Telerehabilitation in Proximal Muscle Weakness

Study Overview
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Brief Summary
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The aim of our study is to investigate the effectiveness of two exercise programs supervised by a physiotherapist, performed in the hospital or at their home via electronic connection in a group of LGMD and SMA patients. One exercise session will consist of breathing, posture, dynamic core stabilization, upper and lower extremity strengthening exercises. The basic exercises from each group will be performed as 1 set of 5 repetitions at the beginning and will be gradually increased according to the tolerability of the patient. Fourteen subjects will be enrolled this randomized controlled study. Demographic characteristics, Vignos scale, Brooke scale, Barthel index, upper extremity functional index, Nottingham Health profile, short form-36, 6 minute walk test and muscle thicknesses measured by ultrasound of certain muscles will be recorded.

Detailed Description
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Some neuromuscular diseases with myogenic or neurogenic origin present with proximal muscle weakness. These diseases preferentially affect muscles around shoulders and hips and hence are called limb girdle neuromuscular diseases (LG-NMD). Patients have difficulty in climbing stairs or hills, and reaching high shelves. The vast majority of these diseases are X-linked recessive, autosomal recessive or dominantly inherited muscular dystrophies and are called limb girdle muscular dystrophies (LGMD). Spinal muscular atrophy (SMA), a disease of motor neurons, is also characterized by progressive proximal muscle weakness. Although the diseases are progressive in both conditions, regular and proper doses of exercise starting from the early stages may delay the progression of muscle weakness and may prevent joint stiffness, and contractures. The strengthening and aerobic exercise program increases overall wellness, condition and muscle strength in patients with neuromuscular diseases. The aerobic exercise program under supervision enhances oxidative capacity and muscle function without causing fatigue. Patients performing regular exercise program report an increase in their physical endurance, leg muscle strength and walking distance. Submaximal strengthening exercises combined with aerobic exercise are highly effective. Regulation of the exercise program according to the needs of the patient is the most important step. Appropriate individualized exercises should be programmed by a physical medicine and rehabilitation physician and performed under the supervision of a physiotherapist in the hospital environment in order to be effective. Otherwise, success of rehabilitation and patient compliance might be reduced. With the development of technology, patients can perform exercises at their home under remote supervision by a physiotherapist via electronic connection. Therefore, the aim of our study is to investigate the effectiveness of two exercise programs supervised by a physiotherapist, performed in the hospital or at their home via electronic connection in a group of LGMD and SMA patients. Fourteen consecutive patients from the Koç University Center for Muscle Diseases (KUCMD) diagnosed with LGMD or SMA are enrolled. Patients followed by KUCMD outpatient clinic with proximal muscle weakness who held LGMD or SMA diagnoses will be evaluated by a physical medicine and rehabilitation physician. Demographic characteristics, Vignos scale, Brooke scale, Barthel index, upper extremity functional index, Nottingham Health profile, short form-36, 6 minute walk test and muscle thicknesses measured by ultrasound of certain muscles will be recorded. Patients will be randomized into two groups. The first group will perform the exercises in the hospital under the supervision of a physiotherapist (Group 1: Hospital based rehabilitation group) and the second group will perform the exercises at their home under remote supervision of a physiotherapist via internet connection (Group 2: Telerehabilitation group). The exercise program will be administered 3 times in a week for 10 weeks. Patients will be examined by a blinded neurologist and functional assessments and ultrasonographic evaluations will be performed by a blinded physical medicine and rehabilitation physician before and after the rehabilitation program and at the 6th and 12th months following the completion of the rehabilitation. One exercise session will consist of breathing, posture, dynamic core stabilization, upper and lower extremity strengthening exercises. The basic exercises from each group will be performed as 1 set of 5 repetitions at the beginning and will be gradually increased according to the tolerability of the patient. If the patient has difficulty performing the basic exercises, modifications of the relevant exercise will be applied.

Official Title
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Is Telerehabilitation as Effective as the Traditional Hospital-based Rehabilitation in Patients With Proximal Muscle Weakness? - A Randomized Controlled Study

Conditions
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Telemedicine, Muscular Dystrophies, Proximal Myopathy

Intervention / Treatment
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* Other: exercise


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: To be older than 18 years To have proximal muscle weakness with a diagnosis of LGMD of any kind and SMA To have ability to walk independently Exclusion Criteria: history of cervical and lumbar radiculopathy history of cerebrovascular disease history of spinal cord injury

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 50 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Randomized
Intervention Model: Parallel Assignment
Interventional Model Description: There will be two group. The first group will perform the exercises in the hospital under the supervision of a physiotherapist (Group 1: Hospital based rehabilitation group) and the second group will perform the exercises at their home under remote supervision of a physiotherapist via internet connection (Group 2: Telerehabilitation group).
Masking: Single


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Active Comparator: Hospital based rehabilitation group<br>This group will perform the exercises in the hospital under the supervision of a physiotherapist | Other: exercise<br>* Breathing, posture, dynamic core stabilization, upper and lower extremity strengthening exercises.<br>|
| Active Comparator: Telerehabilitation group<br>This group will perform the exercises at their home under remote supervision of a physiotherapist via internet connection | Other: exercise<br>* Breathing, posture, dynamic core stabilization, upper and lower extremity strengthening exercises.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 6 minute walk test | This is a field test evaluating submaximal aerobic capacity. The individuals are asked to walk as far as possible in a 30-meter corridor in 6 minutes. The technical standards are defined by European Respiratory Society and American Thoracic Society. Six minute walking distance will be recorded. Higher walking distance shows better outcome. | 6 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Vignos scale | Vignos scale evaluates the functionality of the lower extremity and walking ability. This scale evaluates the patient's ability to standup from the chair, as well as to walk assisted with a long leg device in patients who can't walk without help. 1 indicates the best condition, 10 indicates the worst. | 6 months | 
| Brooke scale | Brooke scale evaluates the functionality of the upper extremity and hand skills ranging between 1- 6. 1 indicates the best condition, 6 indicates the worst. | 6 months | 
| Barthel index | Barthel index is a scale including ten items to assess functional disability. It is used to measure the performance of the individuals in daily life activities and the degree of care the patient needs in daily activities. 4. Total score is obtained by summing scores of 10 items. 0 indicates the worst, 100 indicates the best results. | 6 months | 
| Nottingham health profile | Nottingham health profile is a self-reported quality-of-life questionnaire. It consists of 38 items and evaluates 6 dimensions related to general health: energy, pain, emotional reactions, sleep, social isolation and physical activity. It is scored between 0-100 separately for each section. 0 indicates the best condition, 100 indicates the worst. The sum of the sub-scores gives the total Nottingham Health Profile score. | 6 months | 
| The upper extremity functional index | The upper extremity functional index. It consists of 20 items: each item is scored between 0 (extreme difficulty/inability to perform) and 4 (no difficulty) depending on the ability to perform specific activities. Total score is obtained by summing scores of 20 items. 0 indicates the worst, 80 indicates the best functional status. | 6 months | 
| Short form - 36 | Short form - 36 measures health related quality of life. It is a self-reported survey that evaluates individual health status with eight parameters consisting of physical function, pain, role limitations attributed to physical problems, role limitations attributed to emotional problems, mental health, social functioning, energy/ vitality, general health perception. There is not a summary score, each section is scored between 0-100, 0 indicates the worst condition, 100 indicates the best. | 6 months | 
| Muscle size and quality | All ultrasound evaluations will be performed using a MyLab Class C ultrasonic imaging system equipped with a 4-13-MHZ linear-array or 55 mm convex array according to the depth of the muscle. No compression to the skin will be applied. Evaluations will be performed by the same physiatrist who is blinded to the allocation groups. Measurements will be performed on the most affected side. Muscles assessed are supraspinatus, deltoid, biseps brachi for upper extremity; transversus abdominis, internal and external oblique for trunk; gluteus medius, quadriceps (rectus femoris, vastus intermedius, vastus lateralis, vastus medialis), medial gastrocnemius and hamstring muscles (semimembranosus, semitendinosus, biceps femoris) for the lower extremity. Qualitative analysis of all muscles will be performed using Heckmatt scale based on muscle echogenicity, grade 1 indicates normal, grade 4 the worst. | 6 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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telerehabilitation, telehealth, muscular dystrophy, proximal muscle weakness, spinal muscular atrophy, telemedicine