Isokinetic strength and degeneration of lower extremity muscles in patients with myotonic dystrophy; an MRI study

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Isokinetic strength and degeneration of lower extremity muscles in patients with myotonic dystrophy; an MRI study. / Steenkjaer, C. H.; Mencagli, R. A.; Vaeggemose, M.; Andersen, H.

I: Neuromuscular Disorders, Bind 31, Nr. 3, 03.2021, s. 198-211.

Publikation: Bidrag til tidsskrift/Konferencebidrag i tidsskrift /Bidrag til avisTidsskriftartikelForskningpeer review

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Steenkjaer, C. H. ; Mencagli, R. A. ; Vaeggemose, M. ; Andersen, H. / Isokinetic strength and degeneration of lower extremity muscles in patients with myotonic dystrophy; an MRI study. I: Neuromuscular Disorders. 2021 ; Bind 31, Nr. 3. s. 198-211.

Bibtex

@article{3ab257c51eeb4cd598944b9a7fe11be7,
title = "Isokinetic strength and degeneration of lower extremity muscles in patients with myotonic dystrophy; an MRI study",
abstract = "Our aim was to determine isokinetic strength and degeneration of lower extremity muscles in patients with Myotonic Dystrophy (DM1). In 19 patients with DM1 and 19 matched controls, strength measured by isokinetic dynamometry was expressed as percentage of expected strength (ePct), adjusted for age, height, weight and gender. MRI of the hip, thigh and calf muscles were obtained. Fat fraction (FF), mean contractile cross-sectional area (cCSA) and specific strength (Nm/cm2) were calculated. Patients{\textquoteright} ankle plantar flexors, knee flexors and extensors had higher FF (Δ: 0.08 – 0.42) and lower cCSA (Δ: 3.2 –17.1 cm2) compared to controls (p ≤ 0.005). EPct (Δ: 19.5 – 41.6%) and specific strength (Δ: 0.27 – 0.96 Nm/cm2) were lower in the majority of patients muscle groups (p˂0.05). Close correlations were found for patients when relating ePct to; FF for plantar flexors (R2=0.742, p<0.001) and knee extensors (R2=0.732, p<0.001), cCSA for plantar flexors (R2=0.696, p<0.001) and knee extensors (R2=0.633, p<0.001), and specific strength for dorsal flexors (ρ=0.855, p = 0.008). In conclusion, patients had weaker lower extremity muscles with higher FF, lower cCSA and specific strength compared to controls. Muscle degeneration determined by quantitative MRI strongly correlated to strength supporting its feasibility to quantify muscle dysfunction in DM1.",
keywords = "Isokinetic muscle strength, Muscular dystrophy in lower extremities, Myotonic dystrophy, Quantitative magnetic resonance imaging, Specific strength",
author = "Steenkjaer, {C. H.} and Mencagli, {R. A.} and M. Vaeggemose and H. Andersen",
note = "Publisher Copyright: {\textcopyright} 2021 Elsevier B.V.",
year = "2021",
month = mar,
doi = "10.1016/j.nmd.2020.12.011",
language = "English",
volume = "31",
pages = "198--211",
journal = "Neuromuscular Disorders",
issn = "0960-8966",
publisher = "Elsevier Ltd",
number = "3",

}

RIS

TY - JOUR

T1 - Isokinetic strength and degeneration of lower extremity muscles in patients with myotonic dystrophy; an MRI study

AU - Steenkjaer, C. H.

AU - Mencagli, R. A.

AU - Vaeggemose, M.

AU - Andersen, H.

N1 - Publisher Copyright: © 2021 Elsevier B.V.

PY - 2021/3

Y1 - 2021/3

N2 - Our aim was to determine isokinetic strength and degeneration of lower extremity muscles in patients with Myotonic Dystrophy (DM1). In 19 patients with DM1 and 19 matched controls, strength measured by isokinetic dynamometry was expressed as percentage of expected strength (ePct), adjusted for age, height, weight and gender. MRI of the hip, thigh and calf muscles were obtained. Fat fraction (FF), mean contractile cross-sectional area (cCSA) and specific strength (Nm/cm2) were calculated. Patients’ ankle plantar flexors, knee flexors and extensors had higher FF (Δ: 0.08 – 0.42) and lower cCSA (Δ: 3.2 –17.1 cm2) compared to controls (p ≤ 0.005). EPct (Δ: 19.5 – 41.6%) and specific strength (Δ: 0.27 – 0.96 Nm/cm2) were lower in the majority of patients muscle groups (p˂0.05). Close correlations were found for patients when relating ePct to; FF for plantar flexors (R2=0.742, p<0.001) and knee extensors (R2=0.732, p<0.001), cCSA for plantar flexors (R2=0.696, p<0.001) and knee extensors (R2=0.633, p<0.001), and specific strength for dorsal flexors (ρ=0.855, p = 0.008). In conclusion, patients had weaker lower extremity muscles with higher FF, lower cCSA and specific strength compared to controls. Muscle degeneration determined by quantitative MRI strongly correlated to strength supporting its feasibility to quantify muscle dysfunction in DM1.

AB - Our aim was to determine isokinetic strength and degeneration of lower extremity muscles in patients with Myotonic Dystrophy (DM1). In 19 patients with DM1 and 19 matched controls, strength measured by isokinetic dynamometry was expressed as percentage of expected strength (ePct), adjusted for age, height, weight and gender. MRI of the hip, thigh and calf muscles were obtained. Fat fraction (FF), mean contractile cross-sectional area (cCSA) and specific strength (Nm/cm2) were calculated. Patients’ ankle plantar flexors, knee flexors and extensors had higher FF (Δ: 0.08 – 0.42) and lower cCSA (Δ: 3.2 –17.1 cm2) compared to controls (p ≤ 0.005). EPct (Δ: 19.5 – 41.6%) and specific strength (Δ: 0.27 – 0.96 Nm/cm2) were lower in the majority of patients muscle groups (p˂0.05). Close correlations were found for patients when relating ePct to; FF for plantar flexors (R2=0.742, p<0.001) and knee extensors (R2=0.732, p<0.001), cCSA for plantar flexors (R2=0.696, p<0.001) and knee extensors (R2=0.633, p<0.001), and specific strength for dorsal flexors (ρ=0.855, p = 0.008). In conclusion, patients had weaker lower extremity muscles with higher FF, lower cCSA and specific strength compared to controls. Muscle degeneration determined by quantitative MRI strongly correlated to strength supporting its feasibility to quantify muscle dysfunction in DM1.

KW - Isokinetic muscle strength

KW - Muscular dystrophy in lower extremities

KW - Myotonic dystrophy

KW - Quantitative magnetic resonance imaging

KW - Specific strength

UR - http://www.scopus.com/inward/record.url?scp=85100447978&partnerID=8YFLogxK

U2 - 10.1016/j.nmd.2020.12.011

DO - 10.1016/j.nmd.2020.12.011

M3 - Journal article

C2 - 33568272

AN - SCOPUS:85100447978

VL - 31

SP - 198

EP - 211

JO - Neuromuscular Disorders

JF - Neuromuscular Disorders

SN - 0960-8966

IS - 3

ER -