The study aims to provide a timely update on the role of combining clinical and neuromuscular ultrasound assessments in diagnosis and follow-up of various muscle diseases in clinical practice over 12 months period, and correlating US findings with functional scales, biochemical and electrophysiological studies.
Many muscle diseases share common clinical features that render arriving at appropriate diagnoses difficult. The combination of muscle imaging with clinical can limit the differential diagnosis and yield the most probable one and can direct genetic testing as the only method to arrive at a definite diagnosis. In recent years, the use of high-resolution ultrasound had become an important tool in diagnosis and in the monitoring of disease progression and treatment of both hereditary and acquired myopathies. Additionally, it entails a safe, accessible, low-cost, and no ionizing radiation tool which renders the technique extremely suitable for paediatric patients and patients who cannot lie still without sedation. therefore, it can be used as a complementary tool to electro-diagnosis. Ultrasound permits to evaluate echo intensity, muscle perfusion, transverse and longitudinal sections of the muscle and its thickness at rest and during maximal voluntary contractions, overlying subcutaneous fat, cross-sectional area, and angled fibers of pennate muscles. The use of sonographically guided biopsy is an easy, safe, and reliable method for attaining tissue for histologic diagnosis in neuromuscular disease. In most myopathies, either acute or chronic, muscle tissue undergoes morphological changes giving rise to replacement of muscle by connective tissue and/or fat. Pattern recognition on muscle imaging might be helpful in distinguishing between different disease entities.
Study Type
OBSERVATIONAL
Enrollment
128
Quantitative ultrasound measurements will be performed to studied muscles 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.The study will be performed using My lab 7 ultrasound system (Esaote company, Italy) that is equipped by 7-19 MHz linear array transducer and color and power Doppler. Technique: evaluation of studied muscles for echo intensity (ECHO), quantitative assessments of echointensity, muscle perfusion, transverse and longitudinal sections of the muscle and its thickness at rest and during maximal voluntary contractions (MVC), overlying subcutaneous fat, cross-sectional area (CSA), and angled fibers of pennate muscles.
Includes: Motor and sensory nerve conduction study, F-wave and H-reflex study to assess the proximal roots, Electromyography (EMG) of the studied muscles. using machine: recordings will be performed with a Nihon Kohden equipment (model 7102) with the following parameters: sweep time 8 ms/D, sensitivity 0.5 mV/D, low frequency filter 2 Hz, high frequency filter 10 kHz, stimulation duration 0.1 ms and stimulation frequency 1 Hz.
Assiut University Hospital
Asyut, Egypt
The rate of decline of patients with muscle disorder versus normal subjects as assessed by quantitative ultrasound measurements and electrophysiology studies.
With the successful completion of this aim, the investigators will establish that alterations in both quantitative ultrasound and electromyography will provide meaningful measures of disease progression.
Time frame: up to 12 months
Rate of change in Functional assessment of muscle weakness
Grading score from 0-10 according to affected muscles
Time frame: up to 12 months
Rate of change in manual muscle strength testing by EXPANDED MRC (The modified Medical Research Council)
Grading scale from 0 -5 points, measures strength of each muscle group score 0 is the weakest (worst) and 5 is the strongest (best)
Time frame: up to 12 months
Rate of change of serum CPK and CK-MM levels
measured in U/L using ELISA
Time frame: up to 12 months
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measured in U/L using ELISA.