Diet-induced undernutrition causes deleterious changes in the structure and function of the diaphragm muscle. Diseases associated with somatic washing cause atrophy of the respiratory muscles. In cachectic subjects, the diaphragm muscle mass and thickness are reduced in proportion to the reduction in body weight. In addition, respiratory muscle strength and endurance are reduced more dramatical...

Case Report A right-handed 12-year-old boy had suffered from pathologically proven EDMD for 7 years and required a wheelchair in daily life due to limb weakness. He had been given warfarin for one year because of chronic atrial fibrillation and suddenly developed leftsided motor weakness and was admitted to our hospital. On admission, he was alert and had a conjugate gaze deviation to the right...

Myocardial involvement in progressive muscular dystrophy of the Duchenne type was evaluated in 19 patients using thallium-201 myocardial perfusion imaging. A qualitative analysis was performed from five projection images by three experienced physicians. Distinct perfusion defects were shown in 13 patients, especially in the LV posterolateral or posterior wall (11 patients). There was no signifi...

Tissue engineering is a pioneering field with huge advances in recent times. These advances are not only in the understanding of how cells can be manipulated but also in potential clinical applications. Thus, tissue engineering, when applied to skeletal muscle cells, is an area of huge prospective benefit to patients with muscle disease/damage. This could include damage to muscle from trauma an...

We present a series of 4 patients in whom a ventricular septal defect (VSD) was closed with an Amplatzer muscular VSD device during cardiac catheterization. In one patient with type I truncus arteriosus and subarterial VSD, closure of a wide apical defect was done to allow further surgical correction while avoiding left ventricular ventriculotomy. The second patient had congenitally corrected t...

Duchenne muscular dystrophy is a lethal X-linked muscle disease resulting from a defect in the muscle membrane protein dystrophin. The absence of dystrophin leads to muscle membrane fragility, muscle death (necrosis) and eventual replacement of skeletal muscle by fat and fibrous connective tissue. Extensive muscle wasting and respiratory failure results in premature death often by the early 20s...

Guillain-Barré syndrome (GBS) is an immune-mediated disorder characterized by acute polyneuropathy, ascending paralysis and post infectious polyneuritis. Two-thirds of patients present with a history of recent upper respiratory tract or gastrointestinal infection. The clinical history, neurologic examination and laboratory assessment allow for a straightforward diagnosis in the majority of case...

Many neuromuscular disorders affect more than skeletal muscle. Because of the common structural and now more apparent molecular features between skeletal and cardiac muscles, many of the neuromuscular disorders also result in cardiovascular complications. Cardiomyopathy and conduction system diseases are the most frequent extramuscular features seen with many muscular dystrophies. The most comm...