Ultrasonography of the Neuromuscular Degeneration Behavior in Amyotrophic Lateral Sclerosis

Completed

• Conditions: Amyotrophic Lateral Sclerosis

• Registration Number: NCT03683875
• Lead Sponsor: Hospital General Universitario Santa Lucia

Brief Summary

Amyotrophic Lateral Sclerosis (ALS) is a progressive and fatal neurological disease.

An exhaustive and frequent clinical evaluation can lead to establish an adequate and early treatment of the consequences of its evolution.

Objectives.

1. To evaluate the evolution of diaphragmatic and peripheral neuromuscular degeneration by ultrasound examination in patients with ALS and to establish possible evolution patterns.

2. To verify the relationship between the degenerative peripheral and diaphragmatic neuromuscular changes evaluated by ultrasonography and changes in clinical scales frequently used.

3. To compare the ultrasonographic features of subjects with ALS and a sample of healthy subjects 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, with an interval of at least 3 months between tests. Bilateral and cross sectional ultrasonography of several peripheral muscles and diaphragm will be performed at rest and during muscle contraction. All the images will be processed and analyzed for obtaining morphometric variables (muscle thickness) and textural ones (echogenic variation, entropy, homogeneity, textural contrast and correlation). Frequency of twitches will be also recorded in peripheral muscles.Also clinical features will be noted, every time of the 3 exams, from Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-r), British Medical Council Research Scale(MRC), and routine pulmonary tests.

Detailed Description

Not available

Study Timeline

• Study First Submitted: 2018-09-12
• Study First Submitted QC: 2018-09-23
• Study First Posted: 2018-09-25
• Study Start: 2018-10-01
• Primary Completion: 2018-10-01
• Study Completion: 2020-03-10
• Status Verified: 2021-10
• Last Update Submitted: 2021-10-28
• Last Update Posted: 2021-11-05

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 26

Inclusion Criteria

• Definite ALS

Exclusion Criteria

• Primary Lateral Sclerosis (PLS)
• Other forms of motor neuron disorders

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Measuring echointensity of peripheral muscles using an ultrasound device	3 months	These measurements will be carried out in the biceps brachialis, forearm flexors, thenar eminence muscle, quadriceps and tibialis anterior which will be compared between patient with ALS and control group.
Thickness Fraction of the Diaphragm in ALS patients and control group.	3 months	It is calculated as the difference between thickness at end inspiration and end expiration
Measuring thickness of peripheral muscles using an ultrasound device	3 months	These measurements will be carried out in the biceps brachialis, forearm flexors, thenar eminence muscle, quadriceps and tibialis anterior which will be compared between patient with ALS and control group.
Measuring sonoelastography of peripheral muscles using an ultrasound device	3 months	These measurements will be carried out in the biceps brachialis, forearm flexors, thenar eminence muscle, quadriceps and tibialis anterior which will be compared between patient with ALS and control group.
Diaphragmatic Excursion (quiet and forced) of the Diaphragm in ALS patients and control group	3 months	Displacement of the right diaphragm dome

Secondary Outcome Measures

Name	Time	Method
Measurement of muscle fasciculations using an ultrasound device	3 months	Each peripheral muscle will be screened during 30 seconds
Measurement of muscle strength using Medical Research Council Scale (MRCs)	3 months	Graded on the medical research council scale. This scale grades muscle power on a scale of 0 to 5: Grade 0: no movement is observed Grade 1: only a trace or flicker of movement is seen or flet in the muscle. or fasciculation is observed Grade 2: movement is possible only if the resistance of gravity is removed Grade 3: movement against gravity is possible but not against resistance of the examiner Grade 4: muscle stregth is reduced but muscle contraction can move joint against gravity and resistance Grade 5: muscle contracts normally against full resistance
Measurement Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-r)	3 months	Questionnaire with 12 items with a score from 0 to 4 each item: 1. Speech; 2. Salivation; 3. Swallowing; 4. Handwriting 5a. Cutting food and handling utensils (patients without gastrostomy) 5b. Cutting food and handling utensils(scale for patients with gastrostomy); 6. Dressing and hygiene 7. Turning in bed and adjusting bed clothes 8. Walking 9. Climbing stairs 10. Dyspnea 11. Orthopnea 12. Respiratory insufficiency; There are three main pathways of progression, the questions are also divided in relation to the types of onset. Questions 1 to 3 are related to bulbar onset, questions 4 to 9 are related to limb onset and questions 10-12 are related to respiratory onset. The Scale goes from 0 to 48, more points meaning better situation.
Maximal Inspiratory Pressure (MIP)	3 months	Measure of the strength of inspiratory muscles expressed in centimeters of water, is the highest mouth pressure sustained for 1 s during a maximum inspiratory effort
Partial Pressure of Carbon Dioxide in Arterial Blood (PaCO2)	3 months	Obtained by a radial arterial blood sample and expressed in millimeters of mercury
Measuring Forced Vital Capacity	3 months	This volume expressed in liters will be measured in supine and seated positions.
Sniff- nasal inspiratory pressure (SNIP)	3 months	It consists of measuring nasal pressure in an occluded nostril during a maximal sniff performed through the contralateral nostril, expressed in centimeters of water
Peak Cough Flow (PCF)	3 months	Maximal peak cough flow expressed in liters per minute
Cumulative time percentage with SpO2 under 90% (CT90)	3 months	SpO2 is an estimate of arterial oxygen saturation expressed in percentage under 90% obtained from a nocturnal oximetry

Trial Locations

Locations (1)

Hospital General Universitario Santa Lucia; 🇪🇸 Cartagena, Murcia, Spain