Spinal Cord Compression

The bones that stabilize the connection between head and neck can be malformed (odontoid dysplasia) making the neck unstable. This happens more often in individuals affected with a severe form of MPS I disease. This puts people with MPS I at risk of spinal cord compression (a condition where fluid or tissues such as bones are pressing on the spinal cord). Fusion surgery is used to connect the bones to each other so they do not slip further. Some patients affected with Hurler disease appear to have occasional pain in the back of the neck. It may be recommended that children with MPS I should avoid “high risk” activities such as contact sports and gymnastics. In addition, it is encouraged that these individuals be treated with caution when undergoing positioning for anesthesia. If there is severe pain or pain associated with weakness or tremors in the lower legs, a doctor may recommend studies of the neck to evaluate for slippage of the neck vertebrae.

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