Characterization and Mode of Inheritance of a Paroxysmal Dyskinesia in Chinook Dogs

R. A. Packer, E. E. Patterson, J. F. Taylor, J. R. Coates, R. D. Schnabel, D. P. O'Brien

Research output: Contribution to journalArticlepeer-review

18 Scopus citations

Abstract

Background: Paroxysmal dyskinesias are episodes of abnormal, involuntary movement or muscle tone, distinguished from seizures by the character of the episode and lack of seizure activity on ictal EEG.Hypothesis: Paroxysmal dyskinesia is an inherited, autosomal recessive disorder in Chinook dogs.Animals: Families of Chinook dogs with paroxysmal dyskinesia.Methods: Pedigrees and medical histories were reviewed for 299 Chinook dogs. A family of 51 dogs was used for analysis. Episodes were classified as seizures, paroxysmal dyskinesia, or unknown, and segregation analysis was performed.Results: Paroxysmal dyskinesia was identified in 16 of 51 dogs and characterized by an inability to stand or ambulate, head tremors, and involuntary flexion of 1 or multiple limbs, without autonomic signs or loss of consciousness. Episode duration varied from minutes to an hour. Inter-ictal EEGs recorded on 2 dogs with dyskinesia were normal. Three dogs with dyskinesia also had generalized tonic-clonic seizures. One of 51 dogs had episodes of undetermined type. Phenotype was unknown for 6 of 51 dogs, and 28 dogs were unaffected. Segregation was consistent with an autosomal recessive trait.Conclusions and Clinical Importance: This movement disorder is prevalent in the Chinook breed, and consistent with a partially penetrant autosomal recessive or polygenic trait. Insufficient evidence exists for definitive localization; episodes may be of basal nuclear origin, but atypical seizures and muscle membrane disorders remain possible etiologies. The generalized seizures may be a variant phenotype of the same mutation that results in dyskinesia, or the 2 syndromes may be independent.

Original languageEnglish (US)
Pages (from-to)1305-1313
Number of pages9
JournalJournal of veterinary internal medicine
Volume24
Issue number6
DOIs
StatePublished - Nov 2010

Keywords

  • Basal nuclei
  • Channelopathy
  • Movement disorder
  • Muscle membrane
  • Seizure

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