Special devices as aids in the management of child self-mutilation in the Lesch-Nyhan syndrome.

The Lesch-Nyhan syndrome is a rare inborn error of purine metabolism associated with hyperuricacidemia, mental retardation, and an insatiable urge for self-mutilation, especially of the extremities. Insensitivity to pain and severe muscle spasms create very difficult management problems, especially for children being cared for at home. Fortunately this syndrome is rare (1 per 380,000 live births). Four known patients are under treatment in Manitoba, of which two brothers are the subject of this study. In general, the medical management of these patients has been unsuccessful, especially in controlling self-mutilation. It therefore was decided to manage these children by the use of mechanical aids. Through the utilization of custom-designed seating devices control of the self-mutilation has been obtained, reducing the burden of family care. It is now possible for the children to attend regular school on a half-day basis. They are also able to engage in community activities without the fear that a mishap will occur when not under the vigilance of the immediate family.

Mobile arm supports: an evaluation.

The results of a follow-up survey of 124 patients fitted with mobile arm supports at Mary Marlborough Lodge between 1970 and 1976 are presented. Of the patients fitted with mobile arm supports 33% are known to have used them for over one year, and 47% of those who were contacted were still using them. The reasons for non-use and the implications of the necessity for readjustments are discussed.