title: Disease progression and treatment response of idiopathic epilepsy in Australian Shepherd dogs.
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.
  
  
  OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Clinic of Small Animal Medicine, Ludwig-Maximilians-University, Munich, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Weissl
authors.forename: V
authors.initials: V
authors.lastname: Hülsmeyer
authors.forename: C
authors.initials: C
authors.lastname: Brauer
authors.forename: A
authors.initials: A
authors.lastname: Tipold
authors.forename: L L
authors.initials: LL
authors.lastname: Koskinen
authors.forename: K
authors.initials: K
authors.lastname: Kyöstilä
authors.forename: H
authors.initials: H
authors.lastname: Lohi
authors.forename: C
authors.initials: C
authors.lastname: Sauter-Louis
authors.forename: M
authors.initials: M
authors.lastname: Wolf
authors.forename: A
authors.initials: A
authors.lastname: Fischer
mesh_terms: D020168:ATP Binding Cassette Transporter, Subfamily B, Member 1 / Q000235:genetics; D000818:Animals; D000927:Anticonvulsants / Q000627:therapeutic use*; D015331:Cohort Studies; D004247:DNA / Q000737:chemistry / Q000235:genetics; D004283:Dog Diseases / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology*; D004285:Dogs; D004827:Epilepsy / Q000188:drug therapy / Q000235:genetics / Q000473:pathology / Q000662:veterinary*; D005260:Female; D005838:Genotype; D053208:Kaplan-Meier Estimate; D008137:Longitudinal Studies; D008297:Male; D010375:Pedigree; D010634:Phenobarbital / Q000627:therapeutic use*; D016133:Polymerase Chain Reaction / Q000662:veterinary; D016016:Proportional Hazards Models; D012640:Seizures / Q000188:drug therapy / Q000235:genetics / Q000473:pathology / Q000662:veterinary*
chemical_list: D020168:ATP Binding Cassette Transporter, Subfamily B, Member 1; D000927:Anticonvulsants; D004247:DNA; D010634:Phenobarbital