Clinical Neurophysiology of Myoclonus



Clinical Neurophysiology of Myoclonus


Peter Brown





INTRODUCTION

It has been estimated that around 20% of myoclonus cases in specialist movement disorder clinics have a psychogenic etiology and that this accounts for around 10% of all psychogenic movement disorders evaluated (1). The separation of psychogenic jerks from tics and myoclonus can be a particularly difficult clinical problem. Neurophysiologic investigation can be helpful in this regard, but should be interpreted in the setting of the clinical assessment.


ELECTROPHYSIOLOGIC FINDINGS INDICATIVE OF ORGANIC MYOCLONUS

There are several good electrophysiologic clues that jerks may have an organic etiology. First, there are three general observations that are highly suggestive of organic myoclonus. These are (i) a mean duration of electromyographic (EMG) activity during the jerks of 70 ms or less, (ii) a mean latency of EMG responses of under 70 ms in reflex jerks elicited by sound or peripheral somaesthetic stimuli, and (iii) a recruitment of muscles within jerks that has a stereotyped order and little temporal variability across trials. This stereotyped pattern of muscle recruitment may be the orderly craniocaudal pattern of cortical myoclonus or the caudorostral and rostrocaudal patterns of brainstem jerks (including hyperekplexia) and propriospinal myoclonus, in which EMG activity is initially recorded in muscles innervated by the lower brainstem or a given spinal segment, respectively. Second, there are those electrophysiologic findings specific to cortical myoclonus, namely, the presence of a time-locked cortical correlate that precedes spontaneous and action-induced jerks with a short latency and the presence of a giant cortical evoked potential following peripheral stimulation (2).


ELECTROPHYSIOLOGIC FINDINGS SUGGESTIVE OF PSYCHOGENIC JERKS

Here it is useful to consider spontaneous and reflex jerks separately.



Spontaneous Jerks

Self-paced voluntary movements are preceded by a premovement potential—the readiness potential or Bereitschaftspotential. This is a slow negative potential beginning around one second prior to movement and maximal over the vertex (3). It is illustrated by the thin electroencephalographic (EEG) traces in Figure 30.1. In contrast, a slow premovement potential does not precede organic spontaneous myoclonus, although a very much briefer cortical spike discharge or sharp wave may precede the EMG discharges in cortical myoclonus by an interval of 20 to 40 ms, depending on whether the muscle under investigation is in the upper or lower limb (2). The bold EEG traces in Figure 30.1 show the absence of premovement potential prior to EMG bursts in a patient with abdominal flexion jerks due to tics.

The presence of a long premovement potential beginning more than 800 ms prior to jerks is very good evidence in favor of psychogenic myoclonus during the recording session (4), although it will not distinguish those patients with entirely psychogenic jerks from those with an organic movement disorder who elaborate with a preponderance of psychogenic jerks.

The presence of an abbreviated premovement potential, beginning less than 800 ms prior to the jerks is less helpful. Libet (5) showed that healthy subjects can have such shortened premovement potentials if they do not deliberately think about the timing of the movement, so it is likely that some psychogenic myoclonus may also be accompanied by abbreviated premovement potentials. On the other hand, some organic forms of jerks may also be accompanied by what effectively appear to be abbreviated premovement potentials. This may, for example, be true of the minority of patients with tics. Karp et al. found very brief premovement potentials (similar to the very late NS’ component of the normal premovement potential) in two out of their five patients with tics (6). However, Obeso et al. reported a complete absence of premovement potentials preceding tics in their cases (7), and Tijssen et al. reported three patients with an unusual late-onset posttraumatic tic disorder that was also not preceded by any premovement potential (8). Shibasaki et al. reported premovement potentials in the chorea of choreoacanthocytosis (9), but this is difficult to interpret, given that the chorea in Huntington disease involves no such EEG potential. There has also been a report of abbreviated premovement potentials prior to the jerks of myoclonus-dystonia, although this has not been replicated (10).

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Sep 12, 2016 | Posted by in PSYCHIATRY | Comments Off on Clinical Neurophysiology of Myoclonus

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