Mirror movements studied in a patient with Klippel-Feil syndrome

1. Electromyographic (EMG) recordings have been made from upper limb muscles in a patient with well-defined congenital mirror movements occurring in association with Klippel-Feil syndrome and the results compared to those obtained in normal control subjects. 2. In the patient, liminal percutaneous electrical or magnetic brain stimulation applied over either hemisphere elicited bilateral and symmetrical short-latency muscle responses in relaxed intrinsic hand muscles. In the normal subjects unilateral brain stimulation only elicited contralateral muscle responses. 3. F response and H reflex studies for the patient's ulnar-supplied intrinsic hand muscles were normal. No crossed responses were recorded in the homologous muscles of the contralateral hand. 4. Scalp-recorded somatosensory-evoked responses following ulnar or median nerve stimulation were of normal latency and distribution in the patient. 5. In the patient, cross-correlation analysis of on-going single and multiunit needle EMGs recorded between muscles of left and right hands revealed a central peak in the cross-correlogram. No cross-correlogram peaks were found between left- and right-hand muscles in normal subjects. The magnitude and time course of the central peaks in the cross-correlograms constructed between the firing of motor units on opposite sides of the body in the patient were similar to those found in cross-correlograms constructed between the firing of motor units from muscles on the same side of the body in the patient and in normal subjects. 6. The magnitude of cross-correlogram peaks detected within a muscle and those detected between left and right homologous muscles showed a gradient in which the largest peaks were found in the intrinsic hand and forearm extensor muscles. The smallest peaks were observed in the forearm flexor muscles. No peaks were detected between left and right biceps brachii muscles. In intrinsic hand muscles, the size of the cross-correlogram peak detected between the EMGs of homologous muscle pairs was greater than that found for non-homologous muscle pairs. 7. Cutaneous reflex responses were recorded from first dorsal interosseous muscle following unilateral electrical stimulation of the digital nerves of the index finger. In the patient, this produced an early excitatory (E1) response on the stimulated side. Later excitatory (E2 and E3) responses, of approximately equal size and latency, were distributed bilaterally. In the normal subjects, reflex responses were confined to the stimulated side.(ABSTRACT TRUNCATED AT 400 WORDS)