We present a case of a 39-year-old man with Becker muscular dystrophy and severe congestive cardiac failure. Cardiac magnetic resonance imaging revealed subendocardial late gadolinium enhancement, similar to that seen in myocardial infarction. He had no risk factors for atherosclerotic coronary artery disease and coronary angiography was normal. We propose that regional subendocardial myocardia...

Using succinylcholine and inhalation agents for patients with Duchenne muscular dystrophy is extremely risky. Those risks include heart failure, cardiac dysrhythmias, rhabdomyolysis and malignant hyperthermia. Even in emergent situations, such as intraoperative bronchospasm, succinylcholine and inhalational agents are often considered contraindicated. Nevertheless, if intraoperative bronchospas...

The algebraic sum of the R and S waves (R-S) in the V(1) lead of the electrocardiogram has been found to be significantly greater in female carriers of X-linked Duchenne muscular dystrophy (but not in women with limb-girdle muscular dystrophy) compared with normal women of comparable age. A similar E.C.G. abnormality is found in affected boys, and possibly certain carriers have a latent cardiom...

Among the modifications occurring in the uremic organism, in addition to the consequences of dialysis, myopathy and peripheral neuropathy are very significant. Children are particularly affected, as their growth and development are jeopardized. Histochemistry of muscular biopsy was used to study eighteen children with end-stage renal failure under dialysis during a ten-month period. According t...

abstract bachground: the main objective of this study was to define the clinical changes of facial deformity in patients older than 5 years with congenital torticollis treated by proximal and distal sternocleidomastoid tenotomy. methods: we retrospectively evaluated fifteen patients who had had an open tenotomy of the sternal and clavicular and mastoid origins of the sternocleidomastoid muscle ...

Nine patients with adult onset acid maltase deficiency were seen at the Nijmegen University Hospital and the St Elisabeth Hospital, Tilburg , during the period 1970-1982. Five of these patients developed respiratory failure, and in four this was the initial symptom. The occurrence of respiratory failure as an early symptom of this muscular disease is discussed.

X-linked dilated cardiomyopathy (XLDCM) is a distinct phenotype of dystrophinopathy characterized by preferential cardiac involvement without any overt skeletal myopathy. XLDCM is caused by mutations of the Duchenne muscular dystrophy (DMD) gene and results in lethal heart failure in individuals between 10 and 20 years. Patients with Becker muscular dystrophy, an allelic disorder, have a milder...

1. Emery A. Duchenne muscular dystrophy or Meryon’s disease. Lancet 2001;357:1529. 2. Mavrogeni S, Spargias C, Bratis C, et al. Myocarditis as a precipitating factor for heart failure: evaluation and 1-year follow-up using cardiovascular magnetic resonance and endomyocardial biopsy. Eur J Heart Fail 2011;13:830–7. 3. Bobo JK, Kenneson A, Kolor K, Brown MA. Adherence to American Academy of Pedia...

1. Large-scale mutagenic screens of the zebrafish genome have identified a number of different classes of mutations that disrupt skeletal muscle formation. Of particular interest and relevance to human health is a class of recessive lethal mutations in which muscle differentiation occurs normally, but is followed by tissue-specific degeneration reminiscent of human muscular dystrophies. 2. We h...