Referenced in: none

Automatically associated channels: Kv7.2 , Slo1

Title: Abnormal gating of axonal slow potassium current in cramp-fasciculation syndrome.

Authors: Yoshimitsu Shimatani, Hiroyuki Nodera, Yoshiko Shibuta, Yoshimichi Miyazaki, Sonoko Misawa, Satoshi Kuwabara, Ryuji Kaji

Journal, date & volume: Clin Neurophysiol, 2014 Sep 28 , ,

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/25304174

Abstract
Cramp-fasciculation syndrome (CFS) is a heterogeneous condition with multiple underlying causes. Although dysfunction of slow K(+) channels has been reported in patients with CFS, testing all potential candidates for this problem using conventional in vitro functional analysis would be prohibitively cost- and labor-intensive. However, relatively economical and non-invasive nerve-excitability testing can identify ion channel dysfunction in vivo when combined with numerical modeling.Patients with CFS underwent nerve conduction study, needle electromyography, and nerve excitability testing. Mathematical modeling of axonal properties was applied to identify the pathophysiology.Four patients had distinct electrophysiological findings (i.e., fasciculation potentials, doublet/multiplet motor unit potentials, and sustained F responses); excitability testing showed the following abnormalities: reduction of accommodation during prolonged depolarization, lack of late sub excitability after a supramaximal stimulation, and reduction of the strength-duration time constant. Mathematical modeling showed a loss of voltage-dependence of a slow K(+) current. None of these patients had a mutation in the KCNQ2, 3, or 5 genes.This study showed that patients with CFS might have abnormal kinetics in a slow K(+) current.Nerve-excitability testing may aid the decision to start therapeutic intervention such as administration of slow K(+) channel openers.