Scapholunate Ligament Insufficiency in a Patient with Scaphocapitate Coalition.

We report a case of chronic scapholunate ligament insufficiency in a patient with scaphocapitate coalition. After more than 4 years of mild symptoms, there was no radiological evidence of progression to scapholunate advanced collapse in spite of minimal load-bearing repetitive physical activities undertaken by the patient as a professional guitarist. We believe that scaphocapitate coalition could contribute to mitigate the progression to scapholunate advanced collapse by preventing abnormal flexion of the scaphoid once the ligament is not competent anymore. The biomechanical and surgical implications of this type of carpal coalition are also discussed.

Inertial sensors for gait monitoring and design of adaptive controllers for exoskeletons after stroke: a feasibility study.

Introduction: Tuning the control parameters is one of the main challenges in robotic gait therapy. Control strategies that vary the control parameters based on the user's performance are still scarce and do not exploit the potential of using spatiotemporal metrics. The goal of this study was to validate the feasibility of using shank-worn Inertial Measurement Units (IMUs) for clinical gait analysis after stroke and evaluate their preliminary applicability in designing an automatic and adaptive controller for a knee exoskeleton (ABLE-KS). Methods: First, we estimated the temporal (i.e., stride time, stance, and swing duration) and spatial (i.e., stride length, maximum vertical displacement, foot clearance, and circumduction) metrics in six post-stroke participants while walking on a treadmill and overground and compared these estimates with data from an optical motion tracking system. Next, we analyzed the relationships between the IMU-estimated metrics and an exoskeleton control parameter related to the peak knee flexion torque. Finally, we trained two machine learning algorithms, i.e., linear regression and neural network, to model the relationship between the exoskeleton torque and maximum vertical displacement, which was the metric that showed the strongest correlations with the data from the optical system [r = 0.84; ICC(A,1) = 0.73; ICC(C,1) = 0.81] and peak knee flexion torque (r = 0.957). Results: Offline validation of both neural network and linear regression models showed good predictions (R2 = 0.70-0.80; MAE = 0.48-0.58 Nm) of the peak torque based on the maximum vertical displacement metric for the participants with better gait function, i.e., gait speed > 0.7 m/s. For the participants with worse gait function, both models failed to provide good predictions (R2 = 0.00-0.19; MAE = 1.15-1.29 Nm) of the peak torque despite having a moderate-to-strong correlation between the spatiotemporal metric and control parameter. Discussion: Our preliminary results indicate that the stride-by-stride estimations of shank-worn IMUs show potential to design automatic and adaptive exoskeleton control strategies for people with moderate impairments in gait function due to stroke.

Adapted Assistance and Resistance Training With a Knee Exoskeleton After Stroke.

Studies on robotic interventions for gait rehabilitation after stroke require: (i) rigorous performance evidence; (ii) systematic procedures to tune the control parameters; and (iii) combination of control modes. In this study, we investigated how stroke individuals responded to training for two weeks with a knee exoskeleton (ABLE-KS) using both Assistance and Resistance training modes together with auditory feedback to train peak knee flexion angle. During the training, the torque provided by the ABLE-KS and the biofeedback were systematically adapted based on the subject's performance and perceived exertion level. We carried out a comprehensive experimental analysis that evaluated a wide range of biomechanical metrics, together with usability and users' perception metrics. We found significant improvements in peak knee flexion ( p = 0.0016 ), minimum knee angle during stance ( p = 0.0053 ), paretic single support time ( p = 0.0087 ) and gait endurance ( p = 0.022 ) when walking without the exoskeleton after the two weeks of training. Participants significantly ( ) improved the knee angle during the stance and swing phases when walking with the exoskeleton powered in the high Assistance mode in comparison to the No Exo and the Unpowered conditions. No clinically relevant differences were found between Assistance and Resistance training sessions. Participants improved their performance with the exoskeleton (24-55 %) for the peak knee flexion angle throughout the training sessions. Moreover, participants showed a high level of acceptability of the ABLE-KS (QUEST 2.0 score: 4.5 ± 0.3 out of 5). Our preliminary findings suggest that the proposed training approach can produce similar or larger improvements in post-stroke individuals than other studies with knee exoskeletons that used higher training intensities.

Effectiveness and safety of anti-CGRP monoclonal antibodies in patients over 65 years: a real-life multicentre analysis of 162 patients.

BACKGROUND: Anti-CGRP monoclonal antibodies have shown notable effectiveness and tolerability in migraine patients; however, data on their use in elderly patients is still lacking, as clinical trials have implicit age restrictions and real-world evidence is scarce. In this study, we aimed to describe the safety and effectiveness of erenumab, galcanezumab and fremanezumab in migraine patients over 65 years old in real-life. METHODS: In this observational real-life study, a retrospective analysis of prospectively collected data from 18 different headache units in Spain was performed. Migraine patients who started treatment with any anti-CGRP monoclonal antibody after the age of 65 years were included. Primary endpoints were reduction in monthly migraine days after 6 months of treatment and the presence of adverse effects. Secondary endpoints were reductions in headache and medication intake frequencies by months 3 and 6, response rates, changes in patient-reported outcomes and reasons for discontinuation. As a subanalysis, reduction in monthly migraine days and proportion of adverse effects were also compared among the three monoclonal antibodies. RESULTS: A total of 162 patients were included, median age 68 years (range 65-87), 74.1% women. 42% had dyslipidaemia, 40.3% hypertension, 8% diabetes, and 6.2% previous cardiovascular ischaemic disease. The reduction in monthly migraine days at month 6 was 10.1 ± 7.3 days. A total of 25.3% of patients presented adverse effects, all of them mild, with only two cases of blood pressure increase. Headache and medication intake frequencies were significantly reduced, and patient-reported outcomes were improved. The proportions of responders were 68%, 57%, 33% and 9% for reductions in monthly migraine days ≥ 30%, ≥ 50%, ≥ 75% and 100%, respectively. A total of 72.8% of patients continued with the treatment after 6 months. The reduction in migraine days was similar for the different anti-CGRP treatments, but fewer adverse effects were detected with fremanezumab (7.7%). CONCLUSIONS: Anti-CGRP mAbs are safe and effective treatments in migraine patients over 65 years old in real-life clinical practice.

Oral lacosamide for the treatment of refractory trigeminal neuralgia: A retrospective analysis of 86 cases.

BACKGROUND AND OBJECTIVES: First-line treatment for trigeminal neuralgia (TN) is limited to carbamazepine and oxcarbazepine, and in refractory cases, alternatives are scarce. Lacosamide has been suggested as a valid option. In this study, we describe a series of patients who received oral lacosamide as treatment for TN after first-line drug failure. METHODS: In this retrospective descriptive cohort study, we included patients with refractory TN who attended a tertiary center between 2015 and 2021 and were prescribed oral lacosamide after first-line treatment failure. The primary endpoints were pain relief and adverse effects. We secondarily analyzed clinical outcomes and compared responders versus nonresponders in the search for potential predictors of response. RESULTS: Eighty-six patients were included (mean age: 62 [SD 15.6] years; 54/86 [63%] female). The TN etiology was secondary in 16/86 (19%) patients. Concomitant continuous pain was present in 29/86 (34%) patients. The mean number of previous treatments was 2.7 [SD 1.5]. Pain relief was achieved in 57/86 (66%) cases, with 28/86 (33%) patients presenting adverse effects, all of which were mild. No statistically significant differences were observed between responders and nonresponders, but subtle clinical differences suggested potential predictors of response. CONCLUSION: Lacosamide may be an effective and relatively safe treatment for refractory pain in TN patients after first-line treatment failure.

Multicentric investigation on the safety, feasibility and usability of the ABLE lower-limb robotic exoskeleton for individuals with spinal cord injury: a framework towards the standardisation of clinical evaluations.

BACKGROUND: Robotic lower-limb exoskeletons have the potential to provide additional clinical benefits for persons with spinal cord injury (SCI). However, high variability between protocols does not allow the comparison of study results on safety and feasibility between different exoskeletons. We therefore incorporated key aspects from previous studies into our study protocol and accordingly conducted a multicentre study investigating the safety, feasibility and usability of the ABLE Exoskeleton in clinical settings. METHODS: In this prospective pretest-posttest quasi-experimental study across two SCI centres in Germany and Spain, in- and outpatients with SCI were recruited into a 12-session training and assessment protocol, utilising the ABLE Exoskeleton. A follow-up visit after 4 weeks was included to assess after-training outcomes. Safety outcomes (device-related adverse events (AEs), number of drop-outs), feasibility and usability measures (level of assistance, donning/doffing-time) were recorded at every session together with changes in gait parameters and function. Patient-reported outcome measures including the rate of perceived exertion (RPE) and the psychosocial impact of the device were performed. Satisfaction with the device was evaluated in both participants and therapists. RESULTS: All 24 participants (45 ± 12 years), with mainly subacute SCI (< 1 year after injury) from C5 to L3, (ASIA Impairment Scale A to D) completed the follow-up. In 242 training sessions, 8 device-related AEs (pain and skin lesions) were reported. Total time for don and doff was 6:50 ± 2:50 min. Improvements in level of assistance and gait parameters (time, steps, distance and speed, p < 0.05) were observed in all participants. Walking function and RPE improved in participants able to complete walking tests with (n = 9) and without (n = 6) the device at study start (p < 0.05). A positive psychosocial impact of the exoskeleton was reported and the satisfaction with the device was good, with best ratings in safety (participants), weight (therapists), durability and dimensions (both). CONCLUSIONS: Our study results prove the feasibility of safe gait training with the ABLE Exoskeleton in hospital settings for persons with SCI, with improved clinical outcomes after training. Our study protocol allowed for consistent comparison of the results with other exoskeleton trials and can serve as a future framework towards the standardisation of early clinical evaluations. Trial Registration https://trialsearch.who.int/ , DRKS00023503, retrospectively registered on November 18, 2020.

Control strategies used in lower limb exoskeletons for gait rehabilitation after brain injury: a systematic review and analysis of clinical effectiveness.

BACKGROUND: In the past decade, there has been substantial progress in the development of robotic controllers that specify how lower-limb exoskeletons should interact with brain-injured patients. However, it is still an open question which exoskeleton control strategies can more effectively stimulate motor function recovery. In this review, we aim to complement previous literature surveys on the topic of exoskeleton control for gait rehabilitation by: (1) providing an updated structured framework of current control strategies, (2) analyzing the methodology of clinical validations used in the robotic interventions, and (3) reporting the potential relation between control strategies and clinical outcomes. METHODS: Four databases were searched using database-specific search terms from January 2000 to September 2020. We identified 1648 articles, of which 159 were included and evaluated in full-text. We included studies that clinically evaluated the effectiveness of the exoskeleton on impaired participants, and which clearly explained or referenced the implemented control strategy. RESULTS: (1) We found that assistive control (100% of exoskeletons) that followed rule-based algorithms (72%) based on ground reaction force thresholds (63%) in conjunction with trajectory-tracking control (97%) were the most implemented control strategies. Only 14% of the exoskeletons implemented adaptive control strategies. (2) Regarding the clinical validations used in the robotic interventions, we found high variability on the experimental protocols and outcome metrics selected. (3) With high grade of evidence and a moderate number of participants (N = 19), assistive control strategies that implemented a combination of trajectory-tracking and compliant control showed the highest clinical effectiveness for acute stroke. However, they also required the longest training time. With high grade of evidence and low number of participants (N = 8), assistive control strategies that followed a threshold-based algorithm with EMG as gait detection metric and control signal provided the highest improvements with the lowest training intensities for subacute stroke. Finally, with high grade of evidence and a moderate number of participants (N = 19), assistive control strategies that implemented adaptive oscillator algorithms together with trajectory-tracking control resulted in the highest improvements with reduced training intensities for individuals with chronic stroke. CONCLUSIONS: Despite the efforts to develop novel and more effective controllers for exoskeleton-based gait neurorehabilitation, the current level of evidence on the effectiveness of the different control strategies on clinical outcomes is still low. There is a clear lack of standardization in the experimental protocols leading to high levels of heterogeneity. Standardized comparisons among control strategies analyzing the relation between control parameters and biomechanical metrics will fill this gap to better guide future technical developments. It is still an open question whether controllers that provide an on-line adaptation of the control parameters based on key biomechanical descriptors associated to the patients' specific pathology outperform current control strategies.

Comparing walking with knee-ankle-foot orthoses and a knee-powered exoskeleton after spinal cord injury: a randomized, crossover clinical trial.

Recovering the ability to stand and walk independently can have numerous health benefits for people with spinal cord injury (SCI). Wearable exoskeletons are being considered as a promising alternative to conventional knee-ankle-foot orthoses (KAFOs) for gait training and assisting functional mobility. However, comparisons between these two types of devices in terms of gait biomechanics and energetics have been limited. Through a randomized, crossover clinical trial, this study compared the use of a knee-powered lower limb exoskeleton (the ABLE Exoskeleton) against passive orthoses, which are the current standard of care for verticalization and gait ambulation outside the clinical setting in people with SCI. Ten patients with SCI completed a 10-session gait training program with each device followed by user satisfaction questionnaires. Walking with the ABLE Exoskeleton improved gait kinematics compared to the KAFOs, providing a more physiological gait pattern with less compensatory movements (38% reduction of circumduction, 25% increase of step length, 29% improvement in weight shifting). However, participants did not exhibit significantly better results in walking performance for the standard clinical tests (Timed Up and Go, 10-m Walk Test, and 6-min Walk Test), nor significant reductions in energy consumption. These results suggest that providing powered assistance only on the knee joints is not enough to significantly reduce the energy consumption required by people with SCI to walk compared to passive orthoses. Active assistance on the hip or ankle joints seems necessary to achieve this outcome.

Denervation With Periosteal Resection of the First Carpometacarpal Joint.

Osteoarthritis of the first carpometacarpal joint is a frequent condition that hand surgeons have to deal with. When conservative measures such as physiotherapy, steroid injections, or splinting fail to alleviate symptoms, trapeziectomy is considered the gold standard for surgical treatment. In the present article, a novel technique is presented to denervate the joint capsule together with the periosteum and the endosteum to address sensory receptors located in these 2 extracapsular structures in the proximity of the first carpometacarpal joint area. Denervation with periosteal resection, apart from being a relatively easy and less aggressive technique compared with trapeziectomy, offers satisfactory pain relief with a faster recovery time.

Using a bimanual lever-driven wheelchair for arm movement practice early after stroke: A pilot, randomized, controlled, single-blind trial.

OBJECTIVE: Many patients with subacute stroke rely on the nonparetic arm and leg to propel manual wheelchairs. We designed a bimanual, lever-driven wheelchair (LARA) to promote overground mobility and hemiparetic arm exercise. This study measured the feasibility of using LARA to increase arm movement, achieve mobility, and improve arm motor recovery (clinicaltrials.gov/ct2/show/NCT02830893). DESIGN: Randomized, assessor-blind, controlled trial. SETTING: Two inpatient rehabilitation facilities. SUBJECTS: Nineteen patients with subacute stroke (1 week to 2 months post-stroke) received 30 minutes extra arm movement practice daily, while admitted to inpatient rehabilitation (n = 10) or before enrollment in outpatient therapy (n = 9). INTERVENTIONS: Patients were randomized to train with the LARA wheelchair (n = 11) or conventional exercises with a rehabilitation therapist (n = 8). MAIN MEASURES: Number of arm movements per training session; overground speed; Upper Extremity Fugl-Meyer score at three-month follow-up. RESULTS: Participants who trained with LARA completed 254 (median) arm movements with the paretic arm each session. For three participants, LARA enabled wheelchair mobility at practical indoor speeds (0.15-0.30 m/s). Fugl-Meyer score increased 19 ± 13 points for patients who trained with LARA compared to 14 ± 7 points with conventional exercises (P = 0.32). Secondary measures including shoulder pain and increased tone did not differ between groups. Mixed model analysis found significant interaction between LARA training and treatment duration (P = 0.037), informing power analysis for future investigation. CONCLUSIONS: Practising arm movement with a lever-driven wheelchair is a feasible method for increasing arm movement early after stroke. It enabled wheelchair mobility for a subset of patients and shows potential for improving arm motor recovery.

Systematic review on wearable lower-limb exoskeletons for gait training in neuromuscular impairments.

Gait disorders can reduce the quality of life for people with neuromuscular impairments. Therefore, walking recovery is one of the main priorities for counteracting sedentary lifestyle, reducing secondary health conditions and restoring legged mobility. At present, wearable powered lower-limb exoskeletons are emerging as a revolutionary technology for robotic gait rehabilitation. This systematic review provides a comprehensive overview on wearable lower-limb exoskeletons for people with neuromuscular impairments, addressing the following three questions: (1) what is the current technological status of wearable lower-limb exoskeletons for gait rehabilitation?, (2) what is the methodology used in the clinical validations of wearable lower-limb exoskeletons?, and (3) what are the benefits and current evidence on clinical efficacy of wearable lower-limb exoskeletons? We analyzed 87 clinical studies focusing on both device technology (e.g., actuators, sensors, structure) and clinical aspects (e.g., training protocol, outcome measures, patient impairments), and make available the database with all the compiled information. The results of the literature survey reveal that wearable exoskeletons have potential for a number of applications including early rehabilitation, promoting physical exercise, and carrying out daily living activities both at home and the community. Likewise, wearable exoskeletons may improve mobility and independence in non-ambulatory people, and may reduce secondary health conditions related to sedentariness, with all the advantages that this entails. However, the use of this technology is still limited by heavy and bulky devices, which require supervision and the use of walking aids. In addition, evidence supporting their benefits is still limited to short-intervention trials with few participants and diversity among their clinical protocols. Wearable lower-limb exoskeletons for gait rehabilitation are still in their early stages of development and randomized control trials are needed to demonstrate their clinical efficacy.

Evaluation of an exercise-enabling control interface for powered wheelchair users: a feasibility study with Duchenne muscular dystrophy.

BACKGROUND: Powered wheelchairs are an essential technology to support mobility, yet their use is associated with a high level of sedentarism that can have negative health effects for their users. People with Duchenne muscular dystrophy (DMD) start using a powered wheelchair in their early teens due to the loss of strength in their legs and arms. There is evidence that low-intensity exercise can help preserve the functional abilities of people with DMD, but options for exercise when sitting in a powered wheelchair are limited. METHODS: In this paper, we present the design and the feasibility study of a new version of the MOVit device that allows powered-wheelchair users to exercise while driving the chair. Instead of using a joystick to drive the wheelchair, users move their arms through a cyclical motion using two powered, mobile arm supports that provide controller inputs to the chair. The feasibility study was carried out with a group of five individuals with DMD and five unimpaired individuals. Participants performed a series of driving tasks in a wheelchair simulator and on a real driving course with a standard joystick and with the MOVit 2.0 device. RESULTS: We found that driving speed and accuracy were significantly lowered for both groups when driving with MOVit compared to the joystick, but the decreases were small (speed was 0.26 m/s less and maximum path error was 0.1 m greater). Driving with MOVit produced a significant increase in heart rate (7.5 bpm) compared to the joystick condition. Individuals with DMD reported a high level of satisfaction with their performance and comfort in using MOVit. CONCLUSIONS: These results show for the first time that individuals with DMD can easily transition to driving a powered wheelchair using cyclical arm motions, achieving a reasonable driving performance with a short period of training. Driving in this way elicits cardiopulmonary exercise at an intensity found previously to produce health-related benefits in DMD.

Molecular phenomics of a high-calorie diet-induced porcine model of prepubertal obesity.

As obesity incidence is alarmingly rising among young individuals, we aimed to characterize an experimental model of this situation, considering the similarity between human and porcine physiology. For this reason, we fed prepubertal (63 days old) Duroc breed females (n=21) either with a standard growth diet (3800 kcal/day) or one with a high-calorie content (5200 kcal/day) during 70 days. Computerized tomography, mass-spectrometry-based metabolomics and lipidomics, as well as peripheral blood mononuclear cell transcriptomics, were applied to define traits linked to high-calorie intake. Samples from a human cohort confirmed potential lipidomic markers. Compared to those fed a standard growth diet, pigs fed a high-calorie diet showed an increased weight gain (13%), much higher adiposity (53%), hypertriacylglyceridemia and hypercholesterolemia in parallel to insulin resistance. This diet induced marked changes in the circulating lipidome, particularly in phosphatidylethanolamine-type molecules. Also, circulating specific diacylglycerol and monoacylglycerol contents correlated with visceral fat and intrahepatic triacylglycerol concentrations. Specific lipids associated with obesity in swine (mainly belonging to glycerophospholipid, triacylglyceride and sterol classes) were also linked with obesity traits in the human cohort, reinforcing the usefulness of the chosen approach. Interestingly, no overt inflammation in plasma or adipose tissue was evident in this model. The presented model is useful as a preclinical surrogate of prepubertal obesity in order to ascertain the pathophysiology interactions between energy intake and obesity development.

Perspectives and Consensus among International Orthopaedic Surgeons during Initial and Mid-lockdown Phases of Coronavirus Disease.

With a lot of uncertainty, unclear, and frequently changing management protocols, COVID-19 has significantly impacted the orthopaedic surgical practice during this pandemic crisis. Surgeons around the world needed closed introspection, contemplation, and prospective consensual recommendations for safe surgical practice and prevention of viral contamination. One hundred orthopaedic surgeons from 50 countries were sent a Google online form with a questionnaire explicating protocols for admission, surgeries, discharge, follow-up, relevant information affecting their surgical practices, difficulties faced, and many more important issues that happened during and after the lockdown. Ten surgeons critically construed and interpreted the data to form rationale guidelines and recommendations. Of the total, hand and microsurgery surgeons (52%), trauma surgeons (32%), joint replacement surgeons (20%), and arthroscopy surgeons (14%) actively participated in the survey. Surgeons from national public health care/government college hospitals (44%) and private/semiprivate practitioners (54%) were involved in the study. Countries had lockdown started as early as January 3, 2020 with the implementation of partial or complete lifting of lockdown in few countries while writing this article. Surgeons (58%) did not stop their surgical practice or clinics but preferred only emergency cases during the lockdown. Most of the surgeons (49%) had three-fourths reduction in their total patients turn-up and the remaining cases were managed by conservative (54%) methods. There was a 50 to 75% reduction in the number of surgeries. Surgeons did perform emergency procedures without COVID-19 tests but preferred reverse transcription polymerase chain reaction (RT-PCR; 77%) and computed tomography (CT) scan chest (12%) tests for all elective surgical cases. Open fracture and emergency procedures (60%) and distal radius (55%) fractures were the most commonly performed surgeries. Surgeons preferred full personal protection equipment kits (69%) with a respirator (N95/FFP3), but in the case of unavailability, they used surgical masks and normal gowns. Regional/local anesthesia (70%) remained their choice for surgery to prevent the aerosolized risk of contaminations. Essential surgical follow-up with limited persons and visits was encouraged by 70% of the surgeons, whereas teleconsultation and telerehabilitation by 30% of the surgeons. Despite the protective equipment, one-third of the surgeons were afraid of getting infected and 56% feared of infecting their near and dear ones. Orthopaedic surgeons in private practice did face 50 to 75% financial loss and have to furlough 25% staff and 50% paramedical persons. Orthopaedics meetings were cancelled, and virtual meetings have become the preferred mode of sharing the knowledge and experiences avoiding human contacts. Staying at home, reading, and writing manuscripts became more interesting and an interesting lifestyle change is seen among the surgeons. Unanimously and without any doubt all accepted the fact that COVID-19 pandemic has reached an unprecedented level where personal hygiene, hand washing, social distancing, and safe surgical practices are the viable antidotes, and they have all slowly integrated these practices into their lives. Strict adherence to local authority recommendations and guidelines, uniform and standardized norms for admission, inpatient, and discharge, mandatory RT-PCR tests before surgery and in selective cases with CT scan chest, optimizing and regularizing the surgeries, avoiding and delaying nonemergency surgeries and follow-up protocols, use of teleconsultations cautiously, and working in close association with the World Health Organization and national health care systems will provide a conducive and safe working environment for orthopaedic surgeons and their fraternity and also will prevent the resurgence of COVID-19.

Retinal findings in pediatric patients with Usher syndrome Type 1 due to mutations in MYO7A gene.

PURPOSE: To describe retinal alterations detected by swept-source optical coherence tomography (SS-OCT) in paediatric patients with Usher syndrome type 1 (USH1) and to compare these findings to previously published reports. METHODS: Thirty-two eyes from 16 patients (11 males and 5 females) with a genetic diagnosis of USH1 because of MYO7A mutations underwent SS-OCT. Patients ranged in age from 4 to 17 years (mean, 11,13 ± 4,29). The subfoveal and macular area were analysed with SS-OCT at 1050 nm using 12 radial scans of 12.0 mm. Structural abnormalities were evaluated and correlated with best-corrected visual acuity (BCVA). RESULTS: The most common qualitative retinal abnormality was external layer damage in macular area. Specific alterations included external limiting membrane loss/disruption (27 eyes; 84.4%), disruption of the Myoid zone (27 eyes; 84.4%); Ellipsoid zone disruption (28 eyes; 87.5%), and loss of the outer segments (29 eyes; 90.6%). The damage of the retinal pigment epithelium was divided according to the loss of the different layers: phagosome zone (30 eyes; 93.8%), melanosome zone (29 eyes; 90.6%) and mitochondria zone (0 eyes; 0%). The presence of cystoid macular oedema (CMO) was significantly correlated with alterations in photoreceptors. Disruption or absence of the myoid and ellipsoid zones of the photoreceptors were the only variables independently associated with decreased BCVA. CONCLUSIONS: The findings of this study suggest that the physiopathologic basis of early-stage Usher syndrome (USH) may be changes in the outer retinal layer, particularly the photoreceptors, which in turn may cause alterations-such as CMO-in the inner retinal layers. Accordingly, monitoring the condition of photoreceptors during follow-up may be advisable for the early detection of pathologic changes.

Development and Evaluation of MOVit: An Exercise-Enabling Interface for Driving a Powered Wheelchair.

Powered wheelchair users can experience negative health effects from reduced physical activity. If a user could exercise by driving the chair, it might improve fitness. This paper presents the development of MOVit, an exercise-enabling, wheelchair driving interface. The design goal of MOVit was that users cyclically move their arms to drive the chair, thereby providing a light level of exercise while driving. MOVit supports this arm movement with custom mobile arm supports that also serve as the sensors that provide controller inputs. Here, we first quantified how increasing the frequency and amplitude of arm movement increase oxygen consumption and heart rate. Then, we evaluated two novel control methods for driving by moving the arm supports. Participants without impairment ( N = 24 ) were randomized to one of the two methods, or conventional joystick control, and performed driving tests over two days on a simulator and test course. Our results indicate that driving speed and accuracy were significantly lowered with the exercise-enabling methods compared to joystick control (ANOVA, ), but the decreases were small (speed was ~0.1 m/s less and course tracking error ~1 cm greater). These results show, for the first time, the feasibility of exercising while driving a powered wheelchair.

Evaluation of Control Interfaces for Active Trunk Support.

A feasibility study was performed to evaluate the control interfaces for a novel trunk support assistive device (Trunk Drive), namely, joystick, force on sternum, force on feet, and electromyography (EMG) to be used by adult men with Duchene muscular dystrophy. The objective of this paper was to evaluate the performance of the different control interfaces during a discrete position tracking task. We built a one degree of freedom flexion-extension active trunk support device that was tested on 10 healthy men. An experiment, based on the Fitts law, was conducted, whereby subjects were asked to steer a cursor representing the angle of the Trunk Drive into a target that was shown on a graphical user interface, using the above-mentioned control interfaces. The users could operate the Trunk Drive via each of the control interfaces. In general, the joystick and force on sternum were the fastest in movement time (more than 40%) without any significant difference between them, but there was a significant difference between force on sternum on the one hand, and EMG and force on feet on the other. All control interfaces proved to be feasible solutions for controlling an active trunk support, each of which had specific advantages.

There is plenty of room for motor learning at the bottom of the Fugl-Meyer: Acquisition of a novel bimanual wheelchair skill after chronic stroke using an unmasking technology.

Many people with a stroke have a severely paretic arm, and it is often assumed that they are unable to learn novel, skilled behaviors that incorporate use of that arm. Here, we show that a group of people with chronic stroke (n = 5, upper extremity Fugl-Meyer scores: 31, 30, 26, 22, 8) learned to use their impaired arm to propel a novel, yoked-clutch lever drive wheelchair. Over six daily training sessions, each involving about 134 training movements with their "useless" arm, the users gradually achieved a 3-fold increase in wheelchair speed on average, with a 4-6 fold increase for three of the participants. They did this by learning a bimanual skill: pushing the levers with both arms while activating the yoked-clutches at the right time with their ipsilesional (i.e. "good") hand to propel the wheelchair forward. They perceived the task as highly motivating and useful. The speed improvements exceeded a 1.5-factor improvement observed when young, unimpaired users learned to propel the chair. The learning rate also exceeded a sample of learning rates from a variety of classic learning studies. These results suggest that appropriately-designed assistive technologies (or "unmasking technologies - UTs") can unleash a powerful, latent ability for motor learning even for severely paretic arms. While UTs may not reduce clinical impairment, they may facilitate large improvements in a specific functional ability.