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Myasthenia in Acquired Neuromyotonia

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초록

Background Acquired neuromyotonia (NMT) forms part of the spectrum of acquired peripheral nerve hyperexcitability syndrome, and is thought to be caused by antibodies to voltage-gated potassium channels (VGKC). Exertional weakness is unusual unless autoimmune myastheniagravis (MG) is superimposed. Case Report A case of acquired NMT accompanied by exertional weakness without coexistence of seropositive MG is reported herein. Conclusions Clinical and electrophysiological observations suggest that the cholinergic overactivity in NMT can compromise the safety factor sufficiently to cause a defect in neuromuscular junction transmission.

Abstract

Background Acquired neuromyotonia (NMT) forms part of the spectrum of acquired peripheral nerve hyperexcitability syndrome, and is thought to be caused by antibodies to voltage-gated potassium channels (VGKC). Exertional weakness is unusual unless autoimmune myastheniagravis (MG) is superimposed. Case Report A case of acquired NMT accompanied by exertional weakness without coexistence of seropositive MG is reported herein. Conclusions Clinical and electrophysiological observations suggest that the cholinergic overactivity in NMT can compromise the safety factor sufficiently to cause a defect in neuromuscular junction transmission.

참고문헌 (7)

  1. Maddison P, "Neuromyotonia", Clin Neurophysiol, 117, pp.2118~2127 (2006)
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  3. Singh G, "Continuous muscle fibre activity:description of neuroelectromyographic findings including single fibre EMG", Electromyogr Clin Neurophysiol, 38, pp.121~127 (1998)
  4. van Dijk JG, "Repetitive CMAPs: mechanisms of neural and synaptic genesis", Muscle Nerve, 19, pp.1127~1133 (1996)
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  6. Valli G, "Syndromes of abnormal muscular activity: overlap between continuous muscle fibre activity and the stiff man syndrome", J Neurol Neurosurg Psychiatry, 46, pp.241~247 (1983)
  7. Maddison P, "Clinical electrophysiological characterization of the acquired neuromyotonia phenotype of autoimmune peripheral nerve hyperexcitability", Muscle Nerve, 33, pp.801~808 (2006)

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