J Clin Neurol.  2014 Jan;10(1):69-71.

Myasthenia in Acquired Neuromyotonia

Affiliations
  • 1Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
  • 2Department of Neurology, Keimyung University Dongsan Medical Center, Daegu, Korea.
  • 3Department of Neurology, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul, Korea. nrhong@gmail.com

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 myasthenia gravis (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.

Keyword

neuromyotonia; myasthenia; neuromuscular junction

MeSH Terms

Antibodies
Isaacs Syndrome*
Myasthenia Gravis
Neuromuscular Junction
Peripheral Nerves
Potassium Channels, Voltage-Gated
Antibodies
Potassium Channels, Voltage-Gated

Reference

1. Maddison P. Neuromyotonia. Clin Neurophysiol. 2006; 117:2118–2127.
Article
2. Auerbach A, Akk G. Desensitization of mouse nicotinic acetylcholine receptor channels. A two-gate mechanism. J Gen Physiol. 1998; 112:181–197.
3. Singh G, Avasthi G, Khurana SB. Continuous muscle fibre activity: description of neuroelectromyographic findings including single fibre EMG. Electromyogr Clin Neurophysiol. 1998; 38:121–127.
4. van Dijk JG, Lammers GJ, Wintzen AR, Molenaar PC. Repetitive CMAPs: mechanisms of neural and synaptic genesis. Muscle Nerve. 1996; 19:1127–1133.
Article
5. Torbergsen T, Stålberg E, Brautaset NJ. Generator sites for spontaneous activity in neuromyotonia. An EMG study. Electroencephalogr Clin Neurophysiol. 1996; 101:69–78.
Article
6. Valli G, Barbieri S, Cappa S, Pellegrini G, Scarlato G. Syndromes of abnormal muscular activity: overlap between continuous muscle fibre activity and the stiff man syndrome. J Neurol Neurosurg Psychiatry. 1983; 46:241–247.
Article
7. Maddison P, Mills KR, Newsom-Davis J. Clinical electrophysiological characterization of the acquired neuromyotonia phenotype of autoimmune peripheral nerve hyperexcitability. Muscle Nerve. 2006; 33:801–808.
Article
Full Text Links
  • JCN
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2025 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr