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NCT Number: NCT05022355

Examination of Operative Approach in pwFSHD (Patient With Facioscapulohumeral Muscular Dystrophy)

Facioscapulohumeral Muscular Dystrophy (FSHD) is one of the most common forms of muscular dystrophy, characterized by pronounced skeletal novelistic weakness and with a broad spectrum of diseases. It is a hereditary disease seen in 3-5/100,000 of society, usually starting with weakness in the facial and shoulder muscles and progressing to the trunk, pelvis and leg muscles, giving symptoms in the twenties. In FSHD, which shows slow progression and can lead to loss of ambulation ability in about 20% of patients, patients may have difficulty performing activities above shoulder level with the influence of the periscapular area.

Skeletal muscle weakness leads to posture and balance disorders, and postural instability is a common problem in patients with FSHD. Weakness of the trunk and lower limb muscles found in more than half of patients causes problems with postural balance and walking. The calf, iliopsoas, and gluteus maximus muscles together form the main determinants of walking speed in healthy people, where kalf muscles are known to contribute the most. Individuals with FSHD have been reported to have decreased speed, step length, and step frequency compared to healthy controls. Impaired upper body control can compromise the maintenance of dynamic stability. In patient with FSHD the effects of muscle tone, motor coordination, loss of joint range of motion and muscle weakness on posture, balance control and gait are observed more clearly.

The aim of the study was to compare the effects of scapular management treatments on balance and gait in FSHD patients.

H0: There is no difference in balance and walking parameters of patients with FSHD who have had scapulothoracic arthrodesis surgery and have not undergone surgery.

H1: There is a difference in balance and walking parameters of patients with FSHD who have had scapulothoracic arthrodesis surgery and have not undergone surgery.

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Key information

Conditions

Facio-Scapulo-Humeral Dystrophy Congenital, Hereditary, and Neonatal Diseases and Abnormalities Genetic Diseases, Inborn Muscular Diseases Muscular Disorders, Atrophic Muscular Dystrophies Muscular Dystrophy, Facioscapulohumeral Musculoskeletal Diseases Nervous System Diseases Neuromuscular Diseases

Age range

18 year–55 year

Sex eligibility

All sexes

Study type

Observational

Primary location

Istanbul University-Cerrahpaşa

Istanbul, 34500, Turkey (Türkiye)

About this study

Voluntary patients who have been diagnosed with FSHD will be included in the study. Signed voluntary consent will be obtained from participants. Participants will be divided into two groups. One group will include patients who have undergone scapulothoracic arthrodesis surgery, while the other group will include patients who have not undergone surgery. Both groups will undergo some outcome measures to assess their balance and walking.

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • 18-65 years
  • Score between 0,5 and 3,5 according to CSS (clinical severity rating)
  • Score between 3 and 5 according to FAS (functional ambulation scale)
  • Being included in the surgical group the patient has undergone unilateral or bilateral scapulothoracic arthrodesis surgery

Exclusion criteria

  • Presence of lower extremity orthopedic problems/problems that can cause balance and walking problems
  • Presence of any other upper limb orthopedic problems/problems and surgery
  • Having undergone Spinal fusion surgery
  • Presence of additional neurological problems/problems
  • Having a level of visual and auditory problems that will prevent communication

Treatment and study plan

Primary outcomes

  1. 10 meter walking test

    Time frame: once at baseline of study

    during the test, the patient is asked to walk at a normal walking speed at a distance of 10 meters determined before the test. The time it has traveled the specified distance is recorded. It is a long-term performance test used for dynamic balance assessment.

  2. Gait Analyzer

    Time frame: once at baseline of study

    It is a smartphone-based application for analyzing walking parameters in real time. It can measure walking speed, step time, step length, cadence and symmetry.

Secondary outcomes

  1. Berg Balance Scale

    Time frame: once at baseline of study

    It is a scale containing 14 instructions and a score of 0-4 is given by observing the patient's performance for each instruction. 0 points are given in cases where the patient cannot perform the activity at all, while 4 points are given when the patient completes the activity independently. The highest score is 56 and 0-20 points indicate balance disorder, 21-40 points indicate an acceptable balance, 41-56 points indicate the presence of a good balance. It takes between 10 and 20 minutes to complete the scale.

  2. Disabilities of the arm, shoulder and hand (DASH)

    Time frame: once at baseline of study

    It is a 30-item questionnaire that examines the patient's ability to perform certain upper limb activities. This survey is a self-report questionnaire in which patients can assess difficulty and intervention in daily life on a Likert scale of 5 points.

  3. 30 seconds sit up test

    Time frame: once at baseline of study

    It is a test that evaluates the patient's sit-up activity, lower limb strength and dynamic balance. A 30-second period is initiated when the patient cuts the contact of his pelvis from the chair in which he is sitting. He is constantly asked to sit on the chair and get up from there within 30 seconds. It is scored by counting how many repetitions the patient applies this command within 30 seconds.

  4. 2-minute walk test

    Time frame: once at baseline of study

    it is recommended that the area where the test will be performed should be a corridor with a length of 30 meters, a flat and hard floor. It is necessary to determine the starting and ending points of the field to be tested. The patient is asked to walk the maximum distance he can walk within 2 minutes by standing at the starting point. When it expires, the distance it travels is recorded.

  5. Single leg stance test

    Time frame: once at baseline of study

    It is used to evaluate static posture and balance control. The patient must stand unaided on one leg from the moment the other foot leaves the ground until the foot touches the ground again or the arms are separated from the hips.

  6. Timed up and go test

    Time frame: once at baseline of study

    The Test is a measure of dynamic equilibrium. It requires individuals to get up from a chair, walk 3 feet, turn and sit. The time from the moment the individual lifts the pelvis from the chair until he or she returns with the pelvis in the chair is recorded in seconds.

Sponsors and collaborators

Lead sponsor

Istanbul University - Cerrahpasa

Other

Registry information

Official study title

The Comparison of the Effects of Scapulothoracic Arthrodesis Surgery Versus Usual Care on Balance and Gait Parameters in Patients With Fascioscapulohumeral Dystrophy: One-Blind Study

Important dates

Study start
2021
Primary completion
2022
Study completion
2022
First posted
Aug 26, 2021
Registry last updated
Nov 8, 2022

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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