Disease progression and treatment response of idiopathic epilepsy in Australian Shepherd dogs

J Vet Intern Med. 2012 Jan-Feb;26(1):116-25. doi: 10.1111/j.1939-1676.2011.00853.x. Epub 2011 Dec 19.

Abstract

Background: Idiopathic epilepsy (IE) in Australian Shepherds (ASs) occurs worldwide but there is a lack of description of the epilepsy syndrome in this breed. The ABCB1-1Δ mutation is more prevalent in ASs than in many other dog breeds.

Hypothesis: Australian Shepherds suffer from a poorly controlled IE syndrome with prevailing severe courses. Seizure control and ABCB1-1Δ mutation might be related in this breed.

Animals: Fifty ASs diagnosed with IE and 50 unaffected ASs.

Methods: Predominant study design is a longitudinal cohort study. Pedigrees, medical records, seizure, and treatment data of ASs with IE were analyzed descriptively. Sex, color, and the ABCB1-1Δ genotype were compared between case and control groups and ASs with poorly or well-controlled seizures. Differences in survival times were assessed by logrank tests and Cox regression analysis.

Results: Idiopathic epilepsy in ASs is dominated by moderate and severe clinical courses with the occurrence of cluster seizures and status epilepticus and a high seizure frequency. Poor seizure control and a high initial seizure frequency (≥10 seizure days/first 6 months) are associated with shorter survival times (P < .05). Poor seizure control, unrelated to the ABCB1(MDR1) genotype, is evident in 56% of epileptic ASs. Pedigree analysis suggests a genetic basis.

Conclusion and clinical importance: Frequent severe clinical courses, poor seizure control unrelated to the ABCB1(MDR1) genotype, and a young age at death compromise animal welfare and warrant further genetic studies to unravel the underlaying molecular mechanisms of IE and seizure control in the breed.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • ATP Binding Cassette Transporter, Subfamily B, Member 1 / genetics
  • Animals
  • Anticonvulsants / therapeutic use*
  • Cohort Studies
  • DNA / chemistry
  • DNA / genetics
  • Dog Diseases / drug therapy*
  • Dog Diseases / genetics
  • Dog Diseases / pathology*
  • Dogs
  • Epilepsy / drug therapy
  • Epilepsy / genetics
  • Epilepsy / pathology
  • Epilepsy / veterinary*
  • Female
  • Genotype
  • Kaplan-Meier Estimate
  • Longitudinal Studies
  • Male
  • Pedigree
  • Phenobarbital / therapeutic use*
  • Polymerase Chain Reaction / veterinary
  • Proportional Hazards Models
  • Seizures / drug therapy
  • Seizures / genetics
  • Seizures / pathology
  • Seizures / veterinary*

Substances

  • ATP Binding Cassette Transporter, Subfamily B, Member 1
  • Anticonvulsants
  • DNA
  • Phenobarbital