CONTRACTURES
A contracture is a permanent shortening of a muscle that causes a deformity with or without pain. Providing frequent changes in position and range of motion exercises is important to prevent contrac- tures.
The participation of a physiotherapist in his care is critically important to prevent serious pro- gressive deformity. In the advanced stages of HD, his ability to control movement becomes severely compromised. Those who once had involuntary movements may now be rigid and vulnerable to developing contractures. Even though he may still have involuntary movements, he cannot change his position. The fluctuations in muscle tone and the involuntary movements make it difficult to prevent and manage contractures.
Typical approaches such as orthoplastic splints can easily cause skin problems. More useful, especially for knee and elbow contractures, are newer air-assist splints which use air bladders for support. The "give" in the soft splint prevents skin from breaking down. There are also lightweight, washable foam-core splints that can be helpful in maintaining functional positions of the hands.
Since rigidity is typical in people with juvenile HD, contractures may be a problem earlier in their disease. People with adult-onset HD may have involuntary movements that progress into rigidity. People who develop HD in their early twenties may present with stiffness, slowness, and occasional involuntary movements. Over time all involuntary movements are slowed, and dystonia and loss of motor control dominate.
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Prevention of contractures is very important in maintaining ability to walk and to do daily living activities. Contractures are prevented by actively exercising the muscles and joints through their range daily. If the individual cannot do so due to muscle weakness, the family or friends can do gentle passive range of motion under the guidance of programs developed by the physician, the physical therapist, or the occupational therapist. Splints are also important in preventing contractures, especially at night when sleeping postures frequently encourage the development of contractures.
Once contractures develop they are often very difficult to correct. Physical and occupational therapists frequently help in stretching out the contractures. However, the therapists cannot do this stretching alone. The family and the individual with the neuromuscular disease must be actively involved. If the contracture cannot be corrected with exercise, surgery is sometimes necessary. However, the most important way to deal with contractures is to prevent them. (Johnson, E.R.: Archives Physical Medicine & Rehabilitation 73:807, 1992).
OTHER RESOURCES:
Movement Disorder in HD (CHAPTER 3)
Physical and Occupational Therapy for Huntington
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