title: Abnormal gating of axonal slow potassium current in cramp-fasciculation syndrome.
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.
  
  
  CONCLUSIONS
  Nerve-excitability testing may aid the decision to start therapeutic intervention such as administration of slow K(+) channel openers.
authors.affiliation: Department of Neurology, Tokushima University, Tokushima, Japan.
authors.forename: Yoshimitsu
authors.initials: Y
authors.lastname: Shimatani
authors.affiliation: Department of Neurology, Tokushima University, Tokushima, Japan. Electronic address: hnodera@tokushima-u.ac.jp.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Nodera
authors.affiliation: Department of Neurology, Tokushima University, Tokushima, Japan; Department of Neurology, National Hospital Organization Takamatsu Medical Center, Takamatsu, Japan.
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Shibuta
authors.affiliation: Department of Neurology, Tokushima University, Tokushima, Japan.
authors.forename: Yoshimichi
authors.initials: Y
authors.lastname: Miyazaki
authors.affiliation: Department of Neurology, Chiba University, Chiba, Japan.
authors.forename: Sonoko
authors.initials: S
authors.lastname: Misawa
authors.affiliation: Department of Neurology, Chiba University, Chiba, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Kuwabara
authors.affiliation: Department of Neurology, Tokushima University, Tokushima, Japan.
authors.forename: Ryuji
authors.initials: R
authors.lastname: Kaji
mesh_terms: D000328:Adult; D001369:Axons / Q000502:physiology*; D004576:Electromyography / Q000379:methods; D005260:Female; D006801:Humans; D015640:Ion Channel Gating / Q000502:physiology*; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction / Q000502:physiology; D009468:Neuromuscular Diseases / Q000175:diagnosis* / Q000503:physiopathology*; D011446:Prospective Studies; D051640:Small-Conductance Calcium-Activated Potassium Channels / Q000502:physiology*
chemical_list: D051640:Small-Conductance Calcium-Activated Potassium Channels
keywords: Axonal excitability; Cramp; Fasciculation; Slow potassium channel