Involuntary movements and abnormal spontaneous EMG activity in syringomyelia and syringobulbia

To describe different types of involuntary movements and abnormal spontaneous electromyographic (EMG) activity in patients with syringomyelia.

A comprehensive study on involuntary movements in patients with syringomyelia has not yet been undertaken, to these authors' knowledge.

One hundred adult patients with syringomyelia were examined over the last 15 years. Involuntary movements were videotaped and evaluated by two independent observers. Electromyographic recordings were made using bipolar surface electrodes. The H-reflex recovery curve was obtained after stimulation of the median nerve at the elbow and recording from the flexor carpi radialis.

Involuntary movements or abnormal postures were observed in 22 patients. Three patients showed segmental spinal myoclonus, nine minipolymyoclonus, and four propriospinal myoclonus. Five patients had unilateral or bilateral hand postural tremor (8-10 Hz). Focal or segmental dystonia was observed in three patients. Electromyography showed spontaneous bursts of grouped action potentials synchronous in muscles innervated by the same spinal segment, synchronous firing of neurogenic motor unit potentials, or continuous motor unit activity. Increased H-reflex responses to conditioning stimuli were found in patients with spinal myoclonus. Long latency responses were obtained during peripheral nerve stimulation in four patients. Four patients had rigidity and abnormal upper limb posture. Respiratory synkinesis was observed in three patients. One patient developed inverse masticatory muscle activity.

Patients with syringomyelia showed a wide spectrum of involuntary movements. An increased excitability of spinal motor neurons was probably the basic underlying mechanism.