Telerehabilitation in Proximal Muscle Weakness

Telemedicine Clinical Trial

Official title:

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

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT04392518
• Other study ID #: 2018.150.IRB.1.017
• Status: Recruiting
• Phase: N/A
• Start date: September 15, 2019
• Est. completion date: November 2021

Study information

• Verified date: July 2021
• Source: Koç University
• Contact: Ihsan Solaroglu, Prof
• Phone: + 90 212 338 10 39
• Email: isolaroglu[@]ku.edu.tr
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial Summary

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.

Description:

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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 14
• Est. completion date: November 2021
• Est. primary completion date: September 2021
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years to 50 Years

Eligibility

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

Related Conditions & MeSH terms

• Muscle Weakness
• Muscular Dystrophies
• Paresis
• Proximal Myopathy
• Telemedicine

Intervention

Other:
• exercise
Breathing, posture, dynamic core stabilization, upper and lower extremity strengthening exercises.

Locations

Country	Name	City	State
Turkey	Koc University School of Medicine	Istanbul

Sponsors (1)

Lead Sponsor	Collaborator
Koç University

Country where clinical trial is conducted

Turkey, 

References & Publications (20)

Aytar A, Yuruk ZO, Tuzun EH, Baltaci G, Karatas M, Eker L. The Upper Extremity Functional Index (UEFI): cross-cultural adaptation, reliability, and validity of the Turkish version. J Back Musculoskelet Rehabil. 2015;28(3):489-95. doi: 10.3233/BMR-140545. — View Citation

Balius R, Pedret C, Iriarte I, Sáiz R, Cerezal L. Sonographic landmarks in hamstring muscles. Skeletal Radiol. 2019 Nov;48(11):1675-1683. doi: 10.1007/s00256-019-03208-x. Epub 2019 Apr 17. Review. — View Citation

Berthelsen MP, Husu E, Christensen SB, Prahm KP, Vissing J, Jensen BR. Anti-gravity training improves walking capacity and postural balance in patients with muscular dystrophy. Neuromuscul Disord. 2014 Jun;24(6):492-8. doi: 10.1016/j.nmd.2014.03.001. Epub — View Citation

Brooke MH, Griggs RC, Mendell JR, Fenichel GM, Shumate JB, Pellegrino RJ. Clinical trial in Duchenne dystrophy. I. The design of the protocol. Muscle Nerve. 1981 May-Jun;4(3):186-97. — View Citation

Cho J, Lee K, Kim M, Hahn J, Lee W. The Effects of Double Oscillation Exercise Combined with Elastic Band Exercise on Scapular Stabilizing Muscle Strength and Thickness in Healthy Young Individuals: A Randomized Controlled Pilot Trial. J Sports Sci Med. 2 — View Citation

Collin C, Wade DT, Davies S, Horne V. The Barthel ADL Index: a reliability study. Int Disabil Stud. 1988;10(2):61-3. — View Citation

Demiral Y, Ergor G, Unal B, Semin S, Akvardar Y, Kivircik B, Alptekin K. Normative data and discriminative properties of short form 36 (SF-36) in Turkish urban population. BMC Public Health. 2006 Oct 9;6:247. — View Citation

Gnat R, Saulicz E, Miadowicz B. Reliability of real-time ultrasound measurement of transversus abdominis thickness in healthy trained subjects. Eur Spine J. 2012 Aug;21(8):1508-15. doi: 10.1007/s00586-012-2184-4. Epub 2012 Feb 12. Erratum in: Eur Spine J. — View Citation

Heckmatt JZ, Leeman S, Dubowitz V. Ultrasound imaging in the diagnosis of muscle disease. J Pediatr. 1982 Nov;101(5):656-60. — View Citation

Holland AE, Spruit MA, Troosters T, Puhan MA, Pepin V, Saey D, McCormack MC, Carlin BW, Sciurba FC, Pitta F, Wanger J, MacIntyre N, Kaminsky DA, Culver BH, Revill SM, Hernandes NA, Andrianopoulos V, Camillo CA, Mitchell KE, Lee AL, Hill CJ, Singh SJ. An o — View Citation

Jenkins ND, Miller JM, Buckner SL, Cochrane KC, Bergstrom HC, Hill EC, Smith CM, Housh TJ, Cramer JT. Test-Retest Reliability of Single Transverse versus Panoramic Ultrasound Imaging for Muscle Size and Echo Intensity of the Biceps Brachii. Ultrasound Med — View Citation

Kücükdeveci AA, McKenna SP, Kutlay S, Gürsel Y, Whalley D, Arasil T. The development and psychometric assessment of the Turkish version of the Nottingham Health Profile. Int J Rehabil Res. 2000 Mar;23(1):31-8. — View Citation

Kuyumcu ME, Halil M, Kara Ö, Çuni B, Çaglayan G, Güven S, Yesil Y, Arik G, Yavuz BB, Cankurtaran M, Özçakar L. Ultrasonographic evaluation of the calf muscle mass and architecture in elderly patients with and without sarcopenia. Arch Gerontol Geriatr. 201 — View Citation

Narayanaswami P, Weiss M, Selcen D, David W, Raynor E, Carter G, Wicklund M, Barohn RJ, Ensrud E, Griggs RC, Gronseth G, Amato AA; Guideline Development Subcommittee of the American Academy of Neurology; Practice Issues Review Panel of the American Associ — View Citation

Siciliano G, Simoncini C, Giannotti S, Zampa V, Angelini C, Ricci G. Muscle exercise in limb girdle muscular dystrophies: pitfall and advantages. Acta Myol. 2015 May;34(1):3-8. Review. — View Citation

Strasser EM, Draskovits T, Praschak M, Quittan M, Graf A. Association between ultrasound measurements of muscle thickness, pennation angle, echogenicity and skeletal muscle strength in the elderly. Age (Dordr). 2013 Dec;35(6):2377-88. doi: 10.1007/s11357- — View Citation

VIGNOS PJ Jr, SPENCER GE Jr, ARCHIBALD KC. Management of progressive muscular dystrophy in childhood. JAMA. 1963 Apr 13;184:89-96. — View Citation

Vissing J. Limb girdle muscular dystrophies: classification, clinical spectrum and emerging therapies. Curr Opin Neurol. 2016 Oct;29(5):635-41. doi: 10.1097/WCO.0000000000000375. Review. — View Citation

Ware JE: Scoring the SF-36. SF-36 Health Survey: Manual and Interpretation Guide 1993

Whittaker JL, Emery CA. Sonographic measures of the gluteus medius, gluteus minimus, and vastus medialis muscles. J Orthop Sports Phys Ther. 2014 Aug;44(8):627-32. doi: 10.2519/jospt.2014.5315. Epub 2014 Jul 16. — View Citation

* Note: There are 20 references in all — Click here to view all references

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	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	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
Secondary	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
Secondary	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
Secondary	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
Secondary	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
Secondary	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
Secondary	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