Emery-dreifuss Muscular Dystrophy- Information & Treatment

By: Juliet Cohen

Emery-Dreifuss sinewy dystrophy is an uncommon and genetically heterogeneous disorder. The leading reason of mortality and morbidity in emery dreifuss sinewy dystrophy is cardiac disease, which is consistently existing. It usually begins in childhood or adolescence. In general the condition is less severe than many other forms of muscular dystrophy and though life expectancy may be shortened, many affected individuals can expect to reach middle age or older. Almost all people with Emery dreifuss muscular dystrophy have heart problems by adulthood. In many cases, these heart problems stem from abnormalities of the electrical signals that control the heartbeat and abnormal heart rhythms. The disorders affect different muscles and have different ages of onset, severity and inheritance patterns.

Onset is normally in the teenage years, but the circumstance can submit with neonatal hypotonia or through the third decade. Patients typically produce failing of peroneal muscles with toe-walking recently in the best decade or in the earlier teenage years. Among the earliest features of this disorder are joint deformities called contractures, which restrict the movement of certain joints. Contractures become noticeable in early childhood and most often involve the elbows, ankles, and neck. The condition is inherited and can therefore affect other family members. In many families it is inherited as a X linked trait and therefore only affects males and is carried by unaffected females. All the sons of an affected male will be unaffected but all his daughters will be carriers. Most affected individuals also experience slowly progressive muscle weakness and wasting, beginning in muscles of the upper arms and lower legs and progressing to muscles in the shoulders and hips.

No particular handling for emery dreifuss sinewy dystrophy exists. Aggressive positive maintenance is vital to maintain muscle action, to offer for maximal utilitarian power, and to extend living expectancy. However, having a sufficient diet and maintaining better general health are really significant as in all sinewy dystrophies. Females who carry one altered copy of the EMD gene usually do not experience the muscle weakness and wasting that are characteristic of this condition. In some cases, however, they may experience heart problems associated with this disorder. Orthopedic surgery may be needed to correct or prevent contractures and to increase range of motion. Regular gentle exercise, which is tolerated without causing stress, is beneficial. But hard physical exercise should be avoided. Antithromboembolic drugs are probably required to prevent cerebral thromboembolism of cardiac origin.

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