CORRELATION OF FLEXION CONTRACTURES WITH UPPER EXTREMITY FUNCTION ANDPAIN FOR SPINAL MUSCULAR-ATROPHY AND CONGENITAL MYOPATHY PATIENTS

To ascertain the patients' perception of the consequences of elbow fle xion. contractures and better understand the circumstances at their in ception, we surveyed 405 spinal muscular atrophy and congenital myopat hy patients. Diagrams of various elbow angles and questions concerning the effect of elbow contractures on daily activities were part of the survey. Of 108 completed responses, effectively a 24% response rate, 49 reported elbow flexion contractures. Thirteen of the 49 patients re ported first noting them during extended periods of inactivity. Elbow flexion contractures greater than 25 degrees were intractable and were reported to hamper 17% (77 of 410) of specifically considered daily a ctivities for the 49 subjects. Twenty-nine of the 49 (59%) subjects wi th contractures complained of contracture-associated hindrance of at l east one daily function. Perceived contracture-associated difficulties increased significantly as a function of contracture severity. Elbow contractures were perceived to be useful by 12% of the respondents. Th e correlation between contracture severity and upper limb discomfort w as also striking. We conclude that elbow flexion contractures are comm on and perceived to be associated with disability and discomfort for a bout one-half of spinal muscular atrophy and congenital myopathy patie nts.