• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.16 no.2
• /
• pp.93-101
• /
• 2016

Purpose: McArdle disease, glycogen storage disease type V (GSD V), is one of the most common adolescent-onset glycogen storage diseases. It is caused by recessive mutations in PYGM encoding myophosphorylase, which is critical to glycogen metabolism. Since only a few korean patients have been reported, we will observe the clinical and genetic features of three korean patients with McArdle disease. Methods: We retrospectively reviewed the medical records of three patients with genetically confirmed McArdle disease, including the results of forearm ischemic exercise test, electromyogram, nerve conduction velocity, muscle biopsy, and PYGM analysis in peripheral leukocytes. Results: All three cases were males and their age of symptom onset was 12, 5, 14 years old, respectively. A high basal level of serum creatine kinase was noted in all three patients. They experienced the recurrent episodes of rhabdomyolysis, but second wind phenomenon was not definite. In muscle biopsy, subsarcolemmal space vacuoles including periodic acid schiff stained materials were found in two patients, while no evidence of glycogen storage disease was found in the other. A total of five different mutations, $p.Arg50^*$, p.Trp798Arg, $p.Arg50^*$, p.Glu779del, $p.Asp511Thrfs^*28$ and p.Phe710del, were found in three patients. Avoidance of isometric exercise, aerobic exercise and glucose intake before each exercise were recommended for all patients. Conclusion: The three Korean patients with McArdle disease showed the typical manifestations of the condition. The most mutations were private. Therefore, identification of more cases with long-term follow-up will be required to understand the clinical and genetic features of this disease among Korean population.

• Physical Therapy Korea
• /
• v.10 no.3
• /
• pp.29-40
• /
• 2003

The purpose of this study was to compare the muscle activity during a push-up on a suspension sling and a fixed support at the same level. Tests were performed on 15 male subjects. Electromyography using a surface EMG recorded the activity of the triceps, pectoralis major, and internal and external oblique muscles during each push-up. EMG activity was recorded at 0, 45, and 90 degrees of elbow flexion in the push-up position on a suspension sling or a fixed support at the same height above the floor (30 cm). The testing order was selected randomly. The subjects were asked to maintain the push-up position with straight knees, hips, and trunk for 5 seconds at each elbow angle. The mean root mean square (RMS) of EMG activity was calculated. EMG activity was normalized using the maximum voluntary isometric contractιn elicited using a manual muscle testing technique. Two-factor repeated measures analysis of variance (ANOVA) was used to compare the average RMS value of EMG activity for each condition. The EMG activity for the pectoralis major, and internal and external oblique muscles during a push-up on a sling was significantly higher than on a fixed support at all angles of elbow flexion (p<.01). There were significant differences in the EMG activity of the pectoralis major and triceps brachii muscles at difference angles of elbow flexion (p<.05). The pectoralis major muscle had the highest EMG activity at 90 degrees of elbow flexion on both the sling and fixed support. The triceps brachii muscle had the highest EMG activity at 45 degrees of elbow flexion on both the sling and fixed support. The internal and external oblique muscles had the highest EMG activity at 0 degrees of elbow flexion, although the difference with angle of flexion was not significant. These results suggest that to improve proximal and trunk stability and muscle strength, push-ups are more useful when performed on a suspension sling than On a fixed support.

• Physical Therapy Rehabilitation Science
• /
• v.8 no.1
• /
• pp.45-51
• /
• 2019

Objective: The purpose of this study was to investigate the muscle activity ratio of the lower limb according to changes in straight leg raise (SLR) test angles on hamstring muscle shortening during squat exercises. Design: Randomized controlled trial. Methods: The subjects were 14 healthy adults who were informed of and agreed to the method and purpose of the study. The participants were classified into SLR groups according to two angles (over $80^{\circ}$ or under $80^{\circ}$) assessed using the SLR tests. After training and practicing the wall squat posture to be applied to the experiment, electromyography (EMG) was used to measure changes in muscle activity during the performance of a wall squat. After stretching, a sequence of pre-stretch tests were performed again, and the active and passive SLR tests were also reconducted; thereafter, a wall squat was performed again by attaching EMG electrodes. The EMG results before and after stretching were compared. Results: The muscle activity of the vastus lateralis oblique muscle increased in both groups. The muscle activity of the vastus medialis oblique muscle decreased in over both group. Rectus femorus activity increased in the under 80-degree groups but decreased in the over 80-degree group. The muscle activity of the biceps femoris muscle decreased after stretching in the over 80-degree group and increased in the under 80-degree group, and the semitendinosus muscle activity after stretching was decreased. The quadriceps-to-hamstring muscle (Q:H) ratio before and after stretching between groups showed that the hamstring muscle ratio decreased after stretching in both groups. Conclusions: The results of this study showed that the Q:H ratio before and after stretching between groups was not significantly different.