Abstract

Background Several methods have been proposed to diagnose and score disease severity in Amyotrophic Lateral Sclerosis (ALS) patients (pt.). Traditionally neurophysiological tests have been employed. The development of neuromuscular ultrasound (US) have provided a further useful tool. Our aim was to evaluate sonographic changes of muscles and nerve trunks to define diagnosis and assess ALS severity. Materials and methods 20 ALS pt. (15 m e 5 f, mean age: 63.9 + 9.5 yrs) were enrolled. All investigations were performed using an Esaote MyLabGamma device in conventional B-Mode. We evaluated the following muscles: sternocleidomastoid, diaphragm, biceps brachii/brachialis, forearm flexors, rectus femoris and anterior tibial; among the nerve, vagus, median, ulnar and sural. Results We found in all cases increased echogenicity with decreased muscle thickness and fasciculations; mean diaphragm rest thickness was reduced; changes in thickness during inspiration and expiration were also reduced and loss in the most severe cases. Median and Ulnar nerve cross-sectional area (CSA) resulted smaller; sural and vagal nerve resulted spared. ALS disease severity, measured using Functional Rating Scale (ALSFRS), correlated with quantitative ultrasound data. Conclusions neuromuscular ultrasound resulted a simple, fast and easy method, painless and risk-free, able to provide useful functional and structural information in ALS pt. Furthermore, diaphragm US may allow to point out concomitant respiratory failure. Moreover is desirable that US become an indispensable tool of the diagnostic armamentarium of the neuromuscular physician in pt. with ALS.

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