Quantitative Muscle Ultrasound as a Marker of Progression in Children With Muscular Diseases

Quantitative Muscle Ultrasound as a Monitoring Tool of Disease Progression in Children With Inflammatory Myositis and Duchenne Muscular Dystrophy

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.

Study Overview

• Status: Completed
• Conditions: Duchenne Muscular Dystrophy; Inflammatory Myopathy
• Intervention / Treatment: Diagnostic test: Quantitative muscle ultrasound measurements

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

• Study Type: Observational
• Enrollment (Actual): 48

Contacts and Locations

Study Locations

• Egypt
  • Qalubiya
    • Banhā, Qalubiya, Egypt, 13518
      • Benha University Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 2 years to 16 years (CHILD)
• Accepts Healthy Volunteers: N/A
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

the study will be performed on 2 groups Group (I): fifty children diagnosed to have inflammatory myositis and Duchenne muscular dystrophy Group (II): including 20 healthy children matching age and sex as control group.

Description

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.

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
children with muscle disease; fifty children diagnosed to have inflammatory myositis or Duchenne muscular dystrophy in whom Quantitative muscle ultrasound measurements will be performed .The captured images will be analyzed for echo intensity by means of computer-assisted grayscale histogram analysis at baseline and after 24 months.	Diagnostic test: Quantitative muscle ultrasound measurements; Quantitative ultrasound measurements will be performed to biceps, forearm flexors, quadriceps and tibialis anterior according to a standard protocol; for each muscle three consecutive measurements will be made to minimize variation in echo intensity during analysis .The captured images will be analyzed offline for echo intensity by means of computer-assisted grayscale histogram analysis.
control group; 20 healthy children matching age and sex as control group in whom Quantitative muscle ultrasound measurement will be performed at baseline	Diagnostic test: Quantitative muscle ultrasound measurements; Quantitative ultrasound measurements will be performed to biceps, forearm flexors, quadriceps and tibialis anterior according to a standard protocol; for each muscle three consecutive measurements will be made to minimize variation in echo intensity during analysis .The captured images will be analyzed offline for echo intensity by means of computer-assisted grayscale histogram analysis.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Kendall's manual muscle testing	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)	24 months
Childhood myositis assessment scale	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)	24 months
Serum creatine kinase (CK) levels	CK measured in U/L using ELISA	24 months
Serum Lactate dehydrogenase (LDH) levels	CK measured in IU/L using ELISA	24 months
Aspartate aminotransferase (AST)	AST measured in U/L using ELISA	24 MONTHS
alanine aminotransferase (ALT)	ALT measured in U/L using ELISA	24 months
motor unit potential (MUP) duration	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.	24 months
motor unit peak-to-peak amplitude	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	24 months
motor unit area to amplitude ratio (AAR)	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 .	24 months

Collaborators and Investigators

• Sponsor: Benha University

Study record dates

Study Major Dates

• Study Start (ACTUAL): March 8, 2016
• Primary Completion (ACTUAL): February 2, 2019
• Study Completion (ACTUAL): February 2, 2019

Study Registration Dates

• First Submitted: December 21, 2018
• First Submitted That Met QC Criteria: December 21, 2018
• First Posted (ACTUAL): December 26, 2018

Study Record Updates

• Last Update Posted (ACTUAL): February 8, 2019
• Last Update Submitted That Met QC Criteria: February 7, 2019
• Last Verified: February 1, 2019

More Information

Terms related to this study

• Keywords: Duchenne Muscular Dystrophy; Musculoskeletal ultrasound; Inflammatory myositis
• Additional Relevant MeSH Terms: Nervous System Diseases; Genetic Diseases, Inborn; Genetic Diseases, X-Linked; Musculoskeletal Diseases; Muscular Diseases; Neuromuscular Diseases; Muscular Disorders, Atrophic; Muscular Dystrophies; Myositis; Muscular Dystrophy, Duchenne
• Other Study ID Numbers: BenhaU122018

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No