scholarly journals Cellular Transplantation Alters the Disease Progression in Becker’s Muscular Dystrophy

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Alok Sharma ◽  
Amruta Paranjape ◽  
Hemangi Sane ◽  
Khushboo Bhagawanani ◽  
Nandini Gokulchandran ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Muscle Strength ◽  
Disease Progression ◽  
Mononuclear Cells ◽  
Cellular Therapy ◽  
Fatty Infiltration ◽  
Functional Independence ◽  
Manual Muscle Testing ◽  
Muscle Degeneration ◽  
Muscle Testing

Becker’s Muscular Dystrophy (BMD) is a dystrophinopathy manifested as progressive muscle degeneration. Autologous Bone Marrow Mononuclear Cells (BMMNCs) have shown some myogenic potential. The paracrine effects of the BMMNCs reduce the inflammation and are thought to reduce muscle degeneration. We treated a 39 year old dental surgeon suffering from BMD. Muscle strength was reduced when measured using modified Medical Research Council’s Manual Muscle Testing (mMRC-MMT). Static sitting balance was poor. He was wheelchair dependent for ambulation and moderately independent in Activities of Daily Living (ADL). Functional Independence Measure (FIM) score was 93. Musculoskeletal Magnetic Resonance Imaging (MRI-MSK) showed moderate fatty infiltration in the muscles. Three cellular transplantations were carried out. Clinical assessment and the investigations were repeated. Progressive increase in the muscle strength was noted. Ambulation was independent using push-knee splints and minimal assistance when weary. Static and dynamic balance in sitting and standing improved. FIM score increased from 93 to 105. There was no increase in the degree of fatty infiltration, as seen on the MRI-MSK. The case study provides evidence for the putative benefits of cellular therapy in altering the disease progression in BMD. It also suggests augmented clinical benefits of combination of cellular therapy and rehabilitation.

scholarly journals Impact of structured physical therapy intervention on functional recovery in a patient with CAPOS syndrome: a case report

2021 ◽  
Vol 26 (1) ◽  
Author(s):  
Shailesh Gardas ◽  
Aishwarya Mahajan
Keyword(s):  
Functional Performance ◽  
Functional Independence Measure ◽  
Functional Independence ◽  
Measurement Scale ◽  
Manual Muscle Testing ◽  
Trunk Control ◽  
Control Measurement ◽  
Muscle Testing ◽  
Post Test ◽  
Capos Syndrome

Abstract Background CAPOS syndrome (cerebellar ataxia, areflexia, pescavus, optic atrophy, and sensorineural hearing loss) is a rare congenital autosomal dominant disorder. The resulting neurological sequelae of impairments are progressive in nature and may interfere with functional independence, performing activities of daily living (ADL’s), and subsequently, affecting the quality of life (QOL). Since it is an extremely rare disorder, there is a severe dearth in the literature about how specific physiotherapy interventions may affect their functional status. Therefore, our objective was to investigate the effects of proprioceptive neuromuscular facilitation (PNF) and Frenkel’s coordination exercises on functional recovery in a patient with CAPOS syndrome. Case presentation We herein present a case of a 25-year-old Indian male with complaints of generalized body weakness, difficulty visualizing distant objects, nystagmus, progressive sensorineural deafness, and ataxia. He was rehabilitated with a structured/customized physiotherapy protocol consisting of PNF approach and coordination exercises for 4 weeks, 6 days/week, 60 min daily. An improvement in overall functional performance of patient as per post-intervention scores of manual muscle testing, trunk control measurement scale, functional independence measure (components of self-care, transfers, and locomotion), and decline in severity of ataxia on scale for assessment and rating of ataxia scale was observed. Conclusion PNF and Frenkel’s exercises resulted in an improvement in overall functional performance of the patient. Improvement was observed in post-test scores of Manual Muscle Testing (MMT), Trunk Control Measurement Scale (TCMS), and Functional Independence Measure (FIM) for the components of self-care, transfers, and locomotion. Additionally, results also showed a decline in severity of ataxia on post-test scores of scale for the assessment and rating of ataxia (SARA) scale (i.e., from severe to moderate).

scholarly journals Effect of Isometric and Isotonic Exercise Program on Increased Strength of Quardriceps Muscles in Osteoarthritis Patients in Singgasana Rama Physiotherapy Clinic Blitar

2021 ◽  
Vol 5 (1) ◽  
pp. 42-48
Author(s):  
Irwan Syahputra ◽  
Nurwijayanti Nurwijayanti
Keyword(s):  
Muscle Strength ◽  
Exercise Program ◽  
Quadriceps Strength ◽  
Manual Muscle Testing ◽  
Moderate Intensity ◽  
Rank Test ◽  
Control Group ◽  
Isometric Training ◽  
Muscle Testing ◽  
Isotonic Exercise

Background: Osteoarthritis is a disease of the joints that can cause disability and is progressive in the joints which cause changes in the water content, proteoglycin content, and collagen in the joints. One of the ways to improve functional ability in osteoarthritis cases is to use isotonic and isometric training exercise. Pain is a clinical symptom of knee osteoarthritis, limiting movement due to pain over time will cause rehabilitation problems such as impaired flexibility and stability, reduction of muscle mass (atrophy). Mild and moderate intensity quadriceps strength training is effective at achieving optimal muscle strength and function without causing significant muscle damage. Destination: The purpose of this study was to analyze the effect of isotonic and isometric exercises on muscle strength. Research methods: This study uses a pre-experimental research type using "One Group Pre - test and Post - Test". This research has carried out the first observation (pretest) so that researchers can test the changes that occur after the treatment, and the control group. Result: with the Wilcoxon signed rank test test the isometric and isotonic groups had p = 0.000 while the control group had a significance value of p = 1,000; and the 3 groups test has different values, namely: isotonic exercise has a value of 27.67; isometric; 28.60; and control 12.73. The conclusion that can be concluded that the value of isometric training has the highest value, namely 28.60 which means it is the most effective exercise to increase the value of Manual Muscle Testing. Conclusion: There is an effect of giving isometric and isotonic exercises in strengthening the quadriceps muscles.

scholarly journals Monitoring disease activity noninvasively in the mdx model of Duchenne muscular dystrophy

2018 ◽  
Vol 115 (30) ◽  
pp. 7741-7746 ◽  
Author(s):  
Antonio Filareto ◽  
Katie Maguire-Nguyen ◽  
Qiang Gan ◽  
Garazi Aldanondo ◽  
Léo Machado ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Disease Progression ◽  
Premature Death ◽  
Response To Therapy ◽  
Luciferase Reporter ◽  
Luciferase Expression ◽  
Muscle Degeneration ◽  
Reporter Strain ◽  
Novel Treatments

Duchenne muscular dystrophy (DMD) is a rare, muscle degenerative disease resulting from the absence of the dystrophin protein. DMD is characterized by progressive loss of muscle fibers, muscle weakness, and eventually loss of ambulation and premature death. Currently, there is no cure for DMD and improved methods of disease monitoring are crucial for the development of novel treatments. In this study, we describe a new method of assessing disease progression noninvasively in the mdx model of DMD. The reporter mice, which we term the dystrophic Degeneration Reporter strains, contain an inducible CRE-responsive luciferase reporter active in mature myofibers. In these mice, muscle degeneration is reflected in changes in the level of luciferase expression, which can be monitored using noninvasive, bioluminescence imaging. We monitored the natural history and disease progression in these dystrophic report mice and found that decreases in luciferase signals directly correlated with muscle degeneration. We further demonstrated that this reporter strain, as well as a previously reported Regeneration Reporter strain, successfully reveals the effectiveness of a gene therapy treatment following systemic administration of a recombinant adeno-associated virus-6 (rAAV-6) encoding a microdystrophin construct. Our data demonstrate the value of these noninvasive imaging modalities for monitoring disease progression and response to therapy in mouse models of muscular dystrophy.

Hand-held dynamometry for assessment of muscle strength in patients with inflammatory myopathies

Rheumatology ◽  
2020 ◽  
Author(s):  
Didem Saygin ◽  
Chester V Oddis ◽  
Siamak Moghadam-Kia ◽  
Bonny Rockette-Wagner ◽  
Nicole Neiman ◽  
...  
Keyword(s):  
Muscle Strength ◽  
Disease Activity ◽  
Neuromuscular Disorders ◽  
Strong Association ◽  
External Validation ◽  
Manual Muscle Testing ◽  
Inflammatory Myopathies ◽  
University Of Pittsburgh ◽  
Muscle Testing ◽  
The University

Abstract Objectives Muscle weakness in idiopathic inflammatory myopathies (IIMs) is conventionally assessed using manual muscle testing (MMT). However, more objective tools must be developed to accurately and reliably quantify muscle strength in myositis patients. Hand-held dynamometry (HHD) is a quantitative, portable device with reported reliability in neuromuscular disorders. Our aim was to assess the reliability, validity and responsiveness of HHD in myositis. Methods Myositis patients [DM, necrotizing myopathy (NM), PM and anti-synthetase syndrome] evaluated at the University of Pittsburgh myositis centre were prospectively enrolled. Each patient was assessed at 0, 3 and 6 months for validated outcome measures of myositis disease activity and physical function. At each visit, muscle strength was assessed using both MMT and HHD (Micro FET2, Hoggan Health Industries, Draper, UT, USA). The reliability, validity and responsiveness of the HHD was assessed using standard statistical methods. Results Fifty IIM patients (60% female; mean age 51.6 years; 6 PM, 9 NM, 24 DM and 11 anti-synthetase syndrome) were enrolled. HHD showed strong test–retest intrarater reliability (r = 0.96) and interrater reliability (r = 0.98). HHD correlated significantly with the MMT score (r = 0.48, P = 0.0006) and myositis disease activity and functional measures. Longitudinal analysis showed a significant and strong association between the HHD and MMT as well as 2016 ACR/EULAR myositis response criteria (r = 0.8, P < 0.0001) demonstrating responsiveness. The mean effect size and standardized response mean of HHD was large: 0.95 and 1.03, respectively. MMT had a high ceiling effect compared with HHD. Conclusion HHD demonstrated strong reliability, construct validity and responsiveness in myositis patients. External validation studies are required to confirm these findings.

scholarly journals Large variation in effects during 10 years of enzyme therapy in adults with Pompe disease

Neurology ◽  
2019 ◽  
Vol 93 (19) ◽  
pp. e1756-e1767 ◽  
Author(s):  
Laurike Harlaar ◽  
Jean-Yves Hogrel ◽  
Barbara Perniconi ◽  
Michelle E. Kruijshaar ◽  
Dimitris Rizopoulos ◽  
...  
Keyword(s):  
Muscle Strength ◽  
Pulmonary Function ◽  
Pompe Disease ◽  
Manual Muscle Testing ◽  
Controlled Clinical Trial ◽  
Walking Ability ◽  
Enzyme Replacement ◽  
Link Type ◽  
Muscle Testing

ObjectiveTo determine the effects of 10 years of enzyme replacement therapy (ERT) in adult patients with Pompe disease, focusing on individual variability in treatment response.MethodsIn this prospective, multicenter cohort study, we studied 30 patients from the Netherlands and France who had started ERT during the only randomized placebo-controlled clinical trial with ERT in late-onset Pompe disease (NCT00158600) or its extension (NCT00455195) in 2005 to 2008. Main outcomes were walking ability (6-minute walk test [6MWT]), muscle strength (manual muscle testing using Medical Research Council [MRC] grading), and pulmonary function (forced vital capacity [FVC] in the upright and supine positions), assessed at 3- to 6-month intervals before and after the start of ERT. Data were analyzed with linear mixed-effects models for repeated measurements.ResultsMedian follow-up duration on ERT was 9.8 years (interquartile range [IQR] 8.3–10.2 years). At the group level, baseline 6MWT was 49% of predicted (IQR 41%–60%) and had deteriorated by 22.2 percentage points (pp) at the 10-year treatment point (p < 0.001). Baseline FVC upright was 54% of predicted (IQR 47%–68%) and decreased by 11 pp over 10 years (p < 0.001). Effects of ERT on MRC sum score and FVC supine were similar. At the individual level, 93% of patients had initial benefit of ERT. Depending on the outcome measured, 35% to 63% of patients had a secondary decline after ≈3 to 5 years. Still, at 10 years of ERT, 52% had equal or better 6MWT and/or FVC upright compared to baseline.ConclusionsThe majority of patients with Pompe disease benefit from long-term ERT, but many patients experience some secondary decline after ≈3 to 5 years. Individual variation, however, is considerable.Classification of evidenceThis study provides Class IV evidence that for the majority of adults with Pompe disease, long-term ERT positively affects, or slows deterioration in, muscle strength, walking ability, and/or pulmonary function.

scholarly journals Assessment of disease progression in dysferlinopathy

Neurology ◽  
2019 ◽  
Vol 92 (5) ◽  
pp. e461-e474 ◽  
Author(s):  
Ursula Moore ◽  
Marni Jacobs ◽  
Meredith K. James ◽  
Anna G. Mayhew ◽  
Roberto Fernandez-Torron ◽  
...  
Keyword(s):  
Disease Progression ◽  
Clinical Diagnosis ◽  
Clinical Effectiveness ◽  
Manual Muscle Testing ◽  
Walk Test ◽  
Wrist Flexion ◽  
Pinch Grip ◽  
Muscle Testing ◽  
Domain 3 ◽  
Functional Outcome Measures

ObjectiveTo assess the ability of functional measures to detect disease progression in dysferlinopathy over 6 months and 1 year.MethodsOne hundred ninety-three patients with dysferlinopathy were recruited to the Jain Foundation's International Clinical Outcome Study for Dysferlinopathy. Baseline, 6-month, and 1-year assessments included adapted North Star Ambulatory Assessment (a-NSAA), Motor Function Measure (MFM-20), timed function tests, 6-minute walk test (6MWT), Brooke scale, Jebsen test, manual muscle testing, and hand-held dynamometry. Patients also completed the ACTIVLIM questionnaire. Change in each measure over 6 months and 1 year was calculated and compared between disease severity (ambulant [mild, moderate, or severe based on a-NSAA score] or nonambulant [unable to complete a 10-meter walk]) and clinical diagnosis.ResultsThe functional a-NSAA test was the most sensitive to deterioration for ambulant patients overall. The a-NSAA score was the most sensitive test in the mild and moderate groups, while the 6MWT was most sensitive in the severe group. The 10-meter walk test was the only test showing significant change across all ambulant severity groups. In nonambulant patients, the MFM domain 3, wrist flexion strength, and pinch grip were most sensitive. Progression rates did not differ by clinical diagnosis. Power calculations determined that 46 moderately affected patients are required to determine clinical effectiveness for a hypothetical 1-year clinical trial based on the a-NSAA as a clinical endpoint.ConclusionCertain functional outcome measures can detect changes over 6 months and 1 year in dysferlinopathy and potentially be useful in monitoring progression in clinical trials.ClinicalTrials.gov identifier:NCT01676077.

scholarly journals Assessing the Validity and Reliability of A Low-Cost Microcontroller-Based Load Cell Amplifier for Measuring Lower Limb and Upper Limb Muscular Force

Sensors ◽  
2020 ◽  
Vol 20 (17) ◽  
pp. 4999
Author(s):  
Julie Gaudet ◽  
Grant Handrigan
Keyword(s):  
Muscle Strength ◽  
Upper Limb ◽  
Low Cost ◽  
Load Cell ◽  
Manual Muscle Testing ◽  
Muscular Force ◽  
Validity And Reliability ◽  
Important Indicator ◽  
Muscle Testing ◽  
Isometric Muscle

Lower and upper limb maximum muscular force development is an important indicator of physical capacity. Manual muscle testing, load cell coupled with a signal conditioner, and handheld dynamometry are three widely used techniques for measuring isometric muscle strength. Recently, there is a proliferation of low-cost tools that have potential to be used to measure muscle strength. This study examined both the criterion validity, inter-day reliability and intra-day reliability of a microcontroller-based load cell amplifier for quantifying muscle strength. To do so, a low-cost microcontroller-based load cell amplifier for measuring lower and upper limb maximal voluntary isometric muscular force was compared to a commercial grade signal conditioner and to a handheld dynamometer. The results showed that the microcontroller-based load cell amplifier correlated nearly perfectly (Pearson's R-values between 0.947 to 0.992) with the commercial signal conditioner and the handheld dynamometer, and showed good to excellent association when calculating ICC scores, with values of 0.9582 [95% C.I.: 0.9297–0.9752] for inter-day reliability and of 0.9269 [95% C.I.: 0.8909–0.9533] for session one, intra-day reliability. Such results may have implications for how the evaluation of muscle strength measurement is conducted in the future, particularly for offering a commercial-like grade quality, low cost, portable and flexible option.

scholarly journals Relationship between Grade 3 Muscle Strength and the Maximum Muscle Strength in Manual Muscle Testing of Legs: Assessment of Flexion and Extension of the Hip and Knee Joints

2014 ◽  
Vol 29 (6) ◽  
pp. 861-865 ◽  
Author(s):  
Hideyuki USA ◽  
Masashi MATSUMURA ◽  
Daisuke OGAWA ◽  
Masafumi HATA ◽  
Kazuna ICHIKAWA ◽  
...  
Keyword(s):  
Muscle Strength ◽  
Knee Joints ◽  
Manual Muscle Testing ◽  
Muscle Testing ◽  
Grade 3 ◽  
Flexion And Extension
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