- ICH GCP
- US Clinical Trials Registry
- Clinical Trial NCT03683875
Ultrasonography of the Neuromuscular Degeneration Behavior in Amyotrophic Lateral Sclerosis
Ultrasonography of the Neuromuscular Degeneration Behavior in ALS: a Longitudinal Study
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.
- To evaluate the evolution of diaphragmatic and peripheral neuromuscular degeneration by ultrasound examination in patients with ALS and to establish possible evolution patterns.
- To verify the relationship between the degenerative peripheral and diaphragmatic neuromuscular changes evaluated by ultrasonography and changes in clinical scales frequently used.
- 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.
Study Overview
Status
Conditions
Study Type
Enrollment (Actual)
Contacts and Locations
Study Locations
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Murcia
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Cartagena, Murcia, Spain, 30202
- Hospital General Universitario Santa Lucia
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Participation Criteria
Eligibility Criteria
Ages Eligible for Study
Accepts Healthy Volunteers
Genders Eligible for Study
Sampling Method
Study Population
Description
Inclusion Criteria:
- Definite ALS
Exclusion Criteria:
- Primary Lateral Sclerosis (PLS)
- Other forms of motor neuron disorders
Study Plan
How is the study designed?
Design Details
What is the study measuring?
Primary Outcome Measures
Outcome Measure |
Measure Description |
Time Frame |
---|---|---|
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.
|
3 months
|
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 months
|
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.
|
3 months
|
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
|
3 months
|
Diaphragmatic Excursion (quiet and forced) of the Diaphragm in ALS patients and control group
Time Frame: 3 months
|
Displacement of the right diaphragm dome
|
3 months
|
Secondary Outcome Measures
Outcome Measure |
Measure Description |
Time Frame |
---|---|---|
Measurement of muscle fasciculations using an ultrasound device
Time Frame: 3 months
|
Each peripheral muscle will be screened during 30 seconds
|
3 months
|
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 months
|
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:
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. |
3 months
|
Measuring Forced Vital Capacity
Time Frame: 3 months
|
This volume expressed in liters will be measured in supine and seated positions.
|
3 months
|
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
|
3 months
|
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
|
3 months
|
Peak Cough Flow (PCF)
Time Frame: 3 months
|
Maximal peak cough flow expressed in liters per minute
|
3 months
|
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
|
3 months
|
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
|
3 months
|
Collaborators and Investigators
Collaborators
Investigators
- Principal Investigator: Antonio Tomás Ríos Cortés, HOSPITAL GENERAL UNIVERSITARIO SANTA LUCIA - SERVICIO MURCIANO DE SALUD
Publications and helpful links
General Publications
- Arts IM, van Rooij FG, Overeem S, Pillen S, Janssen HM, Schelhaas HJ, Zwarts MJ. Quantitative muscle ultrasonography in amyotrophic lateral sclerosis. Ultrasound Med Biol. 2008 Mar;34(3):354-61. doi: 10.1016/j.ultrasmedbio.2007.08.013. Epub 2007 Oct 26.
- Arts IM, Pillen S, Schelhaas HJ, Overeem S, Zwarts MJ. Normal values for quantitative muscle ultrasonography in adults. Muscle Nerve. 2010 Jan;41(1):32-41. doi: 10.1002/mus.21458.
- Mayans D, Cartwright MS, Walker FO. Neuromuscular ultrasonography: quantifying muscle and nerve measurements. Phys Med Rehabil Clin N Am. 2012 Feb;23(1):133-48, xii. doi: 10.1016/j.pmr.2011.11.009. Epub 2011 Dec 9.
- Arts IMP, Overeem S, Pillen S, Jurgen Schelhaas H, Zwarts MJ. Muscle changes in amyotrophic lateral sclerosis: a longitudinal ultrasonography study. Clin Neurophysiol. 2011 Mar;122(3):623-628. doi: 10.1016/j.clinph.2010.07.023.
- Arts IM, Overeem S, Pillen S, Kleine BU, Boekestein WA, Zwarts MJ, Jurgen Schelhaas H. Muscle ultrasonography: a diagnostic tool for amyotrophic lateral sclerosis. Clin Neurophysiol. 2012 Aug;123(8):1662-7. doi: 10.1016/j.clinph.2011.11.262. Epub 2012 Jan 13.
- Arts IM, Overeem S, Pillen S, Schelhaas HJ, Zwarts MJ. Muscle ultrasonography to predict survival in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 2011 May;82(5):552-4. doi: 10.1136/jnnp.2009.200519. Epub 2010 Apr 14.
- Schreiber S, Abdulla S, Debska-Vielhaber G, Machts J, Dannhardt-Stieger V, Feistner H, Oldag A, Goertler M, Petri S, Kollewe K, Kropf S, Schreiber F, Heinze HJ, Dengler R, Nestor PJ, Vielhaber S. Peripheral nerve ultrasound in amyotrophic lateral sclerosis phenotypes. Muscle Nerve. 2015 May;51(5):669-75. doi: 10.1002/mus.24431. Epub 2015 Mar 14.
- Cartwright MS, Walker FO, Griffin LP, Caress JB. Peripheral nerve and muscle ultrasound in amyotrophic lateral sclerosis. Muscle Nerve. 2011 Sep;44(3):346-51. doi: 10.1002/mus.22035. Epub 2011 Aug 3.
- Martinez-Paya JJ, Del Bano-Aledo ME, Rios-Diaz J, Tembl-Ferrairo JI, Vazquez-Costa JF, Medina-Mirapeix F. Muscular Echovariation: A New Biomarker in Amyotrophic Lateral Sclerosis. Ultrasound Med Biol. 2017 Jun;43(6):1153-1162. doi: 10.1016/j.ultrasmedbio.2017.02.002. Epub 2017 Apr 8.
- Martinez-Paya JJ, Rios-Diaz J, Del Bano-Aledo ME, Tembl-Ferrairo JI, Vazquez-Costa JF, Medina-Mirapeix F. Quantitative Muscle Ultrasonography Using Textural Analysis in Amyotrophic Lateral Sclerosis. Ultrason Imaging. 2017 Nov;39(6):357-368. doi: 10.1177/0161734617711370. Epub 2017 May 28.
- Hiwatani Y, Sakata M, Miwa H. Ultrasonography of the diaphragm in amyotrophic lateral sclerosis: clinical significance in assessment of respiratory functions. Amyotroph Lateral Scler Frontotemporal Degener. 2013 Mar;14(2):127-31. doi: 10.3109/17482968.2012.729595. Epub 2012 Oct 30.
- Pinto S, Alves P, Pimentel B, Swash M, de Carvalho M. Ultrasound for assessment of diaphragm in ALS. Clin Neurophysiol. 2016 Jan;127(1):892-897. doi: 10.1016/j.clinph.2015.03.024. Epub 2015 Apr 25.
- Fantini R, Mandrioli J, Zona S, Antenora F, Iattoni A, Monelli M, Fini N, Tonelli R, Clini E, Marchioni A. Ultrasound assessment of diaphragmatic function in patients with amyotrophic lateral sclerosis. Respirology. 2016 Jul;21(5):932-8. doi: 10.1111/resp.12759. Epub 2016 Mar 19.
Study record dates
Study Major Dates
Study Start (Actual)
Primary Completion (Actual)
Study Completion (Actual)
Study Registration Dates
First Submitted
First Submitted That Met QC Criteria
First Posted (Actual)
Study Record Updates
Last Update Posted (Actual)
Last Update Submitted That Met QC Criteria
Last Verified
More Information
Terms related to this study
Additional Relevant MeSH Terms
Other Study ID Numbers
- ECO-ELA
Plan for Individual participant data (IPD)
Plan to Share Individual Participant Data (IPD)?
Drug and device information, study documents
Studies a U.S. FDA-regulated drug product
Studies a U.S. FDA-regulated device product
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