Differential Temporal Dynamics of Axial and Appendicular Ataxia in SCA3

Background: Disease severity in spinocerebellar ataxia type 3 (SCA3) is commonly defined by the Scale for the Assessment and Rating of Ataxia (SARA) sum score, but little is known about the contributions and progression patterns of individual items. Objectives: To investigate the temporal dynamics of SARA item scores in SCA3 patients and evaluate if clinical and demographic factors are differentially associated with evolution of axial and appendicular ataxia. Methods: In a prospective, multinational cohort study involving 11 European and 2 US sites, SARA scores were determined longitudinally in 223 SCA3 patients with a follow-up assessment after 1 year. Results: An increase in SARA score from 10 to 20 points was mainly driven by axial and speech items, with a markedly smaller contribution of appendicular items. Finger chase and nose-finger test scores not only showed the lowest variability at baseline, but also the least deterioration at follow-up. Compared with the full set of SARA items, omission of both tests would result in lower sample size requirements for therapeutic trials. Sex was associated with change in SARA sum score and appendicular, but not axial, subscore, with a significantly faster progression in men. Despite considerable interindividual variability, the average annual progression rate of SARA score was approximately three times higher in subjects with a disease duration over 10 years than in those within 10 years from onset. Conclusion: Our findings provide evidence for a difference in temporal dynamics between axial and appendicular ataxia in SCA3 patients, which will help inform the design of clinical trials and development of new (etiology-specific) outcome measures. © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.Funding agencies: This publication is an outcome of ESMI, an EU Joint Programme — Neurodegenerative Disease Research (JPND) Project (www.jpnd.eu). The project is supported through the following funding organizations under the aegis of JPND: Germany, Federal Ministry of Education and Research (BMBF; funding codes 01ED1602A/B); Netherlands, The Netherlands Organization for Health Research and Development; Portugal, Foundation for Science and Technology and Regional Fund for Science and Technology of the Azores; United Kingdom, Medical Research Council. This project has received funding from the European Union’s Horizon 2020 research and innovation programme under grant agreement no. 643417. At the United States sites this work was in part supported by the National Ataxia Foundation.Spinocerebellar ataxia type 3Natural historyScale for the Assessment and Rating of AtaxiaDisease progressio

Factors Associated with Revision Surgery after Internal Fixation of Hip Fractures

Background: Femoral neck fractures are associated with high rates of revision surgery after management with internal fixation. Using data from the Fixation using Alternative Implants for the Treatment of Hip fractures (FAITH) trial evaluating methods of internal fixation in patients with femoral neck fractures, we investigated associations between baseline and surgical factors and the need for revision surgery to promote healing, relieve pain, treat infection or improve function over 24 months postsurgery. Additionally, we investigated factors associated with (1) hardware removal and (2) implant exchange from cancellous screws (CS) or sliding hip screw (SHS) to total hip arthroplasty, hemiarthroplasty, or another internal fixation device. Methods: We identified 15 potential factors a priori that may be associated with revision surgery, 7 with hardware removal, and 14 with implant exchange. We used multivariable Cox proportional hazards analyses in our investigation. Results: Factors associated with increased risk of revision surgery included: female sex, [hazard ratio (HR) 1.79, 95% confidence interval (CI) 1.25-2.50; P = 0.001], higher body mass index (fo

Comparison of Optical Performance in Eyes Implanted With Aspheric Foldable, Spherical Foldable, and Rigid PMMA IOLs

PURPOSE: To compare the optical performance of rigid spherical polymethylmethacrylate (PMMA), foldable spherical, and foldable aspheric intraocular lenses (IOLs). METHODS: Measurements were obtained monocularly from pseudophakic patients with a PMMA IOL (Ophtec PC265y or Rayner 105U), spherical AcrySof MA30 IOL (Alcon Laboratories Inc), or aspheric Tecnis ZA9003 IOL (Abbott Medical Optics). Contrast sensitivity was measured using the Holladay automated contrast sensitivity test with 5.0-mm artificial pupil at 3 and 6 cycles per degree at optimal focus and at several defocus levels. The myopic shift (shift of the optimal focus toward more myopic values at lower spatial frequencies) and depth of focus were determined. Wavefront aberrations were assessed with a Hartmann-Shack wavefront analyzer; straylight was measured using the C-Quant meter (Oculus Optikgerate GmbH). RESULTS: Nine patients with a spherical rigid PMMA IOL, 19 patients with a spherical foldable IOL, and 24 patients with an aspheric foldable IOL met the inclusion criteria. Eyes with an aspheric IOL showed less spherical aberration than eyes with other IOLs; no differences were found in overall higher order aberrations. No differences in contrast sensitivity at optimal focus and in straylight were found among the IOLs. Eyes with a PMMA IOL showed a larger depth of focus compared to eyes with an aspheric IOL. Eyes with an aspheric IOL had a smaller myopic shift than eyes with other IOLs. CONCLUSIONS: Optical performance differences among the IOLs in this study are small, concurring with similar higher order aberrations found in the three groups. Reduction in myopic shift appears to be the most obvious effect of aspheric IOLs. [J Refract Surg. 2011; 27(2): 98-105.] doi: 10.3928/1081597X-20100413-0

Clinical comparison of the optical performance of aspheric and spherical intraocular lenses

PURPOSE: To compare the optical performance of aspheric Tecnis ZA9003 and spherical Sensar AR40e intraocular lenses (IOLs). SETTING: Laboratory of Experimental Ophthalmology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. METHODS: An aspheric IOL was implanted in 1 eye and a spherical IOL in the other eye of patients with bilateral age-related cataract. Contrast sensitivity was measured using 2 computerized tests (vertical sine-modulated gratings and circular sine-modulated patterns) with cycloplegia and a 5.0 mm artificial pupil under photopic conditions at optimum refractive correction and at several defocus levels. The depth of focus and the myopic shift (shift of optimum focus toward more myopic values at lower spatial frequencies) were determined. Higher-order aberrations were assessed using a Hartmann-Shack wavefront analyzer; straylight was measured with a straylight meter. RESULTS: In the 60 eyes evaluated, there were no statistically significant differences in contrast sensitivity measured at optimum focus, depth of focus, or straylight between the 2 IOLs. The mean spherical aberration was significantly lower with the aspheric IOL (-0.036 pm) than with the spherical IOL (0.064 mu m) (P CONCLUSIONS: Eyes with the aspheric IOL had lower spherical aberration than eyes with the spherical IOL and, related to that, a smaller myopic shift. No significant differences were found between the 2 IOLs in contrast sensitivity measured at optimum focus, depth of focus, or straylight

An uncemented iso-elastic monoblock acetabular component : Preliminary results

Little is known about the clinical application of highly cross-linked polyethylene (HXLPE) blended with vitamin E. This study evaluates an uncemented iso-elastic monoblock cup with vitamin E blended HXLPE. 112 patients were followed up for 2. years. 95.5% completed the follow-up. The mean VAS score for patient satisfaction was 8.8 and the mean Harris Hip Score was 94.2. In 7 cases initial gaps behind the cup were observed, which disappeared completely during follow-up in 6 cases. The mean femoral head penetration rate was 0.055. mm/year. No adverse reactions or abnormal mechanical behavior was observed with the short term use of vitamin E blended HXLPE. This study shows the promising performance of this cup and confirms the potential of vitamin E blended HXLPE

Straylight measurements in pseudophakic eyes with natural and dilated pupils:One-year follow-up

PURPOSE: To compare the amount of straylight in natural pupils and dilated pupils in pseudophakic eyes 6 weeks and 1 year after cataract extraction. SETTING: Laboratory of Experimental Ophthalmology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. METHODS: This study evaluated patients with bilateral age-related cataract who had cataract surgery with implantation of an aspheric Tecnis ZA9003 or spherical Sensar AR40e intraocular lens (IOL). Stray light measurements were performed with a C-Quant straylight meter 6 weeks after surgery (with natural pupils) and 1 year after surgery (with natural and dilated pupils) in a randomly chosen eye. Retroillumination photographs of dilated pupils were taken to document posterior capsule opacification. The main outcome variable for straylight measurements was the logarithmic straylight parameter, log(s). RESULTS: Twenty-two patients were evaluated. There was a statistically significant decrease in straylight in a natural pupil between 6 weeks (mean 1.44 log[s]) and 1 year (mean 1.30 log[s]) postoperatively (P = .012). The straylight parameter was greater after dilation (mean 1.48 log[s]) than with a natural pupil (1.29 log[s]) at 1 year (P = .012). This difference was greater when more anterior capsule was visible in the pupillary area (P = .031). CONCLUSIONS: Stray light decreased significantly in the first year after cataract surgery. Furthermore, it increased with increasing pupil size, which was associated with a capsulorhexis smaller than the pupil. This indicates the capsulorhexis should be as large as possible to prevent straylight, especially under low-luminance conditions when the pupil is large

Relationship between contrast sensitivity and spherical aberration Comparison of 7 contrast sensitivity tests with natural and artificial pupils in healthy eyes

PURPOSE: To find a contrast sensitivity test that can be used clinically to evaluate interventions aimed at minimizing spherical aberration and determine the circumstances under which these tests should be performed. SETTING: Laboratory of Experimental Ophthalmology, University of Groningen, Groningen, The Netherlands. METHODS: Contrast sensitivity tests were performed using 2 experimental designs. Design 1 was with a natural pupil under mesopic and photopic conditions. Design 2 was with a 5.0 mm artificial pupil after cycloplegia under photopic conditions only. Two computerized tests (vertical sine-modulated gratings [VSG] and Holladay circular sine-modulated patterns [HACSS]) and 5 chart tests (Pelli-Robson, acuity-measuring letter charts at low contrast [2.5% and 10%], VectorVision, and edge contrast sensitivity) were used. Spherical aberration was assessed with a Hartmann-Shack wavefront analyzer. RESULTS: Forty-nine healthy subjects aged 20 to 35 years (n = 24) and 55 to 70 years (n = 25) participated. Design 2 showed a significant relationship between contrast sensitivity and spherical aberration with the HACSS at 3 cycles per degree (cpd) (P = .03) and 6 cpd (P = .01) and with the VSG at 6 cpd (P = .01). Design 1 yielded no significant relationships. CONCLUSIONS: Using an artificial pupil, a relationship between contrast sensitivity and spherical aberration was established with the VSG and HACSS tests but not with the chart tests. No test showed a relationship using natural pupils under either lighting condition. Chart tests are unsuitable for uncovering contrast sensitivity differences related to differences in spherical aberration, as typically found in healthy phakic eyes

Advocacy for use of the modified Iowa Level of Assistance Scale for clinical use in patients after hip replacement: an observational study

Objectives: o test the internal consistency and item difficulty of the modified Iowa Level of Assistance Scale (mILAS). Design: Retrospective observational study. Setting: Two orthopaedic wards of two general hospitals. Participants"Following elective primary unilateral total hip replacement surgery, all participants performed mILAS activities that were scored daily to assess their recovery of activities during hospitalisation. Main outcome measures: The internal consistency and the level of assistance needed by the patient (item difficulty) of the mILAS were calculated using data from Hospital X (n = 255). A cross-validation was performed using data from Hospital Y (n = 224). Results: The internal consistency of the mILAS was acceptable on all three postoperative days (α=0.84 to 0.97). Cronbach’s α and Rasch analysis revealed a misfit of stair climbing with the other items of the mILAS. The item difficulty of the mILAS items changed over the first two postoperative days. During the first three postoperative days, the sit to supine transfer was generally the most difficult item to achieve, and the sit to stand transfer was the least difficult item to achieve as rated by physiotherapists. The cross-validation analysis revealed similar results. Conclusions: The mILAS is a clinically sound measurement tool to assess the ability of patients to perform five functional tasks safely during hospitalisation. Stair climbing appears to be the easiest item to complete, and the sit to supine transfer is generally the most difficult after surgery

Relating cell proliferation to in vivo bone formation in porous Ca/P scaffolds

Most current methods for cell monitoring on 3D porous scaffolds involve end-stage investigation of scaffolds. Repeated measurements on scaffolds, without disturbing cell vitality and proliferation, are needed to relate in vitro to in vivo data. Alamar Blue™ was used for this purpose. Two different Ca/P scaffolds were studied, using rat BMSCs with three different seeding densities [2.5 × 104 (SD1), 2.5 × 105 (SD2), 2.5 × 106 (SD3) cells]. Alamar Blue™ readings were done on days 1, 3, 5 and 7. After 7 days all 96 scaffolds (n = 16) were implanted in 16 mice for 4 weeks. Bone histomorphometry was performed. For both scaffolds, seeding efficiencies were highest with SD1 and SD2, cell proliferation was optimal in SD1, whereas SD3 resulted in an initial drop in vital cell number in the first 3 days. In vivo, upscaling from SD1 to SD2 lead to significantly more bone contact% in both scaffolds. Alamar Blue™ was shown to be a valuable tool in relating in vitro to in vivo data. Cell proliferation may differ depending on seeding density and scaffold type used. Seeding more cells may not necessarily result in more in vivo bone contact