On the basis of selected cases, evaluation of the usefulness of muscular exercises in rehabilitation of patients stricken by a dysfunction of the motor system of the chewing organ was accomplished. The assessment of the electric function of chewing muscles together with the registration of mandibular movements with the axiographic Zebris JMA apparatus was made, both before and after muscular th...

BACKGROUND Cardiomyopathy is a leading cause of mortality among Duchenne muscular dystrophy patients and lacks effective therapies. Phosphodiesterase type 5 is implicated in dystrophic pathology, and the phosphodiesterase type 5 inhibitor tadalafil has recently been studied in a clinical trial for Duchenne muscular dystrophy. METHODS AND RESULTS Tadalafil was evaluated for the prevention of c...

This study evaluated whether cold-induced deterioration in neuromuscular function can be restored by intermittently increasing the workload. We examined the level of muscular strain, agonist-antagonist co-activation, the occurrence of EMG gaps and neuromuscular efficiency in wrist flexor and extensor muscles at 21°C (TN) and 4°C (C(10)) with a 10%MVC workload. During second exposure to 4°C (C(5...

OBJECTIVES Our aim was to measure the cardiac phosphocreatine to adenosine triphosphate ratio (PCr/ATP) noninvasively in patients and carriers of Xp21 muscular dystrophy and to correlate the results with left ventricular (LV) function as measured by echocardiography. BACKGROUND Duchenne and Becker muscular dystrophy (the Xp21 dystrophies) are associated with the absence or altered expression ...

facioscapulohumeral muscular dystrophy (fshd)is characterized by weakness of the facial, scapular muscles  and the dorsiflexors of the foot. severity in this disorder is highly variable. approximately 95% of individuals with fshd phenotype have type 1 fshd, with d4z4 allele of between one and ten repeat units and about 5% have type 2 fshd with mutations in the chromatin modifier smchd1gene whic...

Autophagy has recently emerged as an important cellular process for the maintenance of skeletal muscle health and function. Excessive autophagy can trigger muscle catabolism, leading to atrophy. In contrast, reduced autophagic flux is a characteristic of several muscle diseases, including Duchenne muscular dystrophy, the most common and severe inherited muscle disorder. Recent evidence demonstr...

The class of muscular dystrophies linked to the genetic ablation or mutation of dysferlin, including Limb Girdle Muscular Dystrophy 2B (LGMD2B) and Miyoshi Myopathy (MM), are late-onset degenerative diseases. In lieu of a genetic cure, treatments to prevent or slow the progression of dysferlinopathy are of the utmost importance. Recent advances in the study of dysferlinopathy have highlighted t...