Contractile properties are disrupted in Becker muscular dystrophy, but not in limb girdle type 2I

Nicoline Løkken; Gitte Hedermann; Carsten Thomsen; John Vissing

doi:10.1002/ana.24743

Contractile properties are disrupted in Becker muscular dystrophy, but not in limb girdle type 2I

Nicoline Løkken, Gitte Hedermann, Carsten Thomsen, John Vissing

Institut for Klinisk Medicin

27 Citationer (Scopus)

Abstract

We investigated whether a linear relationship between muscle strength and cross-sectional area (CSA) is preserved in calf muscles of patients with Becker muscular dystrophy (BMD, n = 14) and limb-girdle type 2I muscular dystrophy (LGMD2I, n = 11), before and after correcting for muscle fat infiltration. The Dixon magnetic resonance imaging technique was used to quantify fat and calculate a fat-free contractile CSA. Strength was assessed by dynamometry. Muscle strength/CSA relationships were significantly lower in patients versus controls. The strength/contractile-CSA relationship was still severely lowered in BMD, but was almost normalized in LGMD2I. Our findings suggest close to intact contractile properties in LGMD2I, which are severely disrupted in BMD. Ann Neurol 2016;80:466-471.

Originalsprog	Engelsk
Tidsskrift	Annals of Neurology
Vol/bind	80
Udgave nummer	3
Sider (fra-til)	466-471
Antal sider	6
ISSN	0364-5134
DOI	https://doi.org/10.1002/ana.24743
Status	Udgivet - 1 sep. 2016

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10.1002/ana.24743

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title = "Contractile properties are disrupted in Becker muscular dystrophy, but not in limb girdle type 2I",

abstract = "We investigated whether a linear relationship between muscle strength and cross-sectional area (CSA) is preserved in calf muscles of patients with Becker muscular dystrophy (BMD, n = 14) and limb-girdle type 2I muscular dystrophy (LGMD2I, n = 11), before and after correcting for muscle fat infiltration. The Dixon magnetic resonance imaging technique was used to quantify fat and calculate a fat-free contractile CSA. Strength was assessed by dynamometry. Muscle strength/CSA relationships were significantly lower in patients versus controls. The strength/contractile-CSA relationship was still severely lowered in BMD, but was almost normalized in LGMD2I. Our findings suggest close to intact contractile properties in LGMD2I, which are severely disrupted in BMD. Ann Neurol 2016;80:466-471.",

author = "Nicoline L{\o}kken and Gitte Hedermann and Carsten Thomsen and John Vissing",

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T1 - Contractile properties are disrupted in Becker muscular dystrophy, but not in limb girdle type 2I

AU - Løkken, Nicoline

AU - Hedermann, Gitte

AU - Thomsen, Carsten

AU - Vissing, John

PY - 2016/9/1

Y1 - 2016/9/1

N2 - We investigated whether a linear relationship between muscle strength and cross-sectional area (CSA) is preserved in calf muscles of patients with Becker muscular dystrophy (BMD, n = 14) and limb-girdle type 2I muscular dystrophy (LGMD2I, n = 11), before and after correcting for muscle fat infiltration. The Dixon magnetic resonance imaging technique was used to quantify fat and calculate a fat-free contractile CSA. Strength was assessed by dynamometry. Muscle strength/CSA relationships were significantly lower in patients versus controls. The strength/contractile-CSA relationship was still severely lowered in BMD, but was almost normalized in LGMD2I. Our findings suggest close to intact contractile properties in LGMD2I, which are severely disrupted in BMD. Ann Neurol 2016;80:466-471.

AB - We investigated whether a linear relationship between muscle strength and cross-sectional area (CSA) is preserved in calf muscles of patients with Becker muscular dystrophy (BMD, n = 14) and limb-girdle type 2I muscular dystrophy (LGMD2I, n = 11), before and after correcting for muscle fat infiltration. The Dixon magnetic resonance imaging technique was used to quantify fat and calculate a fat-free contractile CSA. Strength was assessed by dynamometry. Muscle strength/CSA relationships were significantly lower in patients versus controls. The strength/contractile-CSA relationship was still severely lowered in BMD, but was almost normalized in LGMD2I. Our findings suggest close to intact contractile properties in LGMD2I, which are severely disrupted in BMD. Ann Neurol 2016;80:466-471.

U2 - 10.1002/ana.24743

DO - 10.1002/ana.24743

M3 - Journal article

C2 - 27463532

SN - 0364-5134

VL - 80

SP - 466

EP - 471

JO - Annals of Neurology

JF - Annals of Neurology

IS - 3

ER -

Contractile properties are disrupted in Becker muscular dystrophy, but not in limb girdle type 2I

Abstract

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