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This case report describes an 11-year-old boy with spastic diplegia whose reflex status, range of motion (ROM), strength, and motor performance were measured before and after implantation of an indwelling system for delivery of intrathecally administered baclofen. Before baclofen use, the subject experienced clonus that interfered with walking, needed assistance with transfers, and was unable to independently put on underwear and socks. Measures of spasticity, kinematics and electromyographic activity during voluntary movements, ROM, Gross Motor Function Measure (GMFM) scores, and self-reports of change were obtained at baseline, before and after bolus baclofen injection, during a double-blind placebo-controlled clinical trial of baclofen administration via an indwelling pump, and after 1 and 2 years of baclofen therapy. Spasticity, Babinski reflexes, clonus, strength, and coactivation of antagonist muscles during voluntary movement were decreased shortly after baclofen administration began. Hip and ankle ROM increased, upper-extremity movement speed increased, independence in dressing and transfers improved, and orthoses were discarded. After 1 and 2 years, GMFM scores were 7.8% and 6.4% above baseline, respectively; the subject won a fitness award. After 2 years, ROM was worse than at baseline and concerns regarding hip subluxation arose. Single-joint movement control and independence improved and spasticity decreased during baclofen administration.

The purpose of this article is to review the literature on the effects of intrathecally administered baclofen on impairment in spasticity and muscle activation patterns, on functional limitations in mobility and self-care, and on disability in daily life roles. We found plentiful evidence of improvement in spasticity, spasms, and bladder function and some reports of improved patterns of muscle activation and kinematics of single-joint movement. Improved ability to accomplish transfers, self-care, and locomotion is less consistently studied but has also been reported in about 60% to 70% of patients. Evidence of improved quality of life is primarily anecdotal but may be found in 10% to 30% of patients. We conclude that research protocols should be developed to clarify effects on control of voluntary movement, functional limitations, and quality of life.

Background and Purpose: The purpose of this study was to investigate the effects of prolonged practice of a simple motor task (a fast, unidirectional single-joint movement) on different indexes of motor performance in individuals with Down syndrome., Subjects: Eight individuals with Down syndrome were tested before and after practice involving 1,100 movements., Methods: The test consisted of three series of elbow flexion movements. In the first series, the subjects were asked to move "as fast as possible" over four distances. In the second series, the subjects moved over one distance at a "comfortable speed." The initial position of the elbow joint for these two series of movements was 55 degrees into flexion (full elbow extension equals 0 degrees). In the third series, the subjects were also asked to move "as fast as possible" over two distances, but from another initial elbow position (73 degrees into flexion)., Results: After training over a 2-week period, all subjects improved their performance on all tasks as reflected by both kinematic and electromyographic data. In particular, they increased the quantity of the agonist activity, decreased the antagonist onset latency, and doubled their peak velocity. They were able to transfer the improvement in their performance to the nontrained distances and to the different starting position. Subjects decreased their movement time by proportionally decreasing both the acceleration and deceleration times., Conclusion and Discussion: This study supports the idea that subjects with Down syndrome can use patterns of muscle activation that are qualitatively indistinguishable from those used by individuals who are neurologically normal. With appropriate training, individuals with Down syndrome achieved similar levels of motor performance to that described in the literature for individuals who are neurologically normal.