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Ezocgabine or retigabine, an antiepileptic drug of remarkable efficacy, has revolutionized the management of seizures. It is a potassium channel activator, explicitly targeting the family of Q subtype potassium channels. It enhances the transmembrane potassium currents, regulating neuronal excitability. This action stabilizes the resting membrane potential, a pivotal factor in mitigating the hyperexcitability that characterizes epilepsy.
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Absence Seizures as a Feature of Juvenile Myoclonic Epilepsy in Rhodesian Ridgeback Dogs.

F Wielaender1, F M K James2, M A Cortez3,4

  • 1Centre for Clinical Veterinary Medicine, LMU Munich, Munich, Germany.

Journal of Veterinary Internal Medicine
|December 2, 2017
PubMed
Summary

Juvenile myoclonic epilepsy in Rhodesian Ridgebacks is linked to a DIRAS1 variant. Levetiracetam effectively reduced seizures, highlighting parallels with human epilepsy.

Keywords:
DIRAS1CanineElectroencephalography (EEG)Wireless video-EEG

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Area of Science:

  • Veterinary Neurology
  • Canine Genetics
  • Epileptology

Background:

  • Myoclonic epilepsy in Rhodesian Ridgebacks (RR) presents with juvenile onset, myoclonic seizures during relaxation, and occasional generalized tonic-clonic seizures.
  • This condition shares similarities with human juvenile myoclonic epilepsy (JME).

Observation:

  • An 8-month-old RR with early-onset seizures was identified with a homozygous DIRAS1 variant.
  • Video-electroencephalography (EEG) revealed frequent generalized spike-and-wave complexes during staring episodes and myoclonic seizures.
  • Absence seizures were also observed, further strengthening the comparison to human JME.

Findings:

  • The RR dog exhibited frequent, generalized 4 Hz spike-and-wave complexes during staring episodes and myoclonic seizures.
  • Levetiracetam treatment resulted in a significant >95% reduction in myoclonic seizure frequency.
  • Absence seizures were no longer detected after treatment initiation.

Implications:

  • The DIRAS1 variant is implicated in canine myoclonic epilepsy, offering a genetic model for studying epilepsy.
  • Levetiracetam demonstrates efficacy in managing this canine epilepsy, suggesting its potential therapeutic value.
  • This study reinforces the value of canine models for understanding human neurological disorders like juvenile myoclonic epilepsy.