Excitability of motor and sensory axons in multifocal motor neuropathy

被引:8
作者
Kovalchuk, Maria O. [1 ]
Franssen, Hessel [1 ]
van den Berg, Leonard H. [1 ]
van Schelven, Leonard J. [2 ]
Sleutjes, Boudewijn T. H. M. [1 ]
机构
[1] Univ Med Ctr Utrecht, Brain Ctr Utrecht, Dept Neurol, Utrecht, Netherlands
[2] Univ Med Ctr Utrecht, Dept Med Technol & Clin Phys, Utrecht, Netherlands
关键词
Multifocal motor neuropathy; Excitability; Sensory axons; Motor axons; Ion channels; Schwann cell; INTRAVENOUS IMMUNOGLOBULIN THERAPY; NERVE-CONDUCTION; MOLECULAR ARCHITECTURE; CHANNEL DYSFUNCTION; JUNCTIONS; HYPERPOLARIZATION; TEMPERATURE; MECHANISM; CRITERIA; RELEASE;
D O I
10.1016/j.clinph.2020.08.004
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Objective: To assess excitability differences between motor and sensory axons of affected nerves in patients with multifocal motor neuropathy (MMN). Methods: We performed motor and sensory excitability tests in affected median nerves of 20 MMN patients and in 20 age-matched normal subjects. CMAPs were recorded from the thenar and SNAPs from the 3rd digit. Clinical tests included assessment of muscle strength, two-point discrimination and joint position. Results: All MMN patients had weakness of the thenar muscle and normal sensory tests. Motor excitability testing in MMN showed an increased threshold for a 50% CMAP, increased rheobase, decreased stimulus-response slope, fanning-out of threshold electrotonus, decreased resting I/V slope, shortened refractory period, and more pronounced superexcitability. Sensory excitability testing in MMN revealed decreased accommodation half-time and S2-accommodation and less pronounced subexcitability. Mathematical modeling indicated increased Barrett-Barrett conductance for motor fibers and increase in internodal fast potassium conductance for sensory fibers. Conclusions: Excitability findings in MMN suggest myelin sheath or paranodal seal involvement in motor fibers and, possibly, paranodal detachment in sensory fibers. Significance: Excitability properties of affected nerves in MMN differ between motor and sensory nerve fibers. (C) 2020 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.
引用
收藏
页码:2641 / 2650
页数:10
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