Prediction of Changes in Functional Outcomes During the First Year After Inpatient Stroke Rehabilitation: A Longitudinal Study.

OBJECTIVE: To identify meaningful changes in patients in 3 functional domains (basic mobility [BM], daily activity [DA], and applied cognition [AC]) after discharge from inpatient stroke rehabilitation and to identify the predictors of 1-year functional improvement. DESIGN: A longitudinal, multicenter, prospective cohort study. SETTING: The acute care wards of 3 hospitals in the Greater Taipei area of Taiwan. PARTICIPANTS: Five hundred patients with stroke in acute care wards (mean age=60±12.2 years, 62% men, N=500). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURE(S): The Mandarin version of the Activity Measure for Post-Acute Care (AM-PAC) short forms were assessed at discharge and 3-, 6-, and 12-month follow-up. The minimal detectable change (MDC) was used to categorize changes in the scores as improved and unimproved at the 4 time points. RESULTS: The mean scores of the AM-PAC BM and DA subscales substantially increased over the first 3 months after discharge (86% of participants exhibited improvement) and slightly increased during the subsequent 9 months (5∼26% of participants exhibited improvement). However, the mean score of the AC subscale decreased within the first 3 months and increased over the subsequent 9 months (22-23% of participants exhibited improvement). The BM, AC scores at discharge were the dominant predictors of subsequent functional improvement (P<.05). Patients with a higher functional stage at discharge were more likely to experience significant improvement. CONCLUSION: This study established the capacity of the AM-PAC to predict functional improvement in 3 domains during the early, middle, and late stages of recovery. The findings can assist clinicians in identifying patients at risk of unfavorable long-term functional recovery and providing such patients with tailored interventions during the early stage of rehabilitation.

Influence of temperature on sonographic images of the median nerve for the diagnosis of carpal tunnel syndrome: a case control study.

BACKGROUND: In addition to nerve conduction studies (NCSs), ultrasonography has been widely used as an alternative tool for diagnosing carpal tunnel syndrome (CTS). Although the results of NCSs are influenced by local skin temperature, few studies have explored the effects of skin temperature on ultrasonography of the median nerve. Since swelling and intraneural blood flow of the median nerve might be influenced by local temperature changes, the aim of this study was to evaluate the cross-sectional area (CSA) and intraneural blood flow of the median nerve under three skin temperatures (30 °C, 32 °C, 34 °C). METHODS: Fifty patients with CTS and 50 healthy volunteers were consecutively recruited from a community hospital. Each participant received physical examinations and NCSs and underwent ultrasonography, including power Doppler, to evaluate intraneural vascularity. RESULTS: The CSA of the median nerve in the CTS patients was significantly larger than that in the healthy controls at all three temperatures. However, significant differences in the power Doppler signals of the median nerve between the two studied groups were observed only at 30 and 32 °C, not at 34 °C. CONCLUSION: The significant difference in the intraneural vascularity of the median nerve between the patients with CTS and the healthy subjects was lost at higher temperatures (34 °C). Therefore, the results of power Doppler ultrasonography in diagnosing CTS should be cautiously interpreted in patients with a high skin temperature or those who reside in warm environments.

Chrysophanol attenuates hepatitis B virus X protein-induced hepatic stellate cell fibrosis by regulating endoplasmic reticulum stress and ferroptosis.

Hepatitis B virus X protein (HBx) and hepatic stellate cells (HSCs) are critical for liver fibrosis development. Anti-fibrosis occurs via reversion to quiescent-type HSCs or clearance of HSCs via apoptosis or ferroptosis. We aimed to elucidate the role of chrysophanol in rat HSC-T6 cells expressing HBx and investigate whether chrysophanol (isolated from Rheum palmatum rhizomes) influences cell death via ferroptosis in vitro. Analysis of lipid reactive oxygen species (ROS), Bip, CHOP, p-IRE1α, GPX4, SLC7A11, α-SMA, and CTGF showed that chrysophanol attenuated HBx-repressed cell death. Chrysophanol can impair HBx-induced activation of HSCs via endoplasmic reticulum stress (ER stress) and ferroptosis-dependent and GPX4-independent pathways.

Ratio and difference of the cross-sectional area of median nerve to ulnar nerve in diagnosing carpal tunnel syndrome: a case control study.

BACKGROUND: To evaluate the diagnostic accuracy of the median-to-ulnar nerve ratio (MUR) and the median-to-ulnar nerve difference (MUD) in patients with carpal tunnel syndrome (CTS). METHODS: In this study, 32 patients with CTS and 32 healthy volunteers were evaluated. All participants received a series of tests and ultrasound examination for the evaluation of the following criteria: cross-sectional area of the median nerve at the pisiform level (CSA-P), swelling ratio (SR), MUR, MUD, and flattening ratio (FR). RESULTS: CSA-P, SR, MUR, and MUD were all significantly larger in the patients with CTS than in the healthy volunteers. The areas under the receiver operator characteristic curves of MUD, MUR, CSA-P, and SR were 0.78, 0.75, 0.70, and 0.61 respectively. MUD had higher sensitivity (84%) than MUR, CSA-P, and SR (sensitivity: 63, 63, and 53%, respectively). CONCLUSIONS: By using the ulnar nerve area at the pisiform level as an internal control parameter, the MUD and MUR methods showed higher diagnostic accuracy than SR in patients with CTS. Further application of these methods in research and clinical settings is recommended. TRIAL REGISTRATION: Clinicaltrial.gov NCT03033173. Registered 18 January 2017. Retrospectively registered.

Implementing strategy training in Taiwan: perspectives of individuals with Acquired brain injury.

PURPOSE: Strategy training is a rehabilitation intervention that aims to enhance problem-solving skills with respect to daily activity-related challenges and has achieved favorable results in Western countries. This study explored the perspectives of individuals with acquired brain injury (ABI) in Taiwan who received strategy training. MATERIALS AND METHODS: Semi-structured interviews with community-dwelling adults with ABI were conducted, and reflective memos made by research team members were recorded. Interviews and memos were analyzed through thematic analysis. RESULTS: This study included 55 participants. The analysis of the participants' interview responses and memos yielded nine themes under three categories: 1) expectations regarding strategy training, 2) perceived benefits of strategy training, and 3) barriers affecting the process and outcomes of strategy training. CONCLUSIONS: All the participants endorsed strategy training through different gains. Most participants' expectations before the intervention were uncertain. Including family members into the strategy training is of key importance for a successfulness of their goals. The participants' experiences about strategy training were affected by various barriers (i.e., health and medical problems, the physical environment, and natural events). Clinicians and researchers should consider these expectations, benefits, and barriers when studying and implementing strategy training in non-Western contexts.IMPLICATIONS FOR REHABILITATIONStrategy training provides clients the opportunity to actively engage in their own goal setting and decision making.Strategy training increases the client's confidence in their ability to participate in the community, communicate, and perform daily living and physical activities.Therapists should consider the health conditions and physical environment of clients when helping them set goals and before facilitating their engagement in the community.Taiwanese family members play a crucial role in supporting acquired brain injury survivors in strategy training.

Comparative Effectiveness of Two Models of Point-of-Care Ultrasound for Detection of Post-Void Residual Urine during Acute Ischemic Stroke: Preliminary Findings of Real-World Clinical Application.

We conducted a comparative study of two models of point-of-care ultrasound devices for measuring post-void residual urine (PVRU). We prospectively enrolled 55 stroke inpatients who underwent both real-time B-mode ultrasound (Device A) and automated three-dimensional (3D) scanning ultrasound (Device B), with a total of 108 measurements. The median PVRU volume of Device B was 40 mL larger than that of Device A. The PVRU difference between the devices was positively and linearly correlated with PVRU. The correlation of PVRU volume between the devices was strong, but the agreement level was only moderate. Measurement deviations were observed in 43 (40%) and 11 (10%) measurements with Device B and Device A, respectively. The PVRU volume was low in spherical bladder shapes but sequentially increased in triangular, undefined, ellipsoid, and cuboid bladder shapes. Further comparison of 60 sets of PVRU without measurement deviations revealed higher agreements between the devices at correction coefficients of 0.52, 0.66, and 0.81 for PVRU volumes of <100, 100-200, and >200 mL, respectively. The automated 3D scanning ultrasound is more convenient for learning and scanning, but it exhibits larger measurement deviations. Real-time B-mode ultrasound accurately visualizes the urinary bladder but tends to underestimate the urinary bladder when the PVRU volume is large. Hence, real-time B-mode ultrasound with automated PVRU-based adjustment of calculation formulas may be a better solution for estimating bladder volume.

The von Hippel-Lindau Tumor Suppressor Gene Mutations Modulate Lipocalin-2 Expression in Ferroptotic-Inflammatory Pathways.

A previous study of an animal model with tumor suppressor gene von Hippel-Lindau (VHL) conditional knockdown suggested that tissue inflammation and fibrosis play important roles in the development of clear-cell renal cell carcinoma (ccRCC), which is consistent with the epidemiological evidence linking inflammatory kidney disease and renal cancer. Ferroptosis and inflammation have been linked in a recent study, but the exact mechanism remains unclear. This study is aimed at investigating the mechanism of lipocalin-2- (LCN-2-) mediated ferroptosis and inflammation in vhl-mutated HK-2 cells and mouse primary proximal tubule cells (mRTCs) and the polarization of macrophage RAW 264.7 cells. Based on the levels of lipid reactive oxygen species (ROS) and the expression of glutathione peroxidase 4 (GPX4) in HK-2 cells, we observed that a VHL mutation increased ROS production and depressed GPX4 expression, whereas LCN-2 knockdown reversed these effects. Accordingly, VHL appears to affect ferroptosis in an LCN-2-dependent manner. We also revealed that LCN-2 sensitizes HK-2 cells to inflammation and macrophage RAW 264.7 cells to M1-like polarization. This study provides novel insights into the potential therapeutic target and strategy for attenuating the progression of ccRCC by revealing the role of VHL in regulating chronic inflammation within the LCN-2-ferroptosis pathway.

Wild Bitter Melon Extract Abrogates Hypoxia-Induced Cell Death via the Regulation of Ferroptosis, ER Stress, and Apoptosis in Microglial BV2 Cells.

Microglial cells are well-known phagocytic cells that are resistant to the central nervous system (CNS) and play an important role in the maintenance of CNS homeostasis. Activated microglial cells induce neuroinflammation under hypoxia and typically cause neuronal damage in CNS diseases. In this study, we propose that wild bitter melon extract (WBM) has a protective effect on hypoxia-induced cell death via regulation of ferroptosis, ER stress, and apoptosis. The results demonstrated that hypoxia caused microglial BV-2 the accumulation of lipid ROS, ferroptosis, ER stress, and apoptosis. In this study, we investigated the pharmacological effects of WBM on BV-2 cells following hypoxia-induced cell death. The results indicated that WBM reversed hypoxia-downregulated antiferroptotic molecules Gpx4 and SLC7A11, as well as upregulated the ER stress markers CHOP and Bip. Moreover, WBM alleviated hypoxia-induced apoptosis via the regulation of cleaved-caspase 3, Bax, and Bcl-2. Our results suggest that WBM may be a good candidate for preventing CNS disorders in the future.

Turning difficulties after stroke and its relationship with trunk function.

BACKGROUND: Turning difficulties has been reported in stroke patients, but most studies have indicated no differences in turning direction regarding turn time or steps. Recent evidence shows that turning difficulty may correlate with trunk control. Trunk flexibility and strength are considered essential to trunk control, but their association with turning performance has not been elucidated. AIM: The study investigated the differences in turning direction in terms of turn duration and angular velocity and the relationship between turning performance and trunk function in patients with chronic stroke. DESIGN: Cross-sectional study. SETTING: Outpatient clinic at the Department of Physical Medicine and Rehabilitation. POPULATION: Chronic stroke patients. METHODS: Twenty-eight stroke patients were evaluated for turning performance and trunk function. Turn duration and angular velocity were assessed using three wearable sensors during 360° turning in place towards both sides. Trunk function, such as flexibility, strength, and control was measured using a tape measure, a microFET3 dynamometer, and the Trunk Impairment Scale. RESULTS: Stroke patients showed significantly longer turn durations (4.62±2.08 vs. 3.59±1.93 s, P=0.036) and lower angular velocity (118.67±35.78 vs. 135.26±42.41°/s, P=0.009) during turning toward the paretic side than towards the nonparetic side. The turning parameters towards the paretic side associated with trunk flexion (r=-0.550, P=0.003) and rotation (r=0.409, P=0.034), trunk flexor strength (r=-0.387, P=0.046), dynamic sitting balance (r=-0.383, P=0.049) and coordination of trunk movement (r=-0.494, P=0.009). However, no relationship was observed between trunk function and turning towards the nonparetic side. CONCLUSIONS: Stroke participants experienced greater difficulty turning towards the paretic side. Trunk flexibility, strength, and control may affect turning performance, especially when turning towards the paretic side, which could explain the occurrence of falls after a turn towards the paretic side. CLINICAL REHABILITATION IMPACT: Stroke patients experience turning difficulties, particularly during turning towards the paretic side. Stroke patients with limited trunk function are more likely to experience turning dysfunction. Clinical therapists should develop effective strategies for enhancing turning ability through improvement of trunk flexibility, strength, and control for clinical rehabilitation practice.

Loss of Function of von Hippel-Lindau Trigger Lipocalin 2-Dependent Inflammatory Responses in Cultured and Primary Renal Tubular Cells.

Previous studies have shown that mutations in the tumor suppressor gene von Hippel-Lindau (VHL) can result in the overproduction of reactive oxygen species (ROS) and chronic inflammation and are a significant predisposing factor for the development of clear-cell renal cell carcinoma (ccRCC). To study VHL's role in ccRCC formation, we previously developed a novel conditional knockout mouse model that mimicked the features of kidney inflammation and fibrosis that lead to cyst formation and hyperplasia. However, due to VHL's complex cellular functions, the mechanism of this phenomenon remains unclear. Here, we used the HK-2 cells and mouse primary renal tubule cells (mRTCs) carrying VHL mutations as models to study the effects and underlying molecular mechanisms of ROS accumulation. We also studied the role of lipocalin 2 (LCN2) in regulating macrophage recruitment by HK-2 cells. We measured the level of ROS in HK-2 cells in the presence or absence of LCN2 knockdown and found that the VHL mutation caused ROS overproduction, but an LCN2 knockdown could attenuate the process. VHL was also found to mediate the in vitro and in vivo expression and secretion of LCN2. Thus, VHL likely affects ROS production in an LCN2-dependent manner. Our findings also suggest that LCN2 sensitizes the inflammatory response of HK-2 cells and the chemotactic abilities of macrophage RAW264.7 cells. By demonstrating that the loss of function of von Hippel-Lindau triggers lipocalin 2-dependent inflammatory responses in cultured and primary renal tubular cells, our results offer novel insights into a potential therapeutic approach for interfering with the development of ccRCC.

Trunk Muscle Activation Patterns During Standing Turns in Patients With Stroke: An Electromyographic Analysis.

Recent evidence indicates that turning difficulty may correlate with trunk control; however, surface electromyography has not been used to explore trunk muscle activity during turning after stroke. This study investigated trunk muscle activation patterns during standing turns in healthy controls (HCs) and patients with stroke with turning difficulty (TD) and no TD (NTD). The participants with stroke were divided into two groups according to the 180° turning duration and number of steps to determine the presence of TD. The activation patterns of the bilateral external oblique and erector spinae muscles of all the participants were recorded during 90° standing turns. A total of 14 HCs, 14 patients with TD, and 14 patients with NTD were recruited. The duration and number of steps in the turning of the TD group were greater than those of the HCs, independent of the turning direction. However, the NTD group had a significantly longer turning duration than did the HC group only toward the paretic side. Their performance was similar when turning toward the non-paretic side; this result is consistent with electromyographic findings. Both TD and NTD groups demonstrated increased amplitudes of trunk muscles compared with the HC groups. Their trunk muscles failed to maintain consistent amplitudes during the entire movement of standing turns in the direction that they required more time or steps to turn toward (i.e., turning in either direction for the TD group and turning toward the paretic side for the NTD group). Patients with stroke had augmented activation of trunk muscles during turning. When patients with TD turned toward either direction and when patients with NTD turned toward the paretic side, the flexible adaptations and selective actions of trunk muscles observed in the HCs were absent. Such distinct activation patterns during turning may contribute to poor turning performance and elevate the risk of falling. Our findings provide insights into the contribution and importance of trunk muscles during turning and the association with TD after stroke. These findings may help guide the development of more effective rehabilitation therapies that target specific muscles for those with TD.

Impacts of stroke and cognitive impairment on activities of daily living in the Taiwan longitudinal study on aging.

Stroke and cognitive impairment are common in older population. They often occur together and their combined effects significantly increase disability in both basic (BADLs) and instrumental (IADLs) activities of daily living. We investigated the individual and combined impacts of stroke and cognitive impairment on BADLs and IADLs. A total of 3331 community-dwelling older adults were enrolled from the Taiwan longitudinal study on aging in 2011. Both BADLs and IADLs were analyzed. Combination of stroke and cognitive impairment increased severity of ADL disabilities, but similar prevalence, similar numbers of summed BADL and IADL tasks with disability, and similar levels of difficulty for each BADL and IADL task were found between the stroke group and cognitive impairment group. The former had more difficult in dressing while the latter had more difficult in using the telephone, transport, and managing finances. A hierarchy of ADLs was also observed in all groups. ADL skill training supplemented with cognitive and physical interventions should focus on secondary prevention of dementia and improve motor functional capacity to reduce loss of ADLs.

Nephroprotective Role of Chrysophanol in Hypoxia/Reoxygenation-Induced Renal Cell Damage via Apoptosis, ER Stress, and Ferroptosis.

Acute kidney injury (AKI) is caused by hypoxia-reoxygenation (H/R), which is a kidney injury produced by a variety of causes, resulting in the remaining portion of the kidney function being unable to maintain the balance for performing the tasks of waste excretion metabolism, and electrolyte and acid-base balance. Many studies have reported the use of Chinese medicine to slow down the progression and alleviate the complications of chronic renal failure. Chrysophanol is a component of Rheum officinale Baill, a traditional Chinese medicine that has been clinically used to treat renal disease. We aimed to study the nephroprotective effect of chrysophanol on hypoxia/ reoxygenation (H/R)-induced cell damage. The results showed that chrysophanol prevented H/R-induced apoptosis via downregulation of cleaved Caspase-3, p-JNK, and Bax but upregulation of Bcl-2 expression. In contrast, chrysophanol attenuated H/R-induced endoplasmic reticulum (ER) stress via the downregulation of CHOP and p-IRE1α expression. Our data demonstrated that chrysophanol alleviated H/R-induced lipid ROS accumulation and ferroptosis. Therefore, we propose that chrysophanol may have a protective effect against AKI by regulating apoptosis, ER stress, and ferroptosis.

Chrysophanol Prevents Lipopolysaccharide-Induced Hepatic Stellate Cell Activation by Upregulating Apoptosis, Oxidative Stress, and the Unfolded Protein Response.

Hepatic stellate cell (HSC) activation is a vital driver of liver fibrosis. Recent research efforts have emphasized the clearance of activated HSCs by apoptosis, senescence, or reversion to the quiescent state. LPS induces human HSC activation directly and contributes to liver disease progression. Chrysophanol is an anthraquinone with hepatoprotective and anti-inflammatory effects. This study aimed to investigate the pharmacological effects and mechanisms of chrysophanol in an LPS-induced activated rat HSC cell line (HSC-T6). The fibrosis phenotype was identified from the expression of α-smooth muscle actin (α-SMA), connective tissue growth factor (CTGF), and integrin ß1 by western blot analysis. We examined DNA fragmentation by terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL) staining. We detected the apoptotic markers p53 and cleaved caspase-3 by western blot analysis. Intracellular ROS were labeled with 2',7'-dichlorofluorescein diacetate (DCF-DA) and the levels were measured by flow cytometry. Finally, we evaluated the ER stress markers binding immunoglobulin protein (BiP) and C/EBP homologous protein (CHOP) by Western blot analysis. Our results showed that chrysophanol decreased HSC-T6 cell viability in LPS-induced activated HSCs. Chrysophanol increased the expression of α-SMA, CTGF, integrin ßI, p53, cleaved caspase-3, and DNA fragmentation. Chrysophanol also elevated ROS levels and increased the expression of BiP and CHOP. Pretreatment with chrysophanol prevented LPS-induced HSC-T6 cell activation by upregulating apoptosis, ROS accumulation, unfolded protein response (UPR) activation, and the UPR proapoptotic effect.

Role of Chrysophanol in Epithelial-Mesenchymal Transition in Oral Cancer Cell Lines via a Wnt-3-Dependent Pathway.

Oral cancer belongs to the group of head and neck cancers. If not diagnosed or treated early, it can be life threatening. Epithelial-mesenchymal transition (EMT) plays an important role in tumor formation and progression. An increase in the presence of the EMT phenotype causes tumor cell proliferation, migration, invasion, and poor prognosis. Therefore, attenuating carcinogenesis via EMT inhibition is a good strategy. Herein, we will determine the pharmacological effects of chrysophanol on the EMT in FaDu cells. To analyze EMT, we detected the expression EMT markers, including α-SMA, ß-catenin, vimentin, N-cadherin, E-cadherin, phospho-GSK-3ß, and nuclear translocations of p65 and ß-catenin by western blotting. Additionally, accumulating evidence indicates that reactive oxygen species (ROS) mediate EMT. Our results showed that the level of ROS was significantly increased after chrysophanol treatment. We further speculated that chrysophanol-mediated EMT and metastasis are involved in the Wnt-3-dependent signaling pathway. The inhibition of the EMT phenotype and metastasis and accumulation of ROS caused by chrysophanol was reversed by treatment with the Wnt-3 agonist Bml 284. Therefore, our findings indicated that chrysophanol altered EMT formation, ROS accumulation, and metastasis via the Wnt-3-dependent signaling pathway.

Enhancing community participation for stroke survivors with cognitive impairment: study protocol for a randomised controlled trial in Taiwan.

INTRODUCTION: Stroke can lead to life-long disability and constitutes a huge financial burden on the family and society. Stroke survivors with cognitive impairment often experience considerable challenges in the process of recovery and returning to society. Interventions that effectively help individuals resume essential daily activities and return to active participation in their communities are lacking. This study examines the efficacy of a newly-developed intervention programme, the Optimising Participation after Stroke through Strategy-training (OPASS) programme, for improving community participation among stroke survivors with cognitive impairment. METHODS AND ANALYSIS: A single-blind, parallel-group randomised controlled trial with allocation concealment and assessor blinding will be implemented to assess the efficacy of the OPASS programme. An expected 210 adults with cognitive impairment following stroke will be randomly assigned to either the experimental intervention (OPASS) group or the attention control group. In addition to their usual rehabilitation, both groups will receive 45 min sessions, twice weekly for a total of 12-15 sessions. The primary outcome is change in participation performance, which will be measured using the participation measure-three domains, four dimensions scale. Additional measures include the Activity Measure for Post-Acute Care generic outpatient short forms, Montreal Cognitive Assessment, Stroop Test, Trail Making Test and General Self-Efficacy Scale. These scales will be administered at baseline, post-intervention, 3-month follow-up, 6-month follow-up and 12-month follow-up. Their results will be analysed using multiple linear regression models and mixed-effects regression models. Further assessment of feasibility and acceptability of the intervention will be conducted through structured interviews with participants, caregivers and therapists. These interviews will be transcribed and thematically analysed. ETHICS AND DISSEMINATION: Ethics approval was obtained from the Ethics Committee of Taipei Medical University (approval number: N201804055). The findings will be disseminated through presentations at scientific conferences and through publication in peer-reviewed journals. TRIAL REGISTRATION NUMBER: NCT03792061; pre-results.

Hand strength and dexterity in patients with Prader-Willi syndrome: a pilot intervention study.

OBJECTIVE: The study aim was to examine the hand function (hand strength and dexterity) and intervention effects of training in adults with Prader-Willi syndrome (PWS). METHODS: Six adults with PWS (two females; mean age 26.14 years) underwent hand muscle strength and dexterity training for 3 months (2 hours per week). The following hand function tests were performed pre- and post-intervention: (1) hand grip, lateral pinch, and tip pinch hand strength tests, (2) the Box and Block test (BBT) for gross manual dexterity and (3) the Purdue Pegboard test for finger dexterity. RESULTS: Before treatment, all subjects showed lower hand grip, lateral pinch, tip pinch strength, and poorer manual/finger dexterity relative to healthy adults. After training, hand function scores improved on many test items, but only the left hand tip pinch and the right hand BBT performance showed significant improvements. CONCLUSIONS: All subjects showed lower hand strength and poorer manual/finger dexterity compared with healthy adults; this should be considered during physical training programs. Owing to limitations in the intervention intensity and possible subject behavioral deficits, further research is needed to clarify the effects of this intervention on hand function in PWS patients.

Motor performance in Prader-Willi syndrome patients and its potential influence on caregiver's quality of life.

BACKGROUND: Prader-Willi syndrome (PWS) is a complex, multisystem genetic disorder characterized by a variety of physical, cognitive, and behavioral impairments. PWS is a unique sarcopenia model characterized by an abnormal increase in body fat mass and a decrease in muscle mass that predisposes patients to reduced physical activity, functional limitations, and disability. These manifestations may require both symptomatic and supportive management, thus negatively influencing their lifelong family caregiver's quality of life. The aim of this study was to examine the functional motor performance of adults with PWS in Taiwan and to measure the quality of life of their primary family caregivers. METHODS: The functional motor tests consisted of the following: (1) 30-s sit-to-stand test, (2) timed up-and-go test, (3) hand grip and lateral pinch strength tests, and (4) Berg Balance Scale. The World Health Organization Quality of Life-short form (WHOQOL-BREF) and the Short-Form 36 Health Survey Questionnaire (SF-36) were used to evaluate health-related quality of life, and the parenting stress index was used to assess the magnitude of stress within the parent-child system. RESULTS: The participants included seven adults (two females and five males) with genetically confirmed PWS and their respective main caregivers. The mean age of the adults with PWS was 25.28 years; range 18-31 years, SD 5.10; the mean BMI was 29.2 kg/m2, SD 6.43. All adults with PWS showed lower hand grip and lateral pinch strengths, fewer sit-to-stand cycles during the 30-s chair stand test, and greater average time during the timed up-and-go test when compared to the normative data on healthy adults. Balance was negatively correlated with the caregiver's health concepts of social functioning (rs -0.879, P = 0.009) and with role limitations due to physical problems (rs -0.899, P = 0.006) and emotional problems (rs -0.794, P = 0.033); hand grip strength was negatively correlated with bodily pain (rs -0.800, P = 0.031), as assessed using the SF-36 questionnaire. The timed up-and-go test was positively correlated with the social relationship domain (rs 0.831, P = 0.021), as assessed using the WHOQOL-BREF questionnaire. The parenting stress index showed no association with the PWS patient's physical activities. CONCLUSIONS: All adults with PWS showed decreased upper and lower limb strength and functional mobility when compared to healthy adults. Some of their motor performance might have negative effects on their primary family members in terms of social participation and physical and emotional role limitations. Future research should explore the relationship between physical performances, psychological difficulties of PWS and caregiver's QOL.