Description:

Duchenne muscular dystrophy, an X-linked disorder, has an incidence of one in 5000 boys and presents in early childhood with proximal muscle weakness. Untreated boys become wheelchair bound by the age of 12 years and die of cardiorespiratory complications in their late teens to early 20s. The use of corticosteroids, non-invasive respiratory support, and active surveillance and management of associated complications have improved ambulation, function, quality of life and life expectancy. The clinical features, investigations and management of Duchenne muscular dystrophy are reviewed, as well as the latest in some of the novel therapies. Duchenne Muscular Dystrophy (DMD) is an X-linked recessive disorder that affects muscles and causes progressive weakness. Dystrophin, the product of the DMD gene, is one of several membrane proteins that form the dystrophin–glycoprotein complex, which helps to maintain the integrity of muscle cells; loss of these proteins leads to the wasting of muscle cells and to the pathology of DMD.

Motor neuron abnormalities have been implicated in the pathogenesis of Duchenne muscular dystrophy. Evidence concerning the effect of injury on motor neurons of human Duchenne muscular dystrophy (DMD) is lacking. We report a DMD patient having, in addition, an obstetric paresis on his left arm. EMG and muscle histochemistry showed signs of reinnervation superimposed on myopathy in his left arm. This suggests that sprouting is preserved in DMD motor neurons and that muscle fibers retain the capability of accepting reinnervation.  The cloning of the Duchenne muscular dystrophy gene, encoding the protein dystrophin, led to greater understanding of the disease pathogenesis. Studies of dystrophin associated and related proteins has led to a better understanding of other muscular dystrophies and the development of novel approaches to therapy.

Duchenne muscular dystrophy is a debilitating disease that includes significant medically and physically disabling conditions that have an effect on the dental treatment of these patients. The dental treatment considerations for the patient with Duchenne muscular dystrophy include: the current status of the disease; the progressive nature of the disease; lack of ambulation; poor muscular control and muscle weakness; muscle contractures; respiratory and cardiac complications; relative contraindication of general anesthesia; and the oral health needs of the patient. In the case reported, these problems were considered and appropriate treatment modalities selected. With appropriate medical consultation and ample consideration of the patient's physically disabling conditions, dental therapy can be modified to provide a satisfactory level of oral health for the patient with Duchenne muscular dystrophy.

With Regards
sarfania
Journal Coordinator
Global Journal of Research and Review