Search results for "EBE"
showing 10 items of 929 documents
A Phase 3 Trial of Sebelipase Alfa in Lysosomal Acid Lipase Deficiency
2015
BackgroundLysosomal acid lipase is an essential lipid-metabolizing enzyme that breaks down endocytosed lipid particles and regulates lipid metabolism. We conducted a phase 3 trial of enzyme-replacement therapy in children and adults with lysosomal acid lipase deficiency, an underappreciated cause of cirrhosis and severe dyslipidemia. MethodsIn this multicenter, randomized, double-blind, placebo-controlled study involving 66 patients, we evaluated the safety and effectiveness of enzyme-replacement therapy with sebelipase alfa (administered intravenously at a dose of 1 mg per kilogram of body weight every other week); the placebo-controlled phase of the study was 20 weeks long and was followe…
Functional MRI of galvanic vestibular stimulation with alternating currents at different frequencies.
2004
Abstract Functional MRI was performed in 28 healthy volunteers to study the effects of galvanic vestibular stimulation with alternating currents (AC-GVS) of different frequencies on brain activation patterns. The aims of this study were (1) to identify specific areas within the vestibular cortical network that are involved in the processing of frequency-specific aspects by correlation analyses, (2) to determine the optimal frequency for stimulation of the vestibular system with respect to perception, and (3) to analyze whether different frequencies of AC-GVS are mediated in different cortical areas or different sites within the vestibular cortex. AC-GVS was performed using sinusoidal stimul…
Magnetic Resonance Imaging Surveillance for Vestibular Schwannoma After Microsurgical Resection Using a Retrosigmoid Transmeatal Approach.
2020
BACKGROUND Vestibular schwannoma (VS) is a benign, usually slow-growing tumor. The drawback of radical microsurgical VS resection is the increased likelihood of neurologic injury, forcing surgeons to leave a tumor remnant in some cases. We evaluated the prognostic value of magnetic resonance imaging (MRI) enhancement patterns to determine the risk of tumor regrowth. METHODS This clinical study included 30 patients (20 women and 10 men) with VS who underwent surgery via a retrosigmoid transmeatal approach. The extent of resection was assessed by MRI 6 months after surgery. Two subtypes of intracanalicular linear enhancement were defined: linear enhancement of the walls of the internal audito…
Frontoparietal cortex and cerebellum contribution to the update of actual and mental motor performance during the day
2016
AbstractActual and imagined movement speed increases from early morning until mid-afternoon. Here, we investigated the neural correlates of these daily changes. Fifteen subjects performed actual and imagined right finger opposition movement sequences at 8 am and 2 pm. Both actual and imagined movements were significantly faster at 2 pm than 8 am. In the morning, actual movements significantly activated the left primary somatosensory and motor areas, and bilaterally the cerebellum; in the afternoon activations were similar but reduced. Contrast analysis revealed greater activity in the cerebellum, the left primary sensorimotor cortex and parietal lobe in the morning than in the afternoon. Im…
Clinical and Functional Outcome After Abdominal Wall Incisional Hernia Repair: Evaluation of Quality-of-Life Improvement and Comparison of Assessment…
2019
Background Hernias severely impact patient quality of life (QoL), and 80% of patients need surgical operation. The primary outcome of the study is to assess improvements in balance, posture and deambulation after abdominal hernia repair. Moreover, the study investigated the improvement in the postoperative QoL. Methods Patients operated at the Policlinico “Paolo Giaccone” at Palermo University Hospital between June 2015 and June 2017 were identified in a prospective database. The functional outcome measures and QoL assessment scales used were numeric rating scale for pain, performance-oriented mobility assessment (POMA) scale, Quebec back pain disability scale, center of gravity (barycenter…
Radiosurgery as Treatment for Acoustic Neuroma. Ten Years’ Experience
2013
The acoustic neuroma is a benign tumour that usually affects the vestibular portion of the vestibulocochlear nerve. It represents 8% of all intracranial tumours and 80% of those arising at the cerebellopontine angle. There are 3 treatment options: microsurgery (the technique of choice), radiosurgery and observation. The objective of the study was to evaluate the results and side effects obtained using radiosurgery as treatment for acoustic neuroma.We performed a review of all patients treated with radiosurgery (Gamma Knife and linear accelerator) at doses of 1200-1300 cGy for unilateral acoustic neuroma in our hospital from January 1999 until January 2010. In all patients we evaluated the o…
Plasma and fibroblasts of Tangier disease patients are disturbed in transferring phospholipids onto apolipoprotein A-I
1998
Plasmas of patients with Tangier disease (TD) lack lipid-rich α-HDL which, in normal plasma, constitutes the majority of high density lipoprotein (HDL). Residual amounts of apolipoprotein (apo)A-I in TD plasma occur as lipid-poor or even lipid-free preβ-HDL. By contrast to normal plasma, TD plasma does not convert preβ-HDL into α-HDL. Moreover, fibroblasts of TD patients were found to be defective in secreting cholesterol or phospholipids in the presence of lipid-free apoA-I. We have therefore hypothesized that both defective conversion of preβ-HDL into α-HDL and defective lipid efflux from TD cells onto lipid-free apoA-I result from a disturbance in phospholipid transfer occurring in both …
Human Bertiella studeri in Spain, Probably of African Origin
1997
A mature tapeworm and gravid proglottids of Bertiella studeri were reported from the stools of a 33-year-old pregnant Spanish woman. The patient had spent the six months preceding this discovery in Kenya. The evidence suggests a case of parasitism imported to Spain from the African continent. The patient presented no symptoms related to the parasite and the discovery in stools of a number of active, white structures led her to collect them. A morphologic and morphometric description of the material obtained is presented. Mebendazole failed to remove the parasite but niclosamide was effective.
The internal auditory artery: (embryology, anatomy, angiography, pathology).
1975
A review of the literature on the embryology, anatomy and angiography of the internal auditory artery has shown that there may be considerable variation as to the origin and number of internal auditory arteries. The present study, based on serial magnification angiographies of the internal auditory artery, has demonstrated 7 variants of the origin of this artery although in 45.4% of cases the internal auditory artery arose from the anterior inferior cerebellar artery. For the diagnosis of pathological processes in the cerebellopontine angle (tumors, sudden deafness, vascular abnormalities) magnification angiography is of special importance. Acoustic neurinomas in particular can be diagnosed…
The impact of isolated lesions on white-matter fiber tracts in multiple sclerosis patients
2015
Infratentorial lesions have been assigned an equivalent weighting to supratentorial plaques in the new McDonald criteria for diagnosing multiple sclerosis. Moreover, their presence has been shown to have prognostic value for disability. However, their spatial distribution and impact on network damage is not well understood. As a preliminary step in this study, we mapped the overall infratentorial lesion pattern in relapsing–remitting multiple sclerosis patients (N = 317) using MRI, finding the pons (lesion density, 14.25/cm3) and peduncles (13.38/cm3) to be predilection sites for infratentorial lesions. Based on these results, 118 fiber bundles from 15 healthy controls and a subgroup of 23 …