Nemaline myopathy is a congenital neuromuscular disorder, which primarily affects the thin filaments. Clinically the most important feature is muscle weakness; however, this weakness is poorly understood. The present investigation aimed to determine the torque angle relationship of the knee extensor muscles during in vivo muscle contractions in a family with a novel phenotype of nemaline myopathy. The results of this study show that quadriceps weakness occurs predominantly at higher knee flexion angles, but relatively normal strength was found at angles closer to full knee extension. When the relative torque angle relationships were considered, torque loss at smaller than optimum knee flexion angle was greater in the patients compared with the controls. In addition, the optimum angle for maximal quadriceps torque production was shifted towards smaller knee flexion angles in the patients. This suggests that a weakness specifically at higher knee flexion angles probably occurs as a result of adaptations consequently to the disease. Furthermore, it is important to assess muscle function at different joint positions to allow adequate interpretation of muscle weakness.
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November 2003
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Research Article|
November 01 2003
Quadriceps weakness in a family with nemaline myopathy: influence of knee angle
Karin GERRITS;
*Institute for Fundamental and Clinical Human Movement Sciences, Vrije University Amsterdam, 1081 BT Amsterdam, The Netherlands
Correspondence: Karin Gerrits (e-mail [email protected]).
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Inge GOMMANS;
Inge GOMMANS
*Institute for Fundamental and Clinical Human Movement Sciences, Vrije University Amsterdam, 1081 BT Amsterdam, The Netherlands
†Neuromuscular Centre Nijmegen, Institute of Neurology, University Medical Centre Nijmegen, 6500 HB Nijmegen, The Netherlands
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Baziel VAN ENGELEN;
Baziel VAN ENGELEN
†Neuromuscular Centre Nijmegen, Institute of Neurology, University Medical Centre Nijmegen, 6500 HB Nijmegen, The Netherlands
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Arnold DE HAAN
Arnold DE HAAN
*Institute for Fundamental and Clinical Human Movement Sciences, Vrije University Amsterdam, 1081 BT Amsterdam, The Netherlands
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Publisher: Portland Press Ltd
Received:
April 30 2003
Revision Received:
June 27 2003
Accepted:
July 09 2003
Accepted Manuscript online:
July 09 2003
Online ISSN: 1470-8736
Print ISSN: 0143-5221
© 2003 The Biochemical Society
2003
Clin Sci (Lond) (2003) 105 (5): 585–589.
Article history
Received:
April 30 2003
Revision Received:
June 27 2003
Accepted:
July 09 2003
Accepted Manuscript online:
July 09 2003
Citation
Karin GERRITS, Inge GOMMANS, Baziel VAN ENGELEN, Arnold DE HAAN; Quadriceps weakness in a family with nemaline myopathy: influence of knee angle. Clin Sci (Lond) 1 November 2003; 105 (5): 585–589. doi: https://doi.org/10.1042/CS20030159
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