| Muscular dystrophy is a term first coined in the | | | | on his toes. This allows the line of gravity to fall |
| 1890s. It refers to certain hereditary diseases | | | | outside the vertebral column, giving greater |
| which are characterized by progressive | | | | stability. |
| degeneration of muscle. There are a number of | | | | Weakness of the shoulder muscles makes lifting |
| different kind of dystrophies, but the most | | | | the arms above the head difficult. Tiredness is |
| common is Duchenne muscular dystrophy and the | | | | associated with the increased effort required to |
| symptoms are described here. | | | | perform any gross motor task. |
| This is a progressive muscular disease | | | | A characteristic maneuver is often seen when the |
| characterized by proximal muscle (the muscles | | | | child stands up from lying or sitting on the floor. |
| closest to the trunk) weakness and wasting. It | | | | He will move into a prone position, go into the |
| primarily affects boys and becomes apparent | | | | crawl position (on forearms and knees), extend |
| between the ages of 2 and 6 years. Progress of | | | | both arms and legs (into the bear position) and |
| the disease is rapid. Walking becomes difficult and | | | | then 'walk' up his legs with his hands until upright. |
| by adolescence a wheelchair is usually necessary. | | | | The affected muscles are often wasted but this |
| The prognosis is poor, death usually occurring | | | | may be masked by the accumulation of fat. |
| during the late teens or early twenties. | | | | Tendon reflexes become progressively diminished |
| Onset of muscular dystrophy is usually around 3-4 | | | | and finally cannot be elicited. Sensation is |
| years of age. The symptoms of muscular | | | | unaffected and pain and cramps are rare. |
| dystrophy or its signs may be recognized earlier | | | | A number of children with muscular dystrophy |
| or overlooked, depending on the family history. | | | | also have intellectual impairment, particularly |
| The muscles which first show signs of weakness | | | | involving memory and verbal skills. Between the |
| are those around the hips, thighs and shoulder. | | | | ages of 8 and 12 years mobility is so restricted |
| There may be delay in walking or failure of the | | | | that use of a wheelchair become inevitable. |
| gait to become steady and coordinated. The child | | | | Contractures and deformity increase, especially of |
| may fall frequently without apparent cause. | | | | the spine and trunk. In conjunction with the |
| Navigating the steps of stairs is often very | | | | weakness of the respiratory muscles this can lead |
| difficult. Eventually the gait assumes a | | | | to an increased risk of chest infections. Death is |
| characteristic waddle with the feet placed apart | | | | usually from respiratory infection or from heart |
| and an exaggerated lumbar curve. The child walks | | | | failure due to myocardial involvement. |