The Effect of a Muscle-mimicking, Fabric-type Shoulder Orthosis on Functional Movements of the Upper Limb in Patients With Neuromuscular Disorder

Seoul National University Hospital30 enrolled

Overview

The goal of this clinical trial is to investigate the effect of a muscle-mimicking, fabric-type shoulder orthosis on functional movements of the upper limb in patients with neuromuscular disorder. The main questions it aims to answer are: * What is the impact of the muscle-mimicking, fabric-type shoulder orthosis on upper limb functional movements in patients with neuromuscular disorder? * Are there observable differences in upper limb function when the shoulder orthosis is worn versus when it is not? Participants will: * Receive education on how to wear and use the shoulder orthosis. * Undergo evaluations, including assessment of upper limb performance, shoulder muscle strength testing, active range of motion measurements, assessment of functional workspace, goal attainment scale evaluation, surface electromyography, physiological measurements such as blood pressure and heart rate, fatigue assessment, and assessment for any musculoskeletal or skin-related issues. Researchers will compare neuromuscular disorder patients before and while wearing and operating the shoulder orthosis to see if there are any significant effects on variables such as upper limb function, range of motion, functional workspace, goal attainment scale, and surface electromyography.

Study Type

INTERVENTIONAL

Allocation

Purpose

SUPPORTIVE_CARE

Masking

NONE

Enrollment

Conditions

Muscular Dystrophy, Duchenne Orthotic Devices

Eligibility

Sex: ALLMin age: 10 Years

Medical Language ↔ Plain English

Inclusion Criteria 1. Patients with a confirmed diagnosis of a neuromuscular disease (NMD) by genetic testing, muscle biopsy, or electrodiagnostic studies, presenting with prominent upper limb muscle weakness. Examples include: 1. Muscular Dystrophies: Duchenne/Becker Muscular Dystrophy (DMD/BMD), Limb-Girdle Muscular Dystrophy (LGMD), Facioscapulohumeral Muscular Dystrophy (FSHD), etc. 2. Motor Neuron Diseases: Spinal Muscular Atrophy (SMA, Types 2 and 3), Amyotrophic Lateral Sclerosis (ALS, upper limb-dominant), etc. 3. Peripheral Neuropathies: Charcot-Marie-Tooth (CMT) disease, etc. 4. Other Neuromuscular Conditions: Including but not limited to cervical spinal cord injury. 2. Aged over 10 years. 3. A score of 2 to 5 on the Brooke Upper Extremity Functional Rating Scale. 4. Manual Muscle Test (MMT) grade of less than 3 for shoulder abduction. 5. Ability to provide written informed consent from the participant and/or their legal representative, indicating willingness to participate in the study. Exclusion Criteria 1. Unwillingness or inability to provide informed consent. 2. A score of 1 or 6 on the Brooke Upper Extremity Functional Rating Scale. 3. Cognitive impairment severe enough to interfere with the proper use of a shoulder orthosis. 4. Any other condition which, in the opinion of the investigator, would make study participation inappropriate or unsafe for the patient.

Outcomes

Primary Outcomes

Performance of the upper limb module 2.0 (PUL 2.0)

The Performance of the Upper Limb module (PUL) was specifically designed to assess upper limb function across the spectrum of function in patients with neuromuscular disorder, providing information on three domains of upper limb function (shoulder, middle, distal) and overall upper limb functional abilities. Each domain (shoulder, middle, distal) can be scored separately, and the scores from the three levels are summed up to a maximum of 44 points.