S54. Neuromuscular ultrasound as diagnostic tool and marker of disease severity in amyotrophic lateral sclerosis

Abstract

Introduction Several methods have been proposed to diagnose and quantify disease severity in patients (pt.) with Amyotrophic Lateral Sclerosis (ALS). Traditionally neurophysiological tests have been employed. The development of neuromuscular ultrasound (US) have provided a further useful tool to supplement clinical and neurophysiological assessment of ALS. Our aim was to evaluate sonographic changes of muscles and nerve trunks in an attempt to define diagnosis and assess severity of the disease. Methods 35 consecutive ALS pt. (20 males e 15 females, mean age: 64.6 ± 10.5 yrs) were enrolled; in four cases the diagnosis was not previously established. All investigations were performed using an Esaote MyLabGamma or a Telemed Echo-wave II device in conventional B-Mode. We evaluated the following muscles: tongue, sternocleidomastoid, diaphragm, biceps brachii/brachialis, forearm flexors, rectus femoris and anterior tibial; among the nerve, brachial plexus, vagus, median, ulnar and sural. Results We found in all cases increased echogenicity with decreased muscle thickness and fasciculations; these findings allowed us to diagnose an ALS in the 4 cases not previously identified and later confirmed. Mean diaphragm rest thickness was reduced; changes in thickness during inspiration and expiration were also reduced and loss in the most severe cases (3 pt.). Brachial plexus, Median and Ulnar nerve cross-sectional area (CSA) resulted smaller in ALS; vagus and sural nerve resulted spared. ALS disease severity, measured using Functional Rating Scale (ALSFRS), correlated with quantitative ultrasound data. Conclusion Neuromuscular ultrasound resulted a simple, fast and easy method, painless and risk-free, able to provide useful functional and structural informations 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.