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.
Study Type
OBSERVATIONAL
Enrollment
26
Hospital General Universitario Santa Lucia
Cartagena, Murcia, Spain
Measuring thickness of peripheral muscles using an ultrasound device
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.
Time frame: 3 months
Measuring echointensity of peripheral muscles using an ultrasound device
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.
Time frame: 3 months
Measuring sonoelastography of peripheral muscles using an ultrasound device
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.
Time frame: 3 months
Thickness Fraction of the Diaphragm in ALS patients and control group.
It is calculated as the difference between thickness at end inspiration and end expiration
Time frame: 3 months
Diaphragmatic Excursion (quiet and forced) of the Diaphragm in ALS patients and control group
Displacement of the right diaphragm dome
Time frame: 3 months
Measurement of muscle fasciculations using an ultrasound device
Each peripheral muscle will be screened during 30 seconds
Time frame: 3 months
Measurement of muscle strength using Medical Research Council Scale (MRCs)
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
Time frame: 3 months
Measurement Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-r)
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.
Time frame: 3 months
Measuring Forced Vital Capacity
This volume expressed in liters will be measured in supine and seated positions.
Time frame: 3 months
Maximal Inspiratory Pressure (MIP)
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
Time frame: 3 months
Sniff- nasal inspiratory pressure (SNIP)
It consists of measuring nasal pressure in an occluded nostril during a maximal sniff performed through the contralateral nostril, expressed in centimeters of water
Time frame: 3 months
Peak Cough Flow (PCF)
Maximal peak cough flow expressed in liters per minute
Time frame: 3 months
Partial Pressure of Carbon Dioxide in Arterial Blood (PaCO2)
Obtained by a radial arterial blood sample and expressed in millimeters of mercury
Time frame: 3 months
Cumulative time percentage with SpO2 under 90% (CT90)
SpO2 is an estimate of arterial oxygen saturation expressed in percentage under 90% obtained from a nocturnal oximetry
Time frame: 3 months
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