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Published on: January 13, 2022
Decremental responses to repetitive nerve stimulation (RNS) in motor neuron disease
Tomoko Iwanami1, Masahiro Sonoo, Yuki Hatanaka
1Department of Neurology, Teikyo University School of Medicine, Kaga 2-11-1, Itabashi-ku, Tokyo 1738605, Japan.
Objective:
To clarify the features of decremental responses following repetitive nerve stimulation in patients with motor neuron diseases (MNDs), in comparison with myasthenia gravis (MG).
Methods:
The subjects consisted of 48 MND, 39 generalized MG and 19 ocular MG patients. Six muscles, both proximal and distal muscles, were tested.
Results:
Significant decrements (>5%) in at least one muscle were observed in 83% of the MND patients, and 74% and 47% of the generalized MG and ocular MG patients, respectively. Decrements were more frequently observed in the proximal muscles both in MND and MG patients (deltoid 76% and 62%, and trapezius 71% and 51% for MND and generalized MG, respectively), suggesting lower safety factors in neuromuscular transmission in those muscles. Decrements in the nasalis were rare in MND (8%) in comparison with generalized MG (54%).
Conclusions:
Decremental responses were frequently observed in MND patients. There were small differences between MND and MG regarding the distribution and other features of decrements, such as the degree of the U-shape or the responses to different stimulus frequencies and to brief exercise.
Significance:
These results imply that the underlying mechanism regulating the decrements is common to MND and MG.
