Rhythmic palatal myoclonus (RPM) is a rare movement disorder consisting of continuous synchronous jerks of the soft palate, muscles innervated by other cranial nerves and, rarely, trunk and limb muscles. It usually develops secondary to brainstem or cerebellar disease (symptomatic RPM). Some patients, however, fail to show evidence of a structural lesion (essential RPM). A total of 287 cases with RPM from the literature including 210 cases with symptomatic and 77 cases with essential RPM have been reviewed and analysed statistically to look for criteria separating the two conditions. Patients with essential RPM usually have objective earclicks as their typical complaint which is rare in the symptomatic form. Eye and extremity muscles are never involved. The jerk frequency is lower in essential than in symptomatic RPM. Patients with essential RPM are younger and have a balanced sex distribution as compared with a male preponderance in the symptomatic form. The rhythmicity of RPM seems to be more profoundly influenced by sleep, coma and general anaesthesia in essential than in symptomatic RPM. We conclude from these results that essential RPM should be separated as a distinct clinical entity. Symptomatic RPM is a rhythmic movement disorder whose pathogenesis is quite well established. The cells of the hypertrophied inferior olives are believed to represent the oscillator. Among other possibilities, essential RPM may represent its functional analogue, based on transmitter changes only. Such a relationship could be of theoretical interest for the understanding of rhythmic hyperkinesias in general.

In order to investigate the neuronal mechanisms underlying the compensatory movements following stance disturbance, leg muscle e.m.g. responses and cerebral potentials evoked by a treadmill acceleration impulse were analysed. It was found that the displacement was followed by a cerebral potential of a latency of 40-45 ms and EMG responses in the calf muscles at a latency of 65-70 ms. The e.m.g. responses represented specific compensatory reactions to the mode of perturbation (with a gastrocnemius activation following positive acceleration but a tibialis ant. activation following negative acceleration). The cerebral potentials, however, showed a common pattern to both conditions. In addition, the leg muscle e.m.g. reactions were not altered by learning effects and by forewarning of displacement onset, while the amplitude of the cerebral potentials was significantly smaller in these conditions compared to those produced in response to randomly induced perturbations. It was therefore concluded that the leg muscle e.m.g. reactions are mediated by a polysynaptic spinal reflex pathway which depends on a supraspinal control. The cerebral potentials seem to represent afferent signals which can be supposed to be subjected to modification and processing by supraspinal motor centres, according to the actual requirements.

The interaction of transcranial electric and magnetic brain stimulation with electrically elicited short- and long latency reflexes (LLR) of hand and forearm flexor muscles has been investigated in normal subjects. In the first paradigm, the motor potential evoked in thenar muscles by transcranial stimulation was conditioned by median nerve stimulation at various conditioning-test intervals. At short intervals (electric: 5-12.5 ms, magnetic: 0-7.5 ms) facilitation occurred that corresponded to the H-reflex and at longer intervals (electric: 25-40 ms, magnetic: 22.5-35 ms) there was a facilitation corresponding to the LLR. Electric and magnetic stimulation resulted in a similar degree of facilitation. A second paradigm investigated the facilitation of the forearm flexor H-reflex by a cutaneo-muscular LLR elicited by radial superficial nerve stimulation and transcranial stimulation used separately or together. When electric and magnetic brain stimulation were compared, magnetic brain stimulation was followed by significant extrafacilitation but electric stimulation was not. This result favours an interaction between the afferent volley eliciting the LLR and transcranial magnetic stimulation most likely at supraspinal level.

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SUMMARY Forty five patients with essential tremor have been investigated by means of clinical examination, polygraphic EMG records and testing of long-latency reflexes. Clinically there were no differences between the patients, whereas the electrophysiological investigations suggested two subtypes. One group of patients may be characterised by normal long-latency reflexes and synchronous tremor bursts in antagonists or activity of the antigravity muscle alone. The second group had abnormal long-latency reflexes and reciprocal EMG activity in antagonists. It is suggested that these two groups represent distinct subgroups of essential tremor. Patients of the first group responded well to propranolol, whereas those of the second group did not.