Kleine Bert U, Stegeman Dick F, Drost Gea, Zwarts Machiel J
Department of Clinical Neurophysiology, Radboud University Nijmegen Medical Centre, P.O. Box 9101, 6500HB Nijmegen, The Netherlands.
Muscle Nerve. 2008 Feb;37(2):269-74. doi: 10.1002/mus.20849.
Neuromyotonia or Isaacs' syndrome is a rare peripheral nerve hyperexcitability disorder caused by antibodies against potassium channels of myelinated axons. We present the high-density surface electromyographic (EMG) recordings of a patient with fasciculations and cramps due to neuromyotonia. To characterize the time course of hyperexcitability, we analyzed the interspike intervals (ISIs) between fasciculation potentials, doublet, and multiplet discharges. ISI duration increased within each burst. The ISI histograms found can be explained by the recovery cycle of the myelinated axon and its dependency on the slow potassium conductance. We conclude that ISI analysis is a useful tool to understand the membrane dynamics underlying abnormal motor unit activity.
神经性肌强直或艾萨克斯综合征是一种罕见的周围神经兴奋性过高疾病,由针对有髓轴突钾通道的抗体引起。我们展示了一名因神经性肌强直出现肌束震颤和痉挛患者的高密度表面肌电图(EMG)记录。为了描述兴奋性过高的时间进程,我们分析了肌束震颤电位、双重放电和多重放电之间的峰间期(ISI)。每次爆发期间ISI持续时间增加。所发现的ISI直方图可以用有髓轴突的恢复周期及其对缓慢钾电导的依赖性来解释。我们得出结论,ISI分析是理解异常运动单位活动背后膜动力学的有用工具。