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.

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.

Inclusion Criteria:

• Definite ALS

Exclusion Criteria:

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