Quantitative Muscle Ultrasound as a Monitoring Tool of Disease Progression in Children With Inflammatory Myositis and Duchenne Muscular Dystrophy
Overview
- Phase
- Not Applicable
- Intervention
- Not specified
- Conditions
- Inflammatory Myopathy
- Sponsor
- Benha University
- Enrollment
- 48
- Locations
- 1
- Primary Endpoint
- motor unit peak-to-peak amplitude
- Status
- Completed
- Last Updated
- 7 years ago
Overview
Brief Summary
The aim of our study is to Assess skeletal muscle structural status in children with inflammatory myositis and Duchenne muscular dystrophy using musculoskeletal ultrasound and to perform a longitudinal follow up of these changes over 2 years and to assess the relation between these findings with clinical parameters, functional scales, biochemical and electromyographic tests.
Detailed Description
This study will be carried out on two groups: • Group (I): fifty children diagnosed to have duchenne muscular dystrophy and inflammatory myositis. Group (II): including 20 healthy children matching age and sex as control group. patients will be subjected to (A) Clinical evaluation 1. Complete history taking. 2. Thorough clinical examination. 3. Body mass index (BMI) assessment. 4. Quantitative muscle strength tests 5. Functional grading 6. Childhood Myositis Assessment Scale. 7 (B) Laboratory assessment: All patients will be subjected to the following measurements: 1. Serum creatine kinase levels (CK). 2. Serum Lactate dehydrogenase levels 3. Serum of Liver enzymes (SGOT\& SGPT) levels. (C) Electromyographic (EMG) assessment: (D) Musculoskeletal ultrasound assessment (E) Statistical analysis
Investigators
Waleed Ahmed Salaheldeen Hassan
Assistant professor
Benha University
Eligibility Criteria
Inclusion Criteria
- •children with Duchenne muscular dystrophy (DMD). Diagnosis with DMD was established according to DMD diagnostic criteria (Jennekens et al., 1991).
- •children with juvenile dermatomyositis (JDM) according to Bohan and Peter diagnostic criteria ( (Bohan and Peter, 1975).
Exclusion Criteria
- •Patients with age less than 2 years were excluded from the study due to inability to perform manual muscle testing and functional scales.
- •If no final diagnosis could be established.
- •The presence of a concomitant illness that may result in peripheral neuropathy or myopathy.
Outcomes
Primary Outcomes
motor unit peak-to-peak amplitude
Time Frame: 24 months
quantitative electromyography (QEMG) in the most affected rectus femoris and biceps brachii muscles will be performed and The motor unit potentials will be reviewed offline for the needle-detected EMG signals will be analyzed by the device software for the peak-to-peak amplitude measured in microvolt
motor unit area to amplitude ratio (AAR)
Time Frame: 24 months
quantitative electromyography (QEMG) in the most affected rectus femoris and biceps brachii muscles will be performed and The motor unit potentials will be reviewed offline for the needle-detected EMG signals will be analyzed by the device software for the motor unit AAR .
Serum creatine kinase (CK) levels
Time Frame: 24 months
CK measured in U/L using ELISA
Serum Lactate dehydrogenase (LDH) levels
Time Frame: 24 months
CK measured in IU/L using ELISA
Aspartate aminotransferase (AST)
Time Frame: 24 MONTHS
AST measured in U/L using ELISA
alanine aminotransferase (ALT)
Time Frame: 24 months
ALT measured in U/L using ELISA
Kendall's manual muscle testing
Time Frame: 24 months
Kendall's 0 -10 point scale measures strength of each muscle group score 0 is the weakest (worst) and 10 is the strongest (best). The following muscles were tested bilaterally: the biceps brachii muscle (BB), the forearm flexors (FF), the rectus femoris muscle (RF), the tibialis anterior muscle (TA)
Childhood myositis assessment scale
Time Frame: 24 months
used to assess the severity of muscle involvement in children with dermatomyositis. The scores for the 14 items are summated to give a total score ranging from 0 (worst) to 52 (best)
motor unit potential (MUP) duration
Time Frame: 24 months
quantitative electromyography (QEMG) in the most affected rectus femoris and biceps brachii muscles will be performed and The motor unit potentials will be reviewed offline for the needle-detected EMG signals will be analyzed by the device software for the MUP duration measured in milliseconds.