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Axonal dysfunction with voltage gated potassium channel complex antibodies.

Susanna B Park1, Cindy S-Y Lin2, Arun V Krishnan2

  • 1Institute of Neurology, University College London, United Kingdom; Neuroscience Research Australia & Prince of Wales Clinical School, University of New South Wales, Australia.

Experimental Neurology
|June 14, 2014
PubMed
Summary

Voltage-gated potassium channel (VGKC)-complex antibody disorders show distinct axonal excitability changes. Limbic encephalitis (LE) patients exhibit peripheral axonal abnormalities, unlike acquired neuromyotonia (aNMT) patients, suggesting different disease mechanisms.

Keywords:
Axonal excitabilityLeucine-rich glioma inactivated 1Limbic encephalitisNeuromyotoniaVoltage gated potassium channels

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Area of Science:

  • Neuroimmunology
  • Neurology
  • Channelopathies

Background:

  • Autoantibodies against voltage-gated potassium channel (VGKC)-associated proteins are implicated in limbic encephalitis (LE) and acquired neuromyotonia (aNMT).
  • The precise role of these antibodies in disease pathophysiology remains unclear.
  • Understanding axonal function is crucial for elucidating disease mechanisms in VGKC-complex antibody-associated disorders.

Purpose of the Study:

  • To investigate peripheral axonal excitability in patients with VGKC-complex antibody-associated disorders.
  • To differentiate the pathophysiological mechanisms underlying limbic encephalitis (LE) and acquired neuromyotonia (aNMT).
  • To explore the potential of axonal excitability studies as a marker for clinical improvement.

Main Methods:

  • Peripheral axonal excitability studies were conducted.
  • A cohort of patients with LE (N=6) and aNMT (N=11) were compared to healthy controls (HC; N=20).
  • Analysis included threshold electrotonus and recovery cycle parameters.

Main Results:

  • Limbic encephalitis (LE) patients showed significant abnormalities in peripheral axonal excitability during the acute phase.
  • These LE-related changes were distinct from the effects of antiepileptic medications and normalized with clinical recovery.
  • Acquired neuromyotonia (aNMT) patients exhibited no significant excitability changes at the stimulation site.

Conclusions:

  • The findings suggest a distal origin of hyperexcitability in aNMT, likely at the motor nerve terminal.
  • Prominent excitability changes in LE indicate a complex disturbance of the axonal membrane, electrolyte imbalance, and adaptive changes.
  • Peripheral axonal excitability studies may serve as a marker for clinical improvement in LE.