[Uroflowmetry: A follow-up tool for neurogenic bladder patients treated by transcutaneous tibial posterior stimulation?] / Débitmétrie : outil de suivi chez les patients avec vessie neurologique traités par neurostimulation tibiale postérieure ?

OBJECTIVE: Neurogenic bladders can suffer from overactivity, underactivity or dyssynergia depending on the level of the initial lesion. These symptoms can lead to severe alterations of the upper urinary tract. One of the first-line treatments is the transcutaneous tibial posterior stimulation (TTNS), which was demonstrated to be efficient on urodynamics. But it is an invasive, expensive and sometimes not patient-accepted examination, contrary to the uroflowmetry. The aim of this study is to assess the feasibility of a follow-up with a uroflowmetry when treated by TTNS and show that the maximum flow rate increased after treatment, displaying a better detrusor contraction. METHODS: In total, 38 patients with neurogenic bladder undergoing a 12-weeks TTNS treatment and with 2 uroflowmetries interpretable before and after treatment were included. The maximum flow rate (Qmax), the urinated volume and the post-void residual (PVR) were retrieved from the uroflowmetry, and the USP-score and the urinary discomfort were asked at each appointment. RESULTS: Qmax is increased from 17,53ml/s to 18,26ml/s, as well as the PVR (from 76,97ml to 79,16ml). Urinated volume is decreased from 241,4ml to 193,66ml. Patients feel enhanced after TTNS according to the decrease in the USP-score and the urinary discomfort scale. CONCLUSION: The increase of the cystomanometric capacity and the delay of the detrusor overactivity due to TTNS explains the reduction of the urinated volume and the increase of PVR. Increased Qmax might show a better voluntary bladder contraction, with a restraint due to the lack of abdominal pressure measurement during voiding.

Visual feedback improves movement illusions induced by tendon vibration after chronic stroke.

BACKGROUND: Illusion of movement induced by tendon vibration is commonly used in rehabilitation and seems valuable for motor rehabilitation after stroke, by playing a role in cerebral plasticity. The aim was to study if congruent visual cues using Virtual Reality (VR) could enhance the illusion of movement induced by tendon vibration of the wrist among participants with stroke. METHODS: We included 20 chronic stroke participants. They experienced tendon vibration of their wrist (100 Hz, 30 times) inducing illusion of movement. Three VR visual conditions were added to the vibration: a congruent moving virtual hand (Moving condition); a static virtual hand (Static condition); or no virtual hand at all (Hidden condition). The participants evaluated for each visual condition the intensity of the illusory movement using a Likert scale, the sensation of wrist's movement using a degree scale and they answered a questionnaire about their preferred condition. RESULTS: The Moving condition was significantly superior to the Hidden condition and to the Static condition in terms of illusion of movement (p < 0.001) and the wrist's extension (p < 0.001). There was no significant difference between the Hidden and the Static condition for these 2 criteria. The Moving condition was considered the best one to increase the illusion of movement (in 70% of the participants). Two participants did not feel any illusion of movement. CONCLUSIONS: This study showed the interest of using congruent cues in VR in order to enhance the consistency of the illusion of movement induced by tendon vibration among participants after stroke, regardless of their clinical severity. By stimulating the brain motor areas, this visuo-proprioceptive feedback could be an interesting tool in motor rehabilitation. Record number in Clinical Trials: NCT04130711, registered on October 17th 2019 ( https://clinicaltrials.gov/ct2/show/NCT04130711?id=NCT04130711&draw=2&rank=1 ).

Risk of prolapse and urinary complications in adult spina bifida patients with neurogenic acontractile detrusor using clean intermittent catheterization versus Valsalva voiding.

AIMS: To assess the relative risks of pelvic organ prolapse (POP) and urinary complications in adult spina bifida patients with neurogenic acontractile detrusor voiding with Valsalva versus those using clean-intermittent catheterization (CIC). METHODS: We conducted a retrospective analysis including all spina bifida patients with neurogenic acontractile detrusor with a minimum follow-up of 12 months. Patients were then divided in two groups according to their bladder management: voiding with Valsalva versus CIC. The primary endpoint was any de novo or worsened rectal and/or pelvic organ prolapse (POP) diagnosed during follow-up. The secondary outcome was urinary complications defined as febrile urinary tract infections (UTI) and/or urolithiasis and/or renal failure. RESULTS: Fifty-five patients (50.9% were males) met the inclusion/exclusion criteria: 28 voiding with Valsalva and 27 performing CIC. At baseline, the rates of vaginal prolapse (44.4% vs 50%; P = 0.99), and rectal prolapse/intussusception (25.9% vs 21.4%; P = 0.76) were similar in both groups. After a median follow-up of 80.6 and 65.6 months, respectively (P = 0.29), the rate of de novo or worsened rectal prolapse/intussusception was higher in the Valsalva voiding group than in the CIC group (32.1% vs 3.7%; P = 0.01). De novo or worsened vaginal prolapses were also more common in the Valsalva voiding group, but it did not reach statistical significance (33.3% vs 11.1%; P = 0.29). CONCLUSIONS: Valsalva voiding might be harmful in adult spina bifida patients with neurogenic acontractile detrusor as it may increase the risk of rectal prolapse/intussusception. Overall, the prevalence of POP and rectal prolapse was high in both groups.

Disturbances of spatial reference frame and postural asymmetry after a chronic stroke.

Asymmetrical postural behaviors are frequently observed after a stroke. They are due in part to the sensorimotor deficit, but they could also be related to a disorder of the representation of the body in space. The objective was to determine whether the asymmetrical postural behaviors of chronic stroke patients are related with a disruption of the perception of spatial frame. 30 chronic stroke patients (mean age 60.3 year ± 10, mean delay post-stroke 4.78 year ± 3), 15 patients with right brain damage (RBD) and 15 patients with left brain damage (LBD), and 20 healthy subjects participated in the study. Postural asymmetry was detected by the evaluation of body weight repartition on a force platform (weight body asymmetry) and was related to the longitudinal body axis (LBA) and the subjective straight ahead (SSA) (egocentric space representation) and to the subjective visual vertical (SVV) (allocentric space representation) by a multivariate analysis of variance adjusted with motor function and sensitivity as covariables. Both patients with RBD (35% ± 8) and LBD (39% ± 4) had body weight asymmetry and there was still space misperception at this stage of recovery, especially in the RBD group. WBA was related to LBA when considering both patients with RBD and LBD (p = 0.03). However, this relation was dependent on the side of the lesion (p = 0.0006) with a stronger relation in the RBD group (0.01). No relation with WBA was found neither with SSA (p = 0.58) nor with SVV (p = 0.47). This study pointed out a strong relationship between disturbance in the perception of the longitudinal body axis and postural asymmetry in chronic strokes, and especially within the RBD group. Conversely, no other spatial perturbations seemed to be involved in this particular postural behavior.

Comparison of neurogenic lower urinary tract dysfunctions in open versus closed spinal dysraphism: A prospective cross-sectional study of 318 patients.

AIMS: To compare the neurogenic lower urinary tract dysfunction (NLUTD) in patients with closed spinal dysraphism (CSD) versus patients with open spinal dysraphism (OSD) as well as their management patterns. METHODS: A prospective cross-sectional study was conducted between September 2007 and December 2015 including all spina bifida patients seen at the multidisciplinary French national referral center for spina bifida. NLUTD and its management were compared between the OSD and CSD groups. RESULTS: Three hundred and eighteen patients were included for analysis: 100 with a CSD (31.5%) and 218 with an OSD (68.6%). The prevalence of urinary incontinence did not differ significantly between the two groups (43% vs 52.8%; P = 0.11), the mean Qualiveen score was also similar (2.7 vs 2.5, P = 0.22). The voiding mechanism was clean intermittent catheterization, spontaneous voiding, suprapubic tube, and ileal conduit in 55% versus 44%; 29.8% versus 47%; 2.8% versus 3% and 11.9% versus 6% of OSD and CSD patients, respectively (P = 0.02). There were comparable prevalences of detrusor overactivity (36.5% vs 38.8%; P = 0.68) and impaired bladder compliance (34.9% vs 31.7%; P = 0.56) in both groups. Augmentation cystoplasty was more common in patients with OSD (32.1% vs 11%; P < 0.0001). CONCLUSIONS: In this prospective cohort, NLUTD were more common in OSD with a higher rate of patients requiring a surgical treatment and a lower rate of patients with preserved spontaneous voiding. However, when present, NLUTD was as severe and troublesome in patients with closed versus open spinal dysraphism.

Kinematic motion abnormalities and bimanual performance in children with unilateral cerebral palsy.

AIM: To evaluate the relationship between the movement abnormalities of the impaired upper limb in children with unilateral cerebral palsy (CP) and bimanual performance. METHOD: Twenty-three children with unilateral CP (mean age 11y 10mo [SD 2y 8mo]) underwent evaluation of bimanual performance (Assisting Hand Assessment [AHA]) and a three-dimensional movement analysis to measure deviations in the movement of their affected upper limb, and compared with 23 typically developing children (TDC) (mean age 11y 11mo [SD 2y 5mo]). Kinematic indices, such as the Global Arm Profile Score (APS), which summarizes the global movement deviation of the upper limb from the norm, and the Global Arm Variable Score (AVS), which represent movement deviations for a given joint, were calculated and correlated to AHA. RESULTS: Values of kinematic indices were significantly higher in children with unilateral CP than in TDC. A strong correlation between Global-APS and AHA score (r=-0.75) was found. Other significant correlations were found with Global-AVS, especially in distal joints. INTERPRETATION: Children with unilateral CP had more movement deviations than TDC. The global movement deviation of the impaired upper limb was strongly correlated with bimanual performance. The influence of distal abnormalities confirms the importance of considering these limitations in therapeutics. WHAT THIS PAPER ADDS: Children with unilateral cerebral palsy had more movement deviations than typically developing children in unimanual tasks. A strong relationship was found between movement deviations of the impaired upper limb and bimanual performance.

Outcomes of intra-detrusor injections of botulinum toxin in patients with spina bifida: A systematic review.

CONTEXT: Bladder management in spina bifida patients relies on clean intermittent catheterization and oral antimuscarinics with a significant failure rate. The efficacy of intradetrusor injections of botulinum toxin has been confirmed in patients with spinal cord injury or multiple sclerosis but not in patients with myelomeningocele. OBJECTIVE: To conduct a systematic review of current evidence regarding the efficacy of intra-detrusor injections of Botulinum Toxin A (BTX-A) in spina bifida patients with neurogenic detrusor overactivity (NDO) refractory to antimuscarinics. METHODS: A research has been conducted on Medline and Embase using the keywords: ("spina bifida" OR "myelomeningocele" OR "dysraphism") AND "toxin." The search strategy and studies selection were performed using the PICOS method according to the PRISMA statement. RESULT: Twelve published series were included (n = 293 patients). All patients were <18 years old. There was no randomized study comparing BTX-A versus placebo and most studies had no control group. Most studies reported a clinical and urodynamic improvement with resolution of incontinence in 32-100% of patients, a decrease in maximum detrusor pressure from 32 to 54%, an increase of maximum cystometric capacity from 27 to 162%, and an improvement in bladder compliance of 28-176%. Two studies suggested lower efficacy in patients with low compliance bladder compared to those with isolated detrusor overactivity. CONCLUSION: Intradetrusor injections of BTX-A could be effective in children with spina bifida but this assumption is not supported by high level of evidence studies. There is no data available in adult patients. Neurourol. Urodynam. 36:557-564, 2017. © 2016 Wiley Periodicals, Inc.

Short-term effect of neck muscle vibration on postural disturbances in stroke patients.

Balance disorders after stroke have a particularly detrimental influence on recovery of autonomy and walking. The present study is aimed at assessing the effect of proprioceptive stimulation by neck muscle vibration (NMV) on the balance of patients with right hemispheric lesion (RHL) and left hemispheric lesion (LHL). Thirty-one (31) patients (15 RHL and 16 LHL), mean age 61.5 years (±10.6), mean delay 3.1 (±1.6) months after one hemispheric stroke were included in this prospective study. The mean position in mediolateral and anteroposterior plane of the CoP (center of pressure) and the surface were evaluated using a force platform at rest and immediately after 10 min of vibration on the contralesional dorsal neck muscle. NMV decreases the lateral deviation balance induced by the stroke. Twenty patients (64.5 %) experienced a visual illusion of light spot moving toward the side opposite stimulus. These patients showed more improvement by vibration than those without visual illusion. There was an interaction between sensitivity and side of stroke on the effect of NMV. Proprioceptive stimulation by NMV reduces postural asymmetry after stroke. This short-term effect of the vibration is more effective in patients susceptible to visual illusion. This result was consistent with a central effect of NMV on the structures involved in the elaboration of perception of body in space.

Diagnosis and treatment of anteroinferior capsular redundancy associated with anterior shoulder instability using an open Latarjet procedure and capsulorrhaphy.

PURPOSE: There is a paucity of data detailing management of anterior capsular redundancy (ACR) when using the Latarjet procedure for unidirectional instability. This study aimed to describe the surgical management and to assess the clinical profile of patients presenting with anterior capsular redundancy [ACR(+)] with anterior shoulder instability. METHODS: Seventy-seven patients who had a Latarjet procedure were followed for a 55-month period. Per-operative ACR was assessed during surgery. ACR was considered present if the inferior capsular flap of a Neer T-shaft capsulorrhaphy was able to cover the superior capsular flap with the arm in the neutral position. Patients with ACR(+) received an additional Neer capsulorrhaphy, while patients with ACR(-) did not. This per-operative finding was correlated with demographics, clinical, radiological pre-operative data and surgical outcome. RESULTS: Patients presenting with a per-operative ACR(+) were significantly associated with a sulcus sign (P < 0.001), a Beighton score >4 (P < 0.01), a low-energy instability history (P < 0.05), a predominant history of subluxations (P < 0.05), fewer Hill-Sachs lesion (P < 0.05) and a female gender (P < 0.05), but not significantly with external rotation >85°. Open standard Latarjet procedures with Neer capsulorrhaphy in ACR(+) patients showed excellent or good results and stability rate of 95 %. All patients except four who presented with a new dislocation after surgery were satisfied with their outcome. Thirteen patients (16 %) had a persistent apprehension sign at the last follow-up. ACR(+) and ACR(-) groups did not show significant difference in the mean values of Rowe, Walch-Duplay and Constant-Murley scores. CONCLUSION: ACR correlated with a sulcus sign, Beighton score and instability history. In anterior shoulder instability associated with ACR, the Latarjet procedure with a Neer capsulorrhaphy appears a satisfactory treatment alternative to arthroscopic or open capsular shift. It decreased apprehension in comparison with Latarjet procedures without capsular repair. LEVEL OF EVIDENCE: Cases series, treatment study, Level IV.

Volumetric definition of shoulder range of motion and its correlation with clinical signs of shoulder hyperlaxity. A motion capture study.

BACKGROUND: Shoulder hyperlaxity (SHL) is assessed with clinical signs. Quantification of SHL remains difficult, however, because no quantitative definition has yet been described. With use of a motion capture system (MCS), the aim of this study was to categorize SHL through a volumetric MCS-based definition and to compare this volume with clinical signs used for SHL diagnosis. METHOD: Twenty-three subjects were examined with passive and active measurement of their shoulder range of motion (SROM) and then with an MCS protocol, allowing computation of the shoulder configuration space volume (SCSV). Clinical data of SHL were assessed by the sulcus sign, external rotation with the arm at the side (ER1) >85° in a standing position, external rotation >90° in a lying position, and Beighton score for general joint laxity. Active and passive ER1, EIR2 (sum of external and internal rotation at 90° of abduction), flexion-extension, and abduction were also measured and correlated to SCSV. RESULTS: Except for the sulcus sign, SCSV was significantly correlated with all clinical signs used for SHL. Passive examination of the different SROMs was better correlated to SCSV than active examination. In passive examination, the worst SROM was ER1 (R = 0.36; P = .09), whereas EIR2, flexion, and abduction were highly correlated to SCSV (P < .01). CONCLUSION: SCSV appears to be an appealing tool for evaluation of SHL regarding its correlation with clinical signs used for SHL diagnosis. The sulcus sign and ER1 >85° in a standing position appear less discriminating and should be replaced by EIR2 measurement for SHL diagnosis.

Long-lasting reduction in postural asymmetry by prism adaptation after right brain lesion without neglect.

BACKGROUND: Right brain damage (RBD) involves postural asymmetry and spatial frame disorders. In acute RBD patients, postural asymmetry is immediately reduced after one single session of prism adaptation (PA), without assessment of effects on spatial frames. AIM: To assess long-term effects of PA on posture and spatial frames in chronic RBD patients, without neglect. METHOD: Six chronic RBD patients without neglect (mean delay 45 months) were included. Each patient sustained 10 PA sessions of 20 min during 2 weeks. Outcome measures were: (1) posturographic analysis (mediolateral position of centre of pressure (X cop), (2) subjective straight ahead (SSA) and perception of longitudinal body axis (LBA). Each parameter was assessed by three pretests and three post-tests (+2 h, day + 3 and day + 7). RESULTS: In pretests, patients showed a shift of the X cop and SSA. In post-tests, results displayed (1) a significant reduction in mediolateral postural asymmetry at D + 7; (2) a significant left deviation of SSA at D + 3 and enduring at D + 7; and (3) no significant modification of LBA. The mean curves of X cop and SSA between pre- and post-tests were similar. CONCLUSIONS: PA involves persistent reduction in postural asymmetry in RBD patients without neglect. These findings were obtained at a chronic stage. This new effect cannot be explained by reduction in spatial attentional shift. Improvement may be explained by a better calibration of extra personal space frames used for posture, without effect on personal space frame. Findings argue in favour of a bottom-up effect of PA on mechanisms underlying spatial cognition.

Satisfaction survey toward an innovative orthosis for children with cerebral palsy with pes planovalgus.

BACKGROUND: Pes planovalgus (PPV) is common in children with cerebral palsy and can cause pain and gait alterations over time. Initial treatment of flexible PPV includes orthotics, despite a lack of consensus on the type of orthosis. We developed an innovative ankle-foot orthosis (RAFO). RAFO is a one-piece orthosis designed to correct both the valgus and the flat of the foot. Its conception situated above the malleolar enables a moderate anti equinus effect. Its precise description and fabrication's process is detailed. OBJECTIVES: We wanted to assess user's satisfaction after several months of use and looked for clinical criteria of satisfaction. STUDY DESIGN: Retrospective. METHODS: We conducted a satisfaction study through a telephonic QUEST (Quebec User Evaluation of Satisfaction with Assistive Technology) survey analysing the device and the process in children and parents. 24 parents were contacted, whose child was wearing RAFO on a daily basis for at least 4 months. 20 parents answered the questionnaire. The results were then related to clinical data to research satisfaction' criteria. RESULTS: Mean QUEST satisfaction was 4.25/5 (4.18/5 for device and 4.38/5 for process). Advantages reported concerned weight and dimensions (95%), although 20% reported the necessity to change to a shoe size above, ease of use (90%) and level of comfort (80%). Parent's satisfaction regarding perceived effectiveness was 80%. Children with equinus due to triceps surae spasticity were more susceptible to develop pain with our orthosis. CONCLUSIONS: Parents were overall very satisfied with the orthosis. Its technical characteristics allowing both to be a corrector of flat foot and valgus and at the same time to be comfortable and discreet makes it innovative. The presence of spastic equinus is a limit for its utilization.

A systematic review of rehabilitation programs for cognitive impairment related to breast cancer: Different programs at different times?

BACKGROUND: "Cancer Related Cognitive Impairment" (CRCI) defines cognitive disorders related to cancer and its treatments. Many people with breast cancer experience signs of CRCI (incidence between 20 and 30 %) and, although several intervention options exist, there is no established standard of care. Our main objective was to provide a detailed description of the methods and results of randomized controlled trials of interventions for CRCI in breast cancer survivors, paying particular attention to the timing of the interventions within the care pathway. METHODS: We conducted a systematic literature review following the PRISMA guidelines from 01 to 01-2019 to 16-07-2023 and included randomized controlled trials of interventions for CRCI after breast cancer with at least one objective cognitive assessment as a primary or secondary outcome. RESULTS: Among 228 identified studies, 35 (including 2821 participants) were retained for inclusion. The interventions were classified into 4 categories: cognitive rehabilitation, physical activity, complementary therapy and pharmacological treatment. Our analysis revealed that pharmacological interventions have no effect, whereas physical activity interventions proposed in the months following the initial cancer treatment improve Quality of Life and Speed of Information Processing while interventions proposed later improve Memory and Attention (Cognitive Rehabilitation) and Perceived Cognitive Function and Depression/Anxiety/Mood (Complementary Therapies). CONCLUSION: CRCI is multifactorial and affected individuals frequently experience high levels of fatigue, pain, anxiety and depression and are most likely to benefit from holistic approaches that include cognition, physical activity, relaxation, psychoeducation, group support and/or psychological counselling. Thus, rehabilitation programs should be designed on multi-modal approaches, using innovative, cost-effective delivery methods that increase access to treatment, and intervention outcomes should be evaluated using measures of participation. DATABASE REGISTRATION: The review was recorded on Prospero (01-03-2020), with the registration number 135,627.

Validity and reliability of the chronic composite XA, an upper limb motor assessment using Active Range of Motion in patients with chronic stroke.

BACKGROUND: Upper limb (UL) spastic paresis has been classically evaluated with assessments of passive movements with limited functional validity. The aim of this study was to assess whether a composite measure of active range of motion (AROM, or XA) is valid and reliable in chronic post-stroke spastic paresis. AIM: The primary objective was to investigate the validity and reliability of a composite score, comprising multiple XA measurements, to assess UL spastic paresis in patients in chronic stages post-stroke. In addition to this, an exploratory analysis was conducted to identify which muscles should be optimally included in this composite score. DESIGN: A psychometric proprieties study. SETTING: Physical and Rehabilitation Medicine Department. POPULATION: twenty-eight chronic post-stroke participants with spastic paresis. METHODS: Composite UL XA measurement in twenty-eight chronic post-stroke participants (age=59±11 years; delay post-stroke=29±37 months) with spastic paresis was repeated twice about 40 days apart in a standardized body position. Concurrent and construct validity was evaluated exploring correlation with the Fugl-Meyer Assessment Upper Extremity (FMA-UE), Action Research Arm Test (ARAT), and grip strength (JAMAR™). Reliability was assessed by calculating intraclass correlation coefficients (ICC). Regarding the exploratory analysis, a linear regression analysis was performed to examine the value of including various muscles. RESULTS: Composite XA against the resistance of elbow, wrist and finger flexors showed strong correlation with FMA-UE and ARAT (r=0.88; P<0.001 and r=0.82; P<0.001 respectively) and a weak association with grip strength (r=0.43; P=0.03). Test-retest reliability was excellent (ICC=0.92). However, the most effective regression model also included XA against the resistance of shoulder adductors as well as forearm pronator (adjusted R2=0.85; AIC=170). CONCLUSIONS: The present study provided satisfactory psychometric data for the upper limb composite active movement (CXA), derived from the Five Step Assessment. For overall measurement of UL mobility after stroke, we strongly recommend including shoulder and forearm muscles to the score. CLINICAL REHABILITATION IMPACT: Composite XA is a valid and reliable measure of upper limb motor function in chronic post-stroke patients and could be used in clinical practice and research.

Phenotype variability and natural history of X-linked myopathy with excessive autophagy.

OBJECTIVE: X-linked myopathy with excessive autophagy (XMEA) linked to the VMA21 gene leads to autophagy failure with progressive vacuolation and atrophy of skeletal muscles. Current knowledge of this rare disease is limited. Our objective was to define the clinical, radiological, and natural history of XMEA. METHODS: We conducted a retrospective study collecting clinical, genetic, muscle imaging, and biopsy data of XMEA patients followed in France and reviewed the literature for additional cases. RESULTS: Eighteen males had genetically confirmed XMEA in France, carrying four different VMA21 variants. Mean age at disease onset was 9.4 ± 9.9 (range 1-40) years. In 14/18 patients (77.8%), onset occurred during childhood (< 15 years); however in four patients, the disease started in adulthood. Patients had anterior and medial compartment thigh muscle weakness, distal contractures (56.3%), elevated CK levels (1287.9 ± 757.8 U/l) and autophagic vacuoles with sarcolemmal features on muscle histopathology. Muscle MRI (n = 10) showed a characteristic pattern of lower limb muscle involvement. In 11 patients, outcome measures were available for an average follow-up period of 10.6 ± 9.8 years and six of them show disease progression. Mean change of functional outcomes was 0.5 ± 1.2 points for Brooke and 2.2 ± 2.5 points for Vignos score, 7/16 patients (43.8%) needed a walking aid and 3/16 (18.8%) were wheelchair-bound (median age of 40 years old, range 39-48). The variant c.164-7 T > G was associated with a later onset of symptoms. Respiratory insufficiency was common (57.1%) but cardiac involvement rare (12.5%). INTERPRETATION: XMEA has variable age of onset, but a characteristic clinical, histopathological, and muscle imaging presentation, guiding the diagnosis. Although slowly, motor disability progresses with time, and relevant genotype-phenotype correlations will help design future clinical trials.

Influence of User Posture and Virtual Exercise on Impression of Locomotion During VR Observation.

A seated user watching his avatar walking in Virtual Reality (VR) may have an impression of walking. In this paper, we show that such an impression can be extended to other postures and other locomotion exercises. We present two user studies in which participants wore a VR headset and observed a first-person avatar performing virtual exercises. In the first experiment, the avatar walked and the participants (n=36) tested the simulation in 3 different postures (standing, sitting and Fowler's posture). In the second experiment, other participants (n=18) were sitting and observed the avatar walking, jogging or stepping over virtual obstacles. We evaluated the impression of locomotion by measuring the impression of walking (respectively jogging or stepping) and embodiment in both experiments. The results show that participants had the impression of locomotion in either sitting, standing and Fowler's posture. However, Fowler's posture significantly decreased both the level of embodiment and the impression of locomotion. The sitting posture seems to decrease the sense of agency compared to standing posture. Results also show that the majority of the participants experienced an impression of locomotion during the virtual walking, jogging, and stepping exercises. The embodiment was not influenced by the type of virtual exercise. Overall, our results suggest that an impression of locomotion can be elicited in different users' postures and during different virtual locomotion exercises. They provide valuable insight for numerous VR applications in which the user observes a self-avatar moving, such as video games, gait rehabilitation, training, etc.

Reliability and minimal detectable change of body-weight distribution and body sway between right and left brain-damaged patients at a chronic stage.

PURPOSE: To assess the reliability and minimal detectable change (MDC) of weight-bearing asymmetry (WBA) and body sway (BS) during "eyes open" (EO) and "eyes closed" (EC) conditions for those with right brain damage (RBD) and left brain damage (LBD) at a chronic stage. METHODS: Sixteen RBD and 16 LBD patients participated in two sessions within 15 days, composed of two trials of 30 s using a double force platform. Intraclass correlation coefficient (ICC2,1), the standard error of measurement (SEM), and MDC were calculated for WBA and BS (area and velocity of sway). RESULTS: Reliability of WBA was excellent (>0.75) except for EC for LBD patients (low SEM was found). The condition of EC was similar to or less reliable than that of EO. The MDC of WBA was 5.4 and 7.3% for LBD and RBD patients, respectively. Velocity of sway should be favored over the area of sway due to better reliability, with an MDC of 9 and 13 mm/s for RBD and LBD patients, respectively. CONCLUSIONS: Parameters related to WBA and BS were highly reliable, without a difference between RBD and LBD patients, but less so in the condition of EC, and could be used for clinical rehabilitation and/or research.Implications for rehabilitationWeight-bearing asymmetry (WBA) and body sway (BS) are highly reliable posturography parameters.Reliability of WBA/BS is similar among right brain damaged (RBD) and left brain damaged (LBD) patients.A change of 5-7% can be interpreted as significant for WBA for chronic stroke.The minimal detectable change in measures is slightly higher for RBD patients.

Effect of Vibrations on Impression of Walking and Embodiment With First- and Third-Person Avatar.

We investigate how underfoot vibrotactile feedback can be used to increase the impression of walking and embodiment of static users represented by a first- or third-person avatar. We designed a multi-sensory setup involving avatar displayed on an HMD, and a set of vibrotactile effects displayed at every footstep. In a first study (N = 44), we compared the impression of walking in 3 vibrotactile conditions : 1) with a "constant" vibrotactile rendering reproducing simple contact information, 2) with a more sophisticated "phase-based" vibrotactile rendering the successive contacts of a walking cycle and 3) without vibrotactile feedback. The results show that overall both constant and phase-based rendering significantly improve the impression of walking in first and third-person perspective. Interestingly, the more realistic phase-based rendering seems to increase significantly the impression of walking in the third-person condition, but not in the first-person condition. In a second study (N=28), we evaluated the embodiment towards first- and third-person avatar while receiving no vibrotactile feedback or by receiving vibrotactile feedback. The results show that vibrotactile feedback improves embodiment in both perspectives of the avatar. Taken together, our results support the use of vibrotactile feedback when users observe first- and third-person avatar. They also suggest that constant and phase-based rendering could be used with first-person avatar and support the use of phase-based rendering with third-person avatar. They provide valuable insight for stimulations in any VR applications in which the impression of walking is prominent such as for virtual visits, walking rehabilitation, video games, etc.

Prevalence and risk factors for scoliosis in adults with closed and open spina bifida: A large, cross-sectional study.

BACKGROUND: Scoliosis develops in a proportion of children with myelomeningocele; however, little is known about scoliosis in adulthood and in other forms of spina bifida (SB). OBJECTIVES: The aims of this study were to describe the prevalence of scoliosis and identify risk factors for its development in a large cohort of adults with open and closed SB. METHODS: This was a cross-sectional study of data from 580 adults with SB attending their first consultation at a French multidisciplinary referral centre for SB. Sex, anatomical location and type of SB (open or closed), neurological level, back pain and ambulatory status (new Functional Ambulation Classification [new FAC]) were compared in adults with and without scoliosis. These characteristics were used to determine scoliosis risk factors. RESULTS: In total, 331 adults fulfilled the inclusion criteria: 221 had open and 110 had closed SB. Of these, 176 (53%) had scoliosis: 57% open and 45% closed SB. As compared with individuals without scoliosis, those with scoliosis more frequently had open SB (p=0.03), more cranially located SB (p<0.0001), more severe neurological deficits (p≤0.02) and poorer walking ability (mean new FAC score 3.5 [SD 3.3] vs 6.1 [2.6], [p<0.0001]). In total, 69% had chronic back pain, with no difference in frequency between those with and without scoliosis. The odds of scoliosis was associated with asymmetrical motor level and a new FAC score <4 (odds ratio 0.46, p<0.006, and 0.75, p<0.0001, respectively). CONCLUSION: About half of adults with open and closed SB had scoliosis. Back pain was frequent in those both with and without scoliosis. Individuals with low walking ability and an asymmetrical motor level should be monitored early and continuously to limit the consequences of scoliosis during their lifetime. A major issue is to determine how scoliosis evolves and to determine appropriate monitoring and treatment strategies for individuals at risk.

Adjunct therapies after botulinum toxin injections in spastic adults: Systematic review and SOFMER recommendations.

BACKGROUND: Adjunct therapies (ATs) may further improve outcomes after botulinum toxin injections in spastic patients, but evidence was unclear in previous systematic reviews. OBJECTIVE: To assess the efficacy of non-pharmacological ATs in spastic adults according to the International Classification of Functioning, Disability and Health and build an expert consensus-based on a Delphi process. METHODS: Four electronic databases were searched up to May 2020 for reports of comparative trials of non-pharmacologic ATs after botulinum toxin injections in spastic adults. Then, 25 French experts participated in a two-round Delphi process to build recommendations on the use of ATs. RESULTS: We included 32 studies (1202 participants, median 32/study) evaluating the effects of physical agents (n=9), joint posture procedures (JPPs, n=11), and active ATs (n=14), mainly after stroke. The average quality of articles was good for randomised controlled trials (median [interquartile range] PEDro score=7 [6-8]) but moderate (n=2) or poor (n=2) for non-randomised controlled trials (Downs & Black checklist). Meta-analysis was precluded owing to the heterogeneity of ATs, control groups and outcome measures. There is evidence for the use of JPPs except low-dose manual stretching and soft posture techniques. Continuous postures (by taping or casting) are recommended; discontinuous postures (by orthosis) may be preferred in patients with active function. Device-free or device-assisted active ATs may be beneficial in the mid-term (>3months after botulinum toxin injections), particularly when performed at a high-intensity (>3h/week) as in constraint-induced movement therapy. Self-rehabilitation remains understudied after a focal treatment, but its interest is highlighted by the experts. The use of physical agents is not recommended. CONCLUSIONS: JPPs and active ATs (device-assisted or device-free) may further improve impairments and activities after botulinum toxin injections. Further studies are needed to better define the best strategies for ATs as a function of the individual treatment goals, participation and quality of life. REVIEW REGISTRATION: PROSPERO (CRD42018105856).