electrochemistry of the copper(II) complexes is discussed. Cyclic voltammetry and square wave voltammetry were used to study the existence of a complexation equilibrium between various species in solution. From the obtained results, it is concluded that the copper(I/II) redox chemistry is propably not a biologic relevant biologic function at neutral pH. Under basic conditions, it is possible that stable copper(I) species exist. It can be proposed that these are carbonate or bicarbonate bridged. Moreover, under basic conditions an oxygenation test reaction was performed, and it was shown that a dinuclear copper(II) complex of an patellamide derivative is involved in an oxygenation reaction under these rather unphysiological conditions. A tentative mechanistic proposal is discussed, which is based on the observation of a radical coupling product and insights obtained from a low temperature NMR experiment. In addition the proposed copper(II) complexation equilibria of the ligand H4pat4 is discussed, and the respective EPR spectra with their simulations are in the focus of the last section of Chapter 3. Especially while regarding the formation of heterodinuclear copper(II)/ zinc(II) complexes with H4pat4 in Chapter 5this equilibrium is requested. Since biological relevant hydrolysis chemistry is often based on zinc(II) enzymes, zinc(II) complexes of the macrocyclic peptides were studied. Chapter 4 presents the formation of zinc(II) complexes, which are explored using isothermal calorimetric titrations in combination with NMR spectroscopy and mass spectrometry. After an overview of the biological relevance of zinc(II) based hydrolases, Chapter 4 presents a proposed zinc(II) complexation equilibrium. In Chapter 5, the formation of heterodinuclear copper(II)/ zinc(II) complexes is described based on spectroscopic results. EPR spectroscopy, paramagnetic NMR and UV/vis spectroscopy in combination with mass spectrometry are used to describe two different heterodinuclear complexes. Furthermore, Chapter 5 describes the formation of a copper(II)/ zinc(II) complex due to a distinct cooperative effect, with a ligand that does not form stable spectroscopically characterizable homodinuclear zinc(II) complex. Phosphoester hydrolysis reactions with a model substrate of all complexes, described in Chapters 3,4 and 5 have been investigated in a kinetic assay. The pH dependent results are discussed in Chapter 6. Furthermore the substrate dependency of the initial rate was tested. All complexes discussed exhibit catalytic activity in a pH range close to neutral. Comparison of all data obtained shows that the zinc(II) complexes are slightly more active than the corresponding homodinuclear copper(II) complexes
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