Idiopathic Focal Dystonia: a Disorder of Muscle Spindle Afferent Processing?

As muscle spindles are involved in the sensation of position and movement of the body, we tested their involvement in the pathophysiology of idiopathic focal dystonia. Twenty patients with torticollis, nine with writer's cramp, two with blepharospasm and 16 healthy control subjects participated. In the first task, the blindfolded subject matched the position of the passively moved forearm with the opposite forearm when the elbow joint was slowly flexed. In a second matching task, passive movement was replaced by stimulation of one biceps tendon with a 50-Hz vibratory stimulus (a selective stimulus for muscle spindle Ia afferents). In normal individuals, this stimulus produces flexion of the vibrated arm around the elbow joint. Movement in both arms was recorded electronically. In experiments without vibratory stimuli, dystonic subjects showed normal movement of the tracking arm during attempts to match the position of the passively moved arm and no difference between the arms in the initial and final steady state positions. In experiments using vibratory stimuli, vibration of biceps tendons in normal subjects elicited flexion of the stimulated arm at the elbow and a matching movement of the opposite arm. In patients with dystonia, there was a similar flexion response to the vibratory stimulus in the stimulated arm but movement of the tracking arm was reduced. Taken together, these experiments suggest that there is abnormal perception of motion, but not position, in dystonic subjects. Dystonic subjects showed bilateral abnormalities of perception of the tonic vibration reflex which were remote from the clinically affected site. These findings are discussed in relationship to the role of muscle spindle Ia afferents in focal dystonia.