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

November 7, 2022 updated by: Aysenur Erekdag, Istanbul University - Cerrahpasa (IUC)

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

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.

Study Overview

Status

Completed

Conditions

Detailed Description

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.

Study Type

Observational

Enrollment (Actual)

24

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Locations

  • Turkey
      • Istanbul, Turkey, 34500
        • Istanbul University-Cerrahpasa

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

18 years to 55 years (Adult)

Accepts Healthy Volunteers

No

Genders Eligible for Study

All

Sampling Method

Probability Sample

Study Population

Patients diagnosed with FSHD and undergoing scapulothoracic arthrodesis or non-operating

Description

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

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

Cohorts and Interventions

Group / Cohort
scapulothoracic arthrodesis

Individuals diagnosed with FSHD who have undergone unilateral or bilateral surgery who meet the inclusion criteria.

application of determined outcome scales on patients
non-operative

Participants diagnosed with FSHD who have not undergone any surgery, who meet the inclusion criteria.

application of determined outcome scales on patients

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
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.
once at baseline of study
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.
once at baseline of study

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
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.
once at baseline of study
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.
once at baseline of study
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.
once at baseline of study
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.
once at baseline of study
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.
once at baseline of study
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.
once at baseline of study

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Istanbul University - Cerrahpasa (IUC)

Investigators

  • Study Director: Ipek Yeldan, PhD, Istanbul University - Cerrahpasa (IUC)

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

November 27, 2021

Primary Completion (Actual)

February 23, 2022

Study Completion (Actual)

August 16, 2022

Study Registration Dates

First Submitted

August 21, 2021

First Submitted That Met QC Criteria

August 21, 2021

First Posted (Actual)

August 26, 2021

Study Record Updates

Last Update Posted (Actual)

November 8, 2022

Last Update Submitted That Met QC Criteria

November 7, 2022

Last Verified

November 1, 2022

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • 38981562-900-

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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