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Pure stimulus-sensitive truncal myoclonus of propriospinal origin

✍ Scribed by A. Schulze-Bonhage; H. Knott; Dr. A. Ferbert


Publisher
John Wiley and Sons
Year
1996
Tongue
English
Weight
372 KB
Volume
11
Category
Article
ISSN
0885-3185

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✦ Synopsis


The clinical and electrophysiological features of stimulus-sensitive truncal myoclonus are described in a 49-year-old woman. Touching the skin of the back and abdomen would evoke jerks in both ipsilateral and contralateral axial muscles; there was no spontaneous jerking. Multichannel EMG recordings showed bilateral short-latency muscle bursts at truncal recording sites both rostral and caudal to stimulus sites. The short latencies of muscle bursts in adjacent segments give evidence of a spinal origin of myoclonus; the pattern of recruitment and the velocity of spread suggest the involvement of propriospinal pathways. The presence of intrathecal IgG synthesis and of oligoclonal bands in the CSF point towards an inflammatory process which may underly the unusual type of myoclonus in this patient.


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## Abstract Two unrelated patients developed bulbar symptoms, followed within several weeks by spontaneous myoclonus and painful, generalized, stimulus‐sensitive jerks triggered by unexpected noises and cutaneous stimuli. They progressed to respiratory arrest and required mechanical ventilation, bu