Accelerated Muscular Development Programs
Currently there are no specific pharmacological treatments for respiratory chain Therapy disorders. Aerobic training improves exercise tolerance, cardiovascular function, and muscle metabolism in some patients. Strength training may help in some patients. A variety of mitochondrial enzyme supplements have been tried with variable success. These include coenzyme Q, creatine, carnitine, thiamine, nicotinamide, riboflavin, succinate, and menadione. Until the specific enzyme defects within a particular Mt myopathy are better defined, enzyme supplements will have a limited role in treatment of this disorder.
A randomized control trial (RCT) (n 68) investigating the efficacy of PT in patients with PD revealed significant improvements at the disability level regarding quality of life as it relates to physical mobility. The most robust findings occurred at the functional level with respect to walking speed and ADL (6). No significant improvements were found at the impairment level with regard to neurological signs. Several other investigations support these findings. Formisano et al. investigated the benefits of a PT program in comparison to a control group and found greater improvements regarding ADL and a 10-m walking test in the group who had received PT (7). Patti and coauthors investigated the effects of a multidisciplinary rehabilitation program in an RCT on individuals with idiopathic PD and found significant increases in walking speed and stride length compared to a nonintervention control condition (8). In a nonrandomized study, Szekely and coauthors reported significant...
BMD often causes calf pain, cramps, and myalgias. Weakness is present in Clinical syndrome approximately 20 of affected patients. Patients may have no symptoms. In general the severity and onset age correlate with muscle dystrophin levels. As with DMD, affected subjects may have calf muscle hypertrophy and contractures in the lower extremities. Patients with BMD often have a severe cardiomyopathy as part of the muscle weakness syndrome, or may have an isolated dilated cardiomyopathy. In general the average IQ of affected children is reduced compared to the general population and may be a major presenting symptom in BMD. Some patients may present with an atypical neuromuscular disorder mimicking SMA, a focal myopathy, or a limb girdle muscular dystrophy.
The p21 protein, which is an important regulator of cell cycle progression and muscle differentiation, was identified as a downstream target for the muscle differentiation defects induced by expanded CTG repeats (Timchenko et al. 2001b). CUG-BP1 was shown to enhance p21 translation by binding to GCN repeats in the 5' UTR of p21 mRNA (Timchenko et al. 2001b). Accumulation of CUG-BP1 in the nuclei of DM cells resulted in reduced levels of p21 translation leading to reduced differentiation of these cells. These results suggest that reduced MyoD and p21 are directly involved in muscle development abnormalities seen in CDM or defects in muscle regeneration seen in DM1. The molecular mechanisms for muscle weakness and wasting in DM2 patients are not clear since there is no involvement of DMPK 3'UTR in DM2. To date, none of the transgenic or knockout mouse models have reproduced a clear progressive muscular dystrophy phenotype as observed in individuals with DM1 (Berul et al. 2000 Kanadia et...
A scenario for exclusively somitic muscle development emerges from the classical studies described above. However, De Angelis and colleagues suggested that satellite cells have nonsomitic origins (28). These authors demonstrated that clonal myogenic MPCs are readily isolated from explants of embryonic aorta. In vitro, these clones exhibit morphology similar to that of satellite cells derived from adult skeletal muscle and coexpress a number of myogenic and endothelial markers. MPCs can also be isolated from limbs of late-stage c-Met- - and Pax3- - mutant embryos. Those mice are severely deficient for determination and migration of myogenic precursors, resulting in complete absence of muscle cells in embryo limbs. However, the vascular system of those embryos is formed normally, suggesting that the MPCs derived from these tissues have endothelial origins. In vivo, aorta-derived MPCs can participate in skeletal muscle regeneration and give rise to many myogenic cells when introduced...
In this defect, which affects 10 of males, the rectum opens at the bladder neck (Fig. 20.9). The patient has a poor prognosis because the levator ani muscle, muscle complex, and external sphincter are frequently poorly developed. Consistent with the caudal regression, the sacrum and entire pelvis is often deformed and underdeveloped. The perineum is often flat, with evidence of poor muscle development.
Earlier studies to determine the effect of RF energy on tissue alteration in the GEJ were performed on porcine and canine models. Histopathic assessment was inconclusive in the pig study whereas the dog model demonstrated marked muscle hypertrophy and fibrosis (Utley et al, 2000). Segments of tissue from the gastric cardia showed a significant increase in thickness after RF energy delivery. However, an endoscopic ultrasonogram (EUS) study in a small group of patients performed prior to and 6 months following RF treatment showed no significant alteration in the wall thickness in the GEJ region (Dibaise et al, 2002).
Deficiency of the third, fourth, and fifth sacral vertebrae is usually accompanied by variable abnormal nerve and muscle development, and most patients are incontinent. 4. The innervation and muscle development accompanying hemisacral defects is unpredictable, but if only one or two vertebrae show the hemisacral defect, then innervation and muscle development may be adequate, and can be determined on cross-sectional imaging.
Some bodybuilders and other power athletes take powdered or liquid amino acid mixtures ( predigested protein ) in the belief that simple amino acids are absorbed more easily and rapidly or that they somehow contribute more to muscle building. Such beliefs are unfounded. Dietary proteins are rapidly digested and absorbed, and there is no added benefit to taking predigested protein. Amino acid
The application of DR to extending longevity in humans at present is out of the question. It is far from certain that it would succeed and, if it did, that exaggerated morbidity would not occur. Furthermore, there is considerable uncertainty about the optimum body composition associated with health and longevity. For example, there are unanswered questions about the optimum proportions of lean muscle and fat masses and the rates of change of those components at different physiological and chronological ages (23-25).
Direct gene therapy is another approach to deliver genes to skeletal muscle. Muscle cells have been successfully transduced in vitro and in vivo using the intramuscular inoculation of replicative-deficient adenovirus, retrovirus, and HSV carrying the -galactosidase (LacZ) reporter gene however, a major limitation of using these viral vectors alone is the differential transducibility observed throughout skeletal muscle development (22,29).
Two common clinical situations are the differentiation between ET and PD and ET and dystonic tremor. Some of the features of PD that generally do not occur in patients with ET are hemibody involvement (e.g., ipsilateral arm and leg) and bradykinesia and rigidity. The postural tremor of ET also tends to involve wrist flexion and extension whereas in PD, wrist rotation (esp., internal) often occurs. Features of dystonic tremor that do not occur in patients with pure ET are dystonic movements or postures, muscle hypertrophy, complaints of pain or pulling in the affected body region, a null point, and a nonrhythmic quality to the tremor.
They often have calf muscle hypertrophy, muscle fibrosis, contractures in the lower extremities, and scoliosis of the spine. In general the average IQ of affected children is reduced compared to the general population to approximately 85. Some patients (20 ) may have more severe cognitive impairment. Other features include a retinal abnormality with night blindness, and a cardiomyopathy that develops by the mid-teens. In DMD, cardiac conduction defects, resting tachycardia, and cardiomyopathy are frequently encountered. Mitral valve prolapse and pulmonary hypertension may also be seen. Death normally occurs by the late teens to early twenties from respiratory or cardiac failure.
There is no clear evidence that exercise will prolong your life, but there is little doubt that it improves the quality of life in old age. It maintains endurance, strength, and joint mobility while it reduces the incidence and severity of hypertension, osteoporosis, obesity, and diabetes melli-tus. This is especially true if you begin a program of regular physical exercise early in life and make a lasting habit of it. If you stop exercising regularly after middle age, the body rapidly becomes deconditioned, although appreciable reconditioning can be achieved even when an exercise program is begun late in life. A person in his or her 90s can increase muscle strength two- or threefold in 6 months with as little as 40 minutes of isometric exercise a week. The improvement results from a combination of muscle hypertrophy and neural efficiency.
Expanded CTG repeats were shown to alter chromatin structure and have regional effects on gene expression (Otten and Tapscott 1995 Wang et al. 1994). The CTG expansion in the DMPK 3'UTR are located immediately upstream of the SIX5 promoter region and were shown to lower SIX5 expression (Gennarelli et al. 1999 Inukai et al. 2000 Klesert et al. 1997 Thornton et al. 1997). Six5 is a transcription factor required for eye development in Drosophila, and the mouse homologue is implicated in distal limb muscle development (Harris et al. 2000). Six5 knockout mice develop ocular cataracts and infertility resembling some features of DM1 (Klesert et al. 2000 Sarkar et al. 2000). Cardiac conduction abnormalities were also noted in Six5 knockout mice (Wakimoto et al. 2002). However, the most common symptoms of DM1 such as muscle weakness, wasting, and myotonia were not reproduced in Six5 knockout mice (Klesert et al. 2000 Sarkar et al. 2000). The identification of a second locus causing DM2 reduced...
Exercise alone is, unfortunately, not a terribly effective method for losing weight. It is difficult for the untrained person to do enough of it, and most if not all of the expended energy is compensated by increased caloric intake. Exercise is, however, a superb way to maintain a lower weight after weight loss, enabling a person to eat somewhat more than a nonexerciser and maintain the same given weight. Regular aerobic exercise and strength training will also improve cardiovascular fitness, trim inches, and promote growth of metabolically more active muscle tissue.
- Chromosome 15q15-linked LGMD (Calpain3) There is considerable variation in the severity of this disease initially described among Amish families and families from La Reunion. Onset is usually before age 10 years, with a wide range of time before loss of ambulation and death. Shoulder and pelvic girdle muscles are affected, facial muscles are spared, calf muscle hypertrophy is common, and the degree of clinical heterogeneity makes it difficult to distinguish from other forms of LGMD.
Myotonia is usually mild, approximately 50 may have percussion myotonia. The myotonia (Fig. 33) is associated with fluctuations, and may be worsened by cold, hunger, fatigue and emotional upset. Muscle hypertrophy is seen in many patients (Fig. 34), and occasionally patients may complain of myalgias. Patients may report a warm-up phenomenon, in which the myotonia decreases after repeated activity. Muscle strength is usually normal. Mild myotonia occurring late in life, with less muscle hypertrophy.
Muscle hypertrophy is much rarer than atrophy and may be generalized, as in myotonia congenita, or localized, as in the pseudohypertrophy of the calf muscles in some types of muscular dystrophy and glycogen storage diseases. Focal hypertrophy is even rarer and may occur in muscle tumors, focal myositis, amyloidosis, or infection. Also, ruptured muscles may mimic a local hypertrophy during contraction.
Proteins are our chief dietary source of nitrogen. Nitrogen balance is a state in which the rate of nitrogen ingestion equals the rate of excretion (chiefly as nitrogenous wastes). Growing children exhibit a state of positive nitrogen balance because they ingest more than they excrete, thus retaining protein for tissue growth. Pregnant women and athletes in resistance training also show positive nitrogen balance. When excretion exceeds ingestion, a person is in a state of negative nitrogen balance. This indicates that body proteins are being broken down and used as fuel. Proteins of the muscles and liver are more easily broken down than others thus negative nitrogen balance tends to be associated with muscle atrophy. Negative nitrogen balance may occur if carbohydrate and fat intake are insufficient to meet the need for energy. Carbohydrates and fats are said to have a protein-sparing effect because they prevent protein catabolism when present in sufficient amounts to meet energy...
In Andersen's syndrome there is a potassium sensitive periodic paralysis with cardiac dysrhythmias and dysmorphic features. Acetazolamide-responsive myotonia congenita is an autosomal dominant sodium channel defect in which there is muscle hypertrophy, and paradoxical myotonia. The disorder is associated with muscle pain and stiffness, is aggravated by potassium, and improved by acetazolamide. It is not associated with weakness. Myotonia permanens is a sodium channel defect associated with severe continuous myotonia that may interfere with breathing. There is usually marked muscle hypertrophy in this disorder. Myotonia fluctuans is an autosomal dominant defect of the SCN4A subunit of the muscle sodium channel. In this disorder there is mild myotonia that varies in severity. Stiffness develops during rest approximately 30 minutes after exercise and may last for up to 60 minutes. Stiffness is worsened by potassium, or depolarizing agents. The stiffness may interfere with respiration if...
Pax, Six, and Eya family members, together with the transcription co-factor Dach, act in an intricate cross-regulatory network in both eye and muscle development (see Reference 21). Dach family members are expressed in part of the preplacodal domain and in various placodes.22-25 It is intriguing to speculate that expression of Six, Eya, and perhaps also Dach genes within the preplacodal domain may provide a pan-placodal regulatory module. Different Pax family members are induced in different locations within the preplacodal domain by specific placode-inducing signals from neighboring tissues (for a comprehensive review of known placode-inducing tissues and signals, see Reference 2). The different Pax proteins might then interact with Six, Eya and Dach members to specify the identities of individual placodes. There is currently no real evidence for this model, but it is eminently testable.
We use a two-channel electromyography electrode with one sensor placed in the anal canal and the other surface electrode placed on the gluteus muscles for strength and endurance training. Patients practice maximum squeeze and muscle isolation in the upright position, which facilitates coordination training and enhances feedback. When patients demonstrate adequate understanding and ability to perform exercises, they are assigned to continue strength training at home, using gradually increasing daily exercises. We have found that patients relearn sensitivity to smaller filling volumes and sensation improves without formalized training.
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