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

Istanbul University - Cerrahpasa (IUC)

Status: Completed

Conditions: Facio-Scapulo-Humeral Dystrophy

Study type: Observational

Funder types: Other

Identifiers

NCT05022355

38981562-900-

Details and patient eligibility

About

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.

Full 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.

Enrollment: 24 patients

Sex: All

Ages: 18 to 55 years old

Volunteers: No Healthy Volunteers

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

Trial design

24 participants in 2 patient groups

scapulothoracic arthrodesis

Description:

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

Description:

Participants diagnosed with FSHD who have not undergone any surgery, who meet the inclusion criteria. application of determined outcome scales on patients