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NCT Number: NCT03683875

Ultrasonography of the Neuromuscular Degeneration Behavior in Amyotrophic Lateral Sclerosis

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.

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Key information

Conditions

Amyotrophic Lateral Sclerosis Central Nervous System Diseases Metabolic Diseases Motor Neuron Disease Nervous System Diseases Neurodegenerative Diseases Neuromuscular Diseases Nutritional and Metabolic Diseases Proteostasis Deficiencies Spinal Cord Diseases TDP-43 Proteinopathies

Age range

18 year and older

Sex eligibility

All sexes

Study type

Observational

Primary location

Hospital General Universitario Santa Lucia

Cartagena, Murcia, 30202, Spain

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • Definite ALS

Exclusion criteria

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

Treatment and study plan

Primary outcomes

  1. Measuring thickness of peripheral muscles using an ultrasound device

    Time frame: 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.

  2. Measuring echointensity of peripheral muscles using an ultrasound device

    Time frame: 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.

  3. Measuring sonoelastography of peripheral muscles using an ultrasound device

    Time frame: 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.

  4. Thickness Fraction of the Diaphragm in ALS patients and control group.

    Time frame: 3 months

    It is calculated as the difference between thickness at end inspiration and end expiration

  5. Diaphragmatic Excursion (quiet and forced) of the Diaphragm in ALS patients and control group

    Time frame: 3 months

    Displacement of the right diaphragm dome

Secondary outcomes

  1. Measurement of muscle fasciculations using an ultrasound device

    Time frame: 3 months

    Each peripheral muscle will be screened during 30 seconds

  2. Measurement of muscle strength using Medical Research Council Scale (MRCs)

    Time frame: 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

  3. Measurement Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-r)

    Time frame: 3 months

    Questionnaire with 12 items with a score from 0 to 4 each item:

    • Speech
    • Salivation
    • Swallowing
    • Handwriting 5a. Cutting food and handling utensils (patients without gastrostomy) 5b. Cutting food and handling utensils(scale for patients with gastrostomy)
    • 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.

  4. Measuring Forced Vital Capacity

    Time frame: 3 months

    This volume expressed in liters will be measured in supine and seated positions.

  5. Maximal Inspiratory Pressure (MIP)

    Time frame: 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

  6. Sniff- nasal inspiratory pressure (SNIP)

    Time frame: 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

  7. Peak Cough Flow (PCF)

    Time frame: 3 months

    Maximal peak cough flow expressed in liters per minute

  8. Partial Pressure of Carbon Dioxide in Arterial Blood (PaCO2)

    Time frame: 3 months

    Obtained by a radial arterial blood sample and expressed in millimeters of mercury

  9. Cumulative time percentage with SpO2 under 90% (CT90)

    Time frame: 3 months

    SpO2 is an estimate of arterial oxygen saturation expressed in percentage under 90% obtained from a nocturnal oximetry

Sponsors and collaborators

Lead sponsor

Hospital General Universitario Santa Lucia

Other

Collaborators

  • Universidad de Murcia

Registry information

Official study title

Ultrasonography of the Neuromuscular Degeneration Behavior in ALS: a Longitudinal Study

Important dates

Study start
2018
Primary completion
2018
Study completion
2020
First posted
Sep 25, 2018
Registry last updated
Nov 5, 2021

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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