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Abnormal gating of axonal slow potassium current in cramp-fasciculation syndrome
Abnormal gating of axonal slow potassium current in cramp-fasciculation syndrome
- Source :
- Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology. 126(6)
- Publication Year :
- 2014
-
Abstract
- Objective 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. Methods Patients with CFS underwent nerve conduction study, needle electromyography, and nerve excitability testing. Mathematical modeling of axonal properties was applied to identify the pathophysiology. Results 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. Conclusions This study showed that patients with CFS might have abnormal kinetics in a slow K + current. Significance Nerve-excitability testing may aid the decision to start therapeutic intervention such as administration of slow K + channel openers.
- Subjects :
- Adult
Male
Refractory period
Small-Conductance Calcium-Activated Potassium Channels
Neural Conduction
Gating
Fasciculation
Physiology (medical)
Medicine
Humans
Prospective Studies
medicine.diagnostic_test
business.industry
Electromyography
Depolarization
Neuromuscular Diseases
Middle Aged
medicine.disease
Sensory Systems
Axons
Compound muscle action potential
Electrophysiology
Neurology
Cramp fasciculation syndrome
Nerve conduction study
Female
Neurology (clinical)
medicine.symptom
business
Neuroscience
Ion Channel Gating
Subjects
Details
- ISSN :
- 18728952
- Volume :
- 126
- Issue :
- 6
- Database :
- OpenAIRE
- Journal :
- Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology
- Accession number :
- edsair.doi.dedup.....15e79a5462617b450cb60a90ac394e84