Intrathecal baclofen normalizes motor strategy for squatting in familial spastic paraplegia: a case study

Abstract

We aimed to assess whether intrathecal baclofen could alter the motor strategy for squatting of a patient with pure familial spastic paraplegia. Before baclofen injection and two, four and six hours after it, the patient was evaluated as follows: self-report of walking stiffness and movement initiation; muscle tone with the Ashworth scale; and kinematic and electromyographic analysis of the squatting movement using the opto-electronic ELITE system. The patient's subjective improvement and decrease in muscle tone were dramatic after baclofen injection. Kinematic analysis of squatting showed gradual improvement. Before the injection, the movement was performed with loss of trunk verticality, backward shift of the hip, multiphasic ascending phase of the knee angular velocity and dynamic ankle stiffening. After baclofen injection, the movement was made with vertical translation of body segments and monophasic ascending phase of the knee angular velocity. The effect was maximal six hours after the injection. Electromyographic activities showed a non-specific co-contraction pattern before the injection, and a reciprocal pattern two hours after it. Moreover, a physiological anticipatory deactivation of the hamstring muscles appeared two hours after the injection. In this study of a single patient with familial spastic paraplegia, intrathecal baclofen has facilitated the emergence of normal, supraspinally determined movement patterns.