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G.P.19.04 Muscle strength and endurance: A successful key for injury prevention
Abstracts / Neuromuscular Disorders 17 (2007) 764–900 Intervertebral disc disease and instabilities of the vertebral column result in painful conditions with or without neurological deficits. After informed consent paravertebral muscle specimens were taken during surgery from 37 patients with disc protrusion and 26 patients with spondylolisthesis. Although myopathy related symptoms were absent, all biopsies showed alterations ranging from mild to severe. Neurogenic atrophy was present in almost half of the patients in both groups. Ragged red fibers were detected in 8 cases (21%) of the disc protrusion group and in 21 cases (80%) of the spondylolisthesis group. Intrasarcoplasmatic lipid and glycogen accumulation as indicators of an impaired mitochondrial function was frequently observed and appeared associated with mitochondrial accumulation. At the ultrastructural level the numerical increase of mitochondria was confirmed. They showed different configurational abnormalities as circular cristae and paracristalline inclusions. Another frequent finding was intrasarcoplasmatic lipofuscin pigment accumulation which was noticed even in young patients. It can be assumed that a premature aging of the muscle may result from inappropriate chronic stress due to pain and/or contractions. This may induce mitochondrial pathology similar to the classic mitochondriopathies as well as mitochondrial alterations found in senescence. In contrast to mtDNA mutations which are responsible for the above mentioned conditions, the pathological findings in this series are supposed to be secondary to local distress. doi:10.1016/j.nmd.2007.06.443
G.P.19.04 Muscle strength and endurance: A successful key for injury prevention He´bert, L. 1,*; Nadeau, S. 2 1 National Defense, Canadian Forces Health Services Headquarters, Health Services Delivery, Ottawa, Canada; 2 Centre de recherche interdisciplinaire en re´adaptation, IRM, Montreal, Canada In modern armies, soldiers are exposed to very physically demanding tasks. To complete these tasks without injury, one must assume that, independently of their age and gender, muscle strength and endurance are sufficient to prevent them from working near their musculoskeletal (MSK) limits. The objective of this study was to quantify trunk and lower limb muscle strength and endurance profiles in Canadian Forces (CF) soldiers. Forty CF soldiers (mean age; 34.8 ± 7.2) were submitted to a dynamometric testing to obtain the maximal torque of ankle plantar (PF) and dorsiflexors (DF), knee flexors (KF) and extensors (KE), hip flexors (HF) and extensors (HE), and the trunk extensors (TE). The torque, angle and velocity measurements were recorded during concentric isokinetic (peripheral joints) and isometric (trunk) tests. Two endurance tests for the knee and trunk extensors were also performed to assess the relative muscular endurance. The maximal peak torques was higher in men than women. In men, the muscle strength was always higher for the 18–30 group followed by the 31–40 and then the >40 group. In women, there was no such clear trend as, depending on the muscle group, the strength was higher in younger (HF 30°, HE 30°), higher in older (KF 60° and 30° ankle DF) or very similar between younger and older soldiers (KE 30°, ankle PF). The mean holding time of the TE on the Biodex was 64.5 s ± 37.7 for men (n = 30) and 89.1 s ± 41.1 for women (n = 10) (p = 0.088) while according to the Sorensen endurance test, the women had more trunk extension endurance (207.0 s ± 70.2) compared to the men (135.6 s ± 38.5) (p = 0.011). For the KE, the mean holding time was 85.9 s ± 104.7 for men (n = 30) and 61.9 s ± 19.2 for women (n = 10) (p = 0.254). The lower values of muscle strength and endurance observed in some CF soldiers compared to normative data of healthy subjects may explain why, during the completion of military routine tasks, some soldiers are using a high level of effort which put them at a higher risk of injury. doi:10.1016/j.nmd.2007.06.444
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G.P.19.05 Statin associated myopathy with normal creatine kinase levels: Case report from a Norwegian family Myreng, K.; Lindal, S. *; Lund, I.; Stensland, E.; Troseid, M. University Hospital of North-Norway (UNN), National Neuromuscular Centre, Tromsoe, Norway Hydroxymethylglutaryl-coenzyme A (HMG-CoA) reductase inhibitors are drugs used in treatment of hypercholesterolaemia and dyslipidaemia. Reports have shown that HMG-CoA reductase inhibitors can induce myopathy with severe clinical presentation with high serum creatine kinase and episodes of rhabdomyolysis. We will present four patients from the same family with unspecific clinical presentation with muscle weakness and myalgias. All four patients had muscle symptoms and normal creatin kinase levels. In two out of four patients (mother and son), pathological findings on EMG suggested myopathy. The light microscopic examination of the muscle biopsies did not reveal structural abnormalities. Ultrastructural examination of the same biopsies showed evidence of mitochondrial pathology with crista abnormalities, vacuoles and increased amounts of lipid droplets. A third patient (daugther) had slight myopathic findings on EMG and muscle biopsy, but not enough to be classified as pathological. In a fourth patient, there were no pathological findings. Creatine kinase levels were normal and symptoms diminished after discontinuation of drugs in all four patients. Our findings are consistent with other reports of statin-associated myopathy with normal creatine kinase levels. An inherited vulnerability, possibly a mitochondrial crista-membrane alteration might cause or aggravate symptoms in some patients. Key words: Mitochondrial pathology, myopathy, statins doi:10.1016/j.nmd.2007.06.445
G.P.19.06 Development of the MDA Monoclonal Antibody Resource for inherited neuromuscular diseases Le, T.; Nguyen Thi, M.; Morris, G. * RJAH Orthopaedic Hospital, Wolfson Centre for Neuromuscular Disease, Oswestry, United Kingdom Since the MDA Monoclonal Antibody Resource was set up over two years ago, it has distributed over 600 units of antibody to 136 laboratories in 15 countries. The available antibodies are listed on a new website at (www.rjah.nhs.uk/cind/MonoclonalAntibodyDatabase/tabid/303/Default.aspx) and include mAbs for research into Duchenne/Becker muscular dystrophy, Emery–Dreifuss MD, spinal muscular atrophy and myotonic dystrophy. Datasheets are now available on-line for nearly every antibody; they are continually up-dated and provide information on validated applications, epitope mapping, species-specificity and any known cross-reaction problems. They also show examples of western blot and immunolocalization applications and give references to published uses. Further recent developments include (a) trials of Zenon technology, which enables double-labelling with two or more mouse mAbs and avoids secondary antibody background staining when mouse mAbs are used on mouse tissue sections, and (b) further expansion of the dystrophin mAb library with mapped antibodies against exons 57–60. This project is supported by the Muscular Dystrophy Association (USA). doi:10.1016/j.nmd.2007.06.446
G.P.19.07 Gene and protein expression of muscle specific genes in children Petersson, S. 1,*; Schroeder, H. 2; Jensen, C. 3; Illum, N. 4 1 University of Southern Denmark, Department of Clinical Pathology, Odense, Denmark; 2 Odense University Hospital, Department of Clinical Pathology, Odense, Denmark; 3 University of Southern Denmark, Immu-
G.P.19.04 Muscle strength and endurance: A successful key for injury prevention He´bert, L. 1,*; Nadeau, S. 2 1 National Defense, Canadian Forces Health Services Headquarters, Health Services Delivery, Ottawa, Canada; 2 Centre de recherche interdisciplinaire en re´adaptation, IRM, Montreal, Canada In modern armies, soldiers are exposed to very physically demanding tasks. To complete these tasks without injury, one must assume that, independently of their age and gender, muscle strength and endurance are sufficient to prevent them from working near their musculoskeletal (MSK) limits. The objective of this study was to quantify trunk and lower limb muscle strength and endurance profiles in Canadian Forces (CF) soldiers. Forty CF soldiers (mean age; 34.8 ± 7.2) were submitted to a dynamometric testing to obtain the maximal torque of ankle plantar (PF) and dorsiflexors (DF), knee flexors (KF) and extensors (KE), hip flexors (HF) and extensors (HE), and the trunk extensors (TE). The torque, angle and velocity measurements were recorded during concentric isokinetic (peripheral joints) and isometric (trunk) tests. Two endurance tests for the knee and trunk extensors were also performed to assess the relative muscular endurance. The maximal peak torques was higher in men than women. In men, the muscle strength was always higher for the 18–30 group followed by the 31–40 and then the >40 group. In women, there was no such clear trend as, depending on the muscle group, the strength was higher in younger (HF 30°, HE 30°), higher in older (KF 60° and 30° ankle DF) or very similar between younger and older soldiers (KE 30°, ankle PF). The mean holding time of the TE on the Biodex was 64.5 s ± 37.7 for men (n = 30) and 89.1 s ± 41.1 for women (n = 10) (p = 0.088) while according to the Sorensen endurance test, the women had more trunk extension endurance (207.0 s ± 70.2) compared to the men (135.6 s ± 38.5) (p = 0.011). For the KE, the mean holding time was 85.9 s ± 104.7 for men (n = 30) and 61.9 s ± 19.2 for women (n = 10) (p = 0.254). The lower values of muscle strength and endurance observed in some CF soldiers compared to normative data of healthy subjects may explain why, during the completion of military routine tasks, some soldiers are using a high level of effort which put them at a higher risk of injury. doi:10.1016/j.nmd.2007.06.444
895
G.P.19.05 Statin associated myopathy with normal creatine kinase levels: Case report from a Norwegian family Myreng, K.; Lindal, S. *; Lund, I.; Stensland, E.; Troseid, M. University Hospital of North-Norway (UNN), National Neuromuscular Centre, Tromsoe, Norway Hydroxymethylglutaryl-coenzyme A (HMG-CoA) reductase inhibitors are drugs used in treatment of hypercholesterolaemia and dyslipidaemia. Reports have shown that HMG-CoA reductase inhibitors can induce myopathy with severe clinical presentation with high serum creatine kinase and episodes of rhabdomyolysis. We will present four patients from the same family with unspecific clinical presentation with muscle weakness and myalgias. All four patients had muscle symptoms and normal creatin kinase levels. In two out of four patients (mother and son), pathological findings on EMG suggested myopathy. The light microscopic examination of the muscle biopsies did not reveal structural abnormalities. Ultrastructural examination of the same biopsies showed evidence of mitochondrial pathology with crista abnormalities, vacuoles and increased amounts of lipid droplets. A third patient (daugther) had slight myopathic findings on EMG and muscle biopsy, but not enough to be classified as pathological. In a fourth patient, there were no pathological findings. Creatine kinase levels were normal and symptoms diminished after discontinuation of drugs in all four patients. Our findings are consistent with other reports of statin-associated myopathy with normal creatine kinase levels. An inherited vulnerability, possibly a mitochondrial crista-membrane alteration might cause or aggravate symptoms in some patients. Key words: Mitochondrial pathology, myopathy, statins doi:10.1016/j.nmd.2007.06.445
G.P.19.06 Development of the MDA Monoclonal Antibody Resource for inherited neuromuscular diseases Le, T.; Nguyen Thi, M.; Morris, G. * RJAH Orthopaedic Hospital, Wolfson Centre for Neuromuscular Disease, Oswestry, United Kingdom Since the MDA Monoclonal Antibody Resource was set up over two years ago, it has distributed over 600 units of antibody to 136 laboratories in 15 countries. The available antibodies are listed on a new website at (www.rjah.nhs.uk/cind/MonoclonalAntibodyDatabase/tabid/303/Default.aspx) and include mAbs for research into Duchenne/Becker muscular dystrophy, Emery–Dreifuss MD, spinal muscular atrophy and myotonic dystrophy. Datasheets are now available on-line for nearly every antibody; they are continually up-dated and provide information on validated applications, epitope mapping, species-specificity and any known cross-reaction problems. They also show examples of western blot and immunolocalization applications and give references to published uses. Further recent developments include (a) trials of Zenon technology, which enables double-labelling with two or more mouse mAbs and avoids secondary antibody background staining when mouse mAbs are used on mouse tissue sections, and (b) further expansion of the dystrophin mAb library with mapped antibodies against exons 57–60. This project is supported by the Muscular Dystrophy Association (USA). doi:10.1016/j.nmd.2007.06.446
G.P.19.07 Gene and protein expression of muscle specific genes in children Petersson, S. 1,*; Schroeder, H. 2; Jensen, C. 3; Illum, N. 4 1 University of Southern Denmark, Department of Clinical Pathology, Odense, Denmark; 2 Odense University Hospital, Department of Clinical Pathology, Odense, Denmark; 3 University of Southern Denmark, Immu-