AUTONOMOUS PERIPHERAL-NERVE ACTIVITY CAUSING GENERALIZED MUSCLE STIFFNESS AND FASCICULATIONS - REPORT OF A CASE WITH PHYSIOLOGICAL, PHARMACOLOGICAL, AND MORPHOLOGICAL OBSERVATIONS

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  • 1 January 1976
    • journal article
    • research article
    • Vol. 139, 49-60
Abstract
A 14 yr old boy with generalized muscle weakness, stiffness and fasciculations associated with profuse and continuous electromyographic (EMG) activity was described. The spontaneous mechanical and electrical muscle activity was unaffected by sleep, general anesthesia or spinal anesthesia but was abolished by small doses of curare, succinylcholine and gallamine. Proximal and distal peripheral nerve block caused moderate and marked reduction of EMG activity, respectively, indicating that the disorder was due to autonomous peripheral nerve activity. The delayed motor nerve conduction velocities and the structural abnormalities seen in some of the myelin sheaths by light microscopy and EM on sural nerve biopsy preparations constituted further evidence that the peripheral nerve was the site of abnormality in this disorder. Diphenylhydantoin and carbamazepine maintenance therapy produced adequate clinical relief.

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