Muscle Ultrasound Changes Correlate With Functional Impairment in Spinal Muscular Atrophy.

Autor:	Moreira AL; Departament of Neurology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. Electronic address: ana.lucila.moreira@hotmail.com., Mendonça RH; Departament of Neurology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil., Polido GJ; Departament of Neurology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil., Oliveira MCB; Departament of Neurology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil., Silva AMS; Departament of Neurology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil., Zanoteli E; Departament of Neurology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
Jazyk:	angličtina
Zdroj:	Ultrasound in medicine & biology [Ultrasound Med Biol] 2023 Jul; Vol. 49 (7), pp. 1569-1574. Date of Electronic Publication: 2023 Apr 08.
DOI:	10.1016/j.ultrasmedbio.2023.02.021
Abstrakt:	Objective: We investigated ultrasound patterns of muscle involvement in different types of spinal muscular atrophy (SMA) and their correlation with functional status to determine the pattern of muscle compromise in patients with SMA and the potential role of ultrasound to evaluate disease progression. Methods: We examined muscles (biceps brachii, rectus femoris, diaphragm, intercostals and thoracic multifidus) of 41 patients with SMA (types 1 to 4) and 46 healthy age- and sex-matched control individuals using B-mode ultrasound for gray-scale analysis (GSA), area (biceps brachii and rectus femoris) and diaphragm thickening ratio. Functional scales were applied to patients only. We analyzed ultrasound abnormalities in specific clinical subtypes and correlated findings with functional status. Results: Compared with controls, patients had reduced muscle area and increased mean GSA for all muscles (p < 0.001), with an established correlation between the increase in GSA and the severity of SMA for biceps brachii, rectus femoris and intercostals (p = 0.03, 0.01 and 0.004 respectively) when using the Hammersmith Functional Motor Scale Expanded. Diaphragm thickening ratio was normal in the majority of patients, and intercostal muscles had higher GSA than diaphragm in relation to the controls. Conclusion: Ultrasound is useful for quantifying muscular changes in SMA and correlates with functional status. Diaphragm thickening ratio can be normal even with severe compromise of respiratory muscles in quantitative analysis, and intercostal muscles were more affected than diaphragm. Competing Interests: Conflict of interest A.L.M. has received financial compensation from General Electric (GE Healthcare) for course teaching. R.H.M. and E.Z. have received financial compensation from Biogen for congress participation and for talks. R.H.M. and E.Z. participate as investigators in clinical trials sponsored by Roche. All other authors declare no competing interests. (Copyright © 2023 World Federation for Ultrasound in Medicine & Biology. Published by Elsevier Inc. All rights reserved.)
Databáze:	MEDLINE
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