Quantitative Neuromuscular Ultrasonography in Amyotrophic Lateral Sclerosis (ALS)

Study Overview
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Brief Summary
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Amyotrophic Lateral Sclerosis (ALS) is a progressive and fatal neurological disease. Nonspecific symptoms lead to a delay in the diagnosis, only confirmed by the electrophysiologic study. Objectives. To establish the diagnostic value of ultrasonography in ALS. To evaluate the rate of muscle and nerve degeneration by ultrasonography in patients with ALS. To check the relationship between ultrasound, clinical variables and functional tests in patients with ALS. Methods. A longitudinal observational study in a consecutive sample of patients diagnosed with ALS will be realized. All the patients will be examined 3 times during 6 months and capabilities associated with ALS and muscle strength will be assessed. Bilateral and cross sectional ultrasonography of several muscles and also median and tibial nerves will be performed. All the images will be processed and analyzed for obtaining morphometric variables (muscle thickness and nerve area) and textural ones (echogenic variation, entropy, homogeneity, textural contrast and correlation). Frequency of twitches will be also recorded. After longitudinal study, a survival study will be performed in relation to functional and sonographic variables.

Official Title
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Ultrasound Markers for the Diagnosis, Disease Progression and Prognosis of Amyotrophic Lateral Sclerosis

Conditions
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Amyotrophic Lateral Sclerosis

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Definite ALS. Exclusion Criteria: Primary Lateral Sclerosis (PLS) Other forms of motor neuron disorders.

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Measuring muscle thickness. | This measurement is carried out in the biceps brachialis, forearm flexors, quadriceps and tibialis anterior, which will be compared between patient with ALS and control group. | 6 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Measurement muscle strength. | Graded on the medical research council scale | 6 months | 
| Measurement ALS Functional Rating Scale (ALSFRS-r) scale. | Recorded as the global score | 6 months | 
| Measurement muscle fasciculations. | Each muscle will be screened during 10 s. | 6 months | 
| Measuring muscle echointensity. | It will be measured using the Image J (v.1.48) software in the biceps brachialis, forearm flexors, quadriceps and tibialis anterior, which will be compared between patient with ALS and control group. | 6 months | 
| Measuring nerve area. | This measurement is carried out in the median and tibialis posterior nerves, which will be compared between patient with ALS and control group. | 6 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Amyotrophic lateral sclerosis, Ultrasonography, Motor neurons, Neuromuscular diseases, Fasciculation