Long-term Evaluation Parameters in GNE Myopathy: A Five-year Observational Follow-up Natural History Study

BackgroundA number of clinical trials targeting GNE myopathy patients have been conducted. However, useful clinical parameters for post-marketing surveillance and long-term clinical observation have not yet been established. ObjectiveWe conducted a 5-year observational follow-up natural history study to identify evaluation parameters which may be useful for the long-term observation of GNE myopathy patients. MethodsThirty-three genetically-confirmed GNE myopathy patients were recruited and evaluated at study entry (baseline) and yearly in a 5-year follow-up. Hand-held dynamometer measurements of knee extension strength, grip power, and pinch power, summed Manual Muscle Testing (MMT) score of 17 muscles, Gross Motor Function Measure (GMFM), 6-minute walk test, percent vital capacity and percent force vital capacity (%FVC), lean body mass (whole body, arms, and legs), creatine kinase (CK), Barthel Index, modified Rankin Scale, and SF-36 national standard scores were examined. ResultsOf the 33 patients, 22 (66%) completed evaluations for the entire 5-year follow-up period. These patients had a significant reduction in summed MMT score (p=0.001), GMFM (p=0.001), grip power (p=0.013), pinch power (p<0.001), CK (p=0.030), %FVC (p<0.001), leg lean body mass (p=0.040), and the Physical Functioning subscale score of the SF-36 (p=0.015) at the 5th year evaluation relative to baseline. Among these parameters, summed MMT score, GMFM, pinch power, and %FVC showed significant changes even in non-ambulant patients.ConclusionsMMT, GMFM, pinch power, CK, %FVC, lean body mass, and Physical Functioning subscale score of the SF-36 are useful parameters for the long-term evaluation of GNE myopathy patients.

Outcome of trapeziectomy with ligament reconstruction tendon interposition for the treatment of advanced thumb carpometacarpal arthritis: a prospective study

<p><strong>Background</strong>: The purpose of this study is to assess the functional outcome of the cases with advanced thumb carpometacarpal (CMC) arthritis treated with trapeziectomy and ligament reconstruction tendon interposition (LRTI) in terms of visual analogue scale (VAS), disabilities of the arm, shoulder and hand (DASH) score, Kapandji score, range of motion (ROM) of radial and volar abduction of the thumb, grip power, key pinch and the height of the trapezial space.</p><p><strong>Methods</strong>: In a prospective before and after interventional study of 30 patients with the advanced thumb CMC joint arthritis underwent the procedure. An average follows up period was 17.9 months.</p><p><strong>Results:</strong> The mean VAS decreased from 8.17 to 2.70. The mean Kapandji score and DASH score improved from 3.47 and 71.62 preoperative to 8.23 and 14.46 postoperative. Mean ROM for radial and volar abduction increased from 42.57° and 48° to 61°and 64.73° respectively. Mean key pinch power and grip power increased from 2.80 kg and 3.47 kg to 4.70 kg and 9.01 kg respectively. There was a significant decrease in height of the trapezial space. Three patient complaint of the persistent pain at the surgical site and not much improvement in the DASH score.</p><p><strong>Conclusions:</strong> Trapeziectomy with LRTI using flexor carpi radialis (FCR) transfer is an effective treatment for the advanced thumb CMC arthritis. This procedure stabilizes thumb metacarpal and provide enough support which prevents the collapse of metacarpal into dead space.</p>

Efficacy of mirror therapy for improving unimanual motor skills in chronic stroke patients: A case series

BACKGROUND: Unimanual activities are suggested as an effective intervention for the moderately or severely affected chronic stroke patients and bimanual activities for mildly affected cases in mirror therapy. OBJECTIVE: To observe the feasibility of unimanual mirror therapy to improve the motor skills of the effected hand in mild chronic stroke patients. METHODS: In this is case series study, n = 20 mildly affected chronic stroke patients were screened for participation in a nursing home setting. They were 45–60 years of stroke included ischemia (n = 3) and aneurysm (n = 1) of anterior cerebral artery, median time:3 years. The gross (Fugl-Meyer Assessment test), fine motor (Box and Block Tests) skills, the tripot pinch grip power (JTech Commander PichTrack) were assessed at baseline and post intervention. The intervention supervised included unimanual activities of the non-affected hands for 30 min, 5 days/week for 6 weeks. RESULTS: N = 4 participants were recruited from a nursing home setting; median age: 45, Gender: 3M; 1W. The causes of stroke included ischemia (n = 3) and aneurysm of anterior cerebral artery (n = 1). The median years of stroke was 3 years. The improvement in the gross motor skills (25%) of affected sides after unimanual training may be considered as feasable. There were also increase in tripot pinch grip power of the non-affected (34%) and affected hands (17%). The improvement in fine motor skills were 19% in affected hands the 10% in non-affected hands. CONCLUSIONS: The unimanual mirror therapy resulted in improvements in gross and fine motor skills and tripod pinch grip power of the affected hands of mild chronic stroke patients. This case series provides further evidence that mirror therapy is effective to improve function in patients with mild chronic stroke.

Comparison of surgical out come between virgin carpal tunnel syndrome and previous local steroid injected tunnel

Carpal tunnel syndrome(CTS) is the most commonly occurring peripheral nerve compression neuropathy and its condition characterized by an abnormality of the median nerve function due to compression of the nerve within the carpal tunnel. Surgical release of the transverse carpal ligament is an effective treatment for patients with CTS. Non-steroidal anti-inflammatory drugs, diuretics, vitamin B6 injection, ultrasound therapy, laser therapy, acupuncture, magnetic therapy, bracing and local steroid injections have been used for closed treatment of CTS and effective results in the short-term treatment have been demonstrated clearly only for bracing and local steroid injections. Aim: was to identify the effect of local steroid injection on the outcome of surgical release of CTS. A prospective study that was conducted in the Orthopedic Department of at Basra General Hospital during the period from 1st of July 2005 till end of September 2006 on 40 patients, 20 of them with received local steroid injection (group A) and 20 without local steroid injection (group B virgin carpal tunnel) operations done to release the tunnel with monthly follow up by telephone call because of social and security problems at time of study. Pearson’s Chi–square test was used to assess statistical association between injection of local steroid and outcome of CTS surgery. A level of P – value less than 0.05 was considered significant. Postoperatively, in Group A, night pain and grip power were found to be improved in 17 (85%) and 11 (55%) of women, respectively, while 16 (80%) relieved from night pain, paresthesia and numbness. On the other hand, the postoperative follow up of women in group B showed that the improvement of night pain was occurred in 20 cases (90%), relieving of night pain, paresthesia and numbness in 17 (85%), and improvement of grip power was reported in 12 (60%). The analysis of association didn’t show statistical significant difference (P > 0.05) in surgical outcome between study groups. In conclusion; local steroid injection for CTS prior to surgery didn’t affect outcome of surgical release.

Comparison of surgical out come between virgin carpal tunnel syndrome and previous local steroid injected tunnel

"Carpal tunnel syndrome(CTS) is the most commonly occurring peripheral nerve compression neuropathy and its condition characterized by an abnormality of the median nerve function due to compression of the nerve within the carpal tunnel. Surgical release of the transverse carpal ligament is an effective treatment for patients with CTS. Non-steroidal anti-inflammatory drugs, diuretics, vitamin B6 injection, ultrasound therapy, laser therapy, acupuncture, magnetic therapy, bracing and local steroid injections have been used for closed treatment of CTS and effective results in the short-term treatment have been demonstrated clearly only for bracing and local steroid injections. Aim: was to identify the effect of local steroid injection on the outcome of surgical release of CTS. A prospective study that was conducted in the Orthopedic Department of at Basra General Hospital during the period from 1st of July 2005 till end of September 2006 on 40 patients, 20 of them with received local steroid injection (group A) and 20 without local steroid injection (group B virgin carpal tunnel) operations done to release the tunnel with monthly follow up by telephone call because of social and security problems at time of study. Pearson’s Chi–square test was used to assess statistical association between injection of local steroid and outcome of CTS surgery. A level of P – value less than 0.05 was considered significant. Postoperatively, in Group A, night pain and grip power were found to be improved in 17 (85%) and 11 (55%) of women, respectively, while 16 (80%) relieved from night pain, paresthesia and numbness. On the other hand, the postoperative follow up of women in group B showed that the improvement of night pain was occurred in 20 cases (90%), relieving of night pain, paresthesia and numbness in 17 (85%), and improvement of grip power was reported in 12 (60%). The analysis of association didn’t show statistical significant difference (P > 0.05) in surgical outcome between study groups. In conclusion; local steroid injection for CTS prior to surgery didn’t affect outcome of surgical release."

Relationships between cystatin C- and creatinine-based eGFR in Japanese rural community- dwelling older adults with sarcopenia

Background Sarcopenia is prevalent in patients with chronic kidney disease (CKD). The indices of physical function, such as grip power and gait speed, decreased according to the decline in the estimated glomerular filtration rate (eGFR). Methods We examined the relationships between cystatin C-based GFR (eGFRcys), creatinine-based GFR (eGFRcre), their ratio (eGFRcys/eGFRcre) and sarcopenia in community-dwelling older adults in Japan. This cross-sectional study included 302 men aged 73.9 ± 6.2 years and 647 women aged 72.9 ± 5.8 years from a rural area in Hyogo Prefecture, Japan. eGFRcys and eGFRcre were simultaneously measured, and sarcopenia based on the Asia Working Group for Sarcopenia (AWGS) 2019 criteria was evaluated. Results eGFRcys and the eGFRcys/eGFRcre ratio were significantly correlated with grip power and gait speed (p < 0.001). The eGFRcys/eGFRcre ratio was also correlated with skeletal muscle mass index (SMI) (p < 0.01). Univariate logistic regression analysis showed eGFRcys and eGFRcys/eGFRcre ratio but not eGFRcre were associated with sarcopenia (p < 0.01). The presence of low eGFRcys (CKDcys) and low eGFRcys/eGFRcre ratio (< 1.0) but not that of low eGFRcre (CKDcre) were associated with sarcopenia (p < 0.01). In the multivariate logistic regression analysis, when the eGFRcys/eGFRcre ratio was added as a covariate to the basic model, it was significantly associated with sarcopenia in women (p < 0.05). Moreover, low eGFRcys/eGFRcre ratio (< 1.0) was associated with a higher risk of sarcopenia in men (p < 0.01). Conclusion In conclusion, CKDcys but not CKDcre is associated with sarcopenia. A lower eGFRcys/eGFRcre ratio may be a practical screening marker of sarcopenia in community-dwelling older adults.

Comparative study of cast immobilization of wrist in functional position versus conventional palmar flexion in Colles’ fracture

Background: Colles’ fracture is a common injury in the elderly population. Although it can be easily reduced, it is difficult to maintain the reduction in the conventional position of the wrist in palmar flexion with a poor functional outcome. Thus our study aimed to compare the radiological and functional outcome of such fractures with the wrist in two different posi­tions of dorsiflexion and palmar flexion. Methods: A prospective, observational study was conducted from April 2019 to March 2020 in the Orthopedics and Emergency department of KIST Medical College Teaching Hospital, Lalitpur, Ne­pal. Sixty-two patients, 31 in each group, all above 45 years with Colles’ fracture underwent close reduction and below elbow cast immobilization under hematoma block. Maintaining palmar flex­ion and ulnar deviation at the fracture site, the wrist was immobilized in dorsiflexion and palmar flexion attitude alternatively. During the respective follow-ups, the Demerit Scoring System of Saito was evaluated. Data collection and entry was done using the statistical package for social science version 16 and analyzed by using descriptive and inferential statistics. Results: All fractures were united. At the end of twelve weeks, Saito Scoring System of good to ex­cellent was markedly better in dorsiflexed group 29(93.5%) patients as compared to palmar flexed group 22(71.0%) patients, similarly grip power was also superior in dorsiflexed group 19(61.3%) patients than in palmar flexed group 11(35.5%) patients. Conclusions: Cast immobilization of Colles’ fracture with the wrist in dorsiflexion prevents re-dis­placement of the fragments resulting in significantly better radiological and functional outcomes.

A Fully Immersive Virtual Reality Method for Upper Limb Rehabilitation in Spinal Cord Injury

Objective To determine whether a fully immersive virtual reality (VR) intervention combined with conventional rehabilitation (CR) can improve upper limb function more than CR alone in patients with spinal cord injury (SCI), we conducted a prospective, randomized, controlled clinical trial.Methods Participants were randomly assigned to either the control group (CG; n=10) or experimental group (EG; n=10). The participants in the CG received 60 minutes of conventional therapy per day, 4 days per week for 4 weeks, whereas those in the EG received 30 minutes of VR training and 30 minutes of conventional therapy per day, 4 days per week for 4 weeks. The clinical outcome measures included Medical Research Council grade, the American Spinal Injury Association upper extremity motor score (ASIA-UEMS), and scores in the Hand Strength Test, Box and Block Test, Nine-Hole Peg Test, Action Research Arm Test, and Korean version of the Spinal Cord Independence Measure (K-SCIM). The assessments were performed at the beginning (T0) and end of the intervention (T1).Results Grip power and K-SCIM score significantly improved in the EG after the intervention. When comparing differences between the groups, elbow extensor, wrist extensor, ASIA-UEMS, grip power, lateral pinch power, and palmar pinch power were all significantly improved.Conclusion VR training of upper limb function after SCI can provide an acceptable adjunctive rehabilitation method without significant adverse effects.