title: Hereditary polyneuropathy in the Alaskan Malamute.
abstract: OBJECTIVE
  To prove the hypothesis that a polyneuropathy in Alaskan Malamutes has a genetic background.
  
  
  METHODS
  Pedigrees of 131 related Alaskan Malamutes were included in the current study. Neurological examination, electrodiagnosis as well as muscle and nerve biopsies could be performed in 10 dogs. Information about the disease status of the other 121 Alaskan Malamutes were supplied by referring veterinarians, breeders and owners. Segregation analysis using four different models (monogenic, polygenic, mixed monogenic-polygenic and the phenotypic model) was performed on 71 dogs to test the different mechanisms of genetic transmission.
  
  
  RESULTS
  In seven clinically affected dogs abnormal electromyographic findings and reduced nerve conduction velocity were detected. Suspected diagnosis of polyneuropathy was confirmed by nerve biopsy results, characterized by axonal degeneration and hypomyelination. Muscle specimens revealed signs of neurogenic myopathy. Three related clinically normal Alaskan Malamutes also displayed moderate neuromuscular changes in histopathology. In the segregation analysis the polygenic model proved as best suitable to explain the observed segregation pattern among all other models tested.
  
  
  CONCLUSIONS
  The current study could demonstrate that polyneuropathy in Alaskan Malamutes is a hereditary disease with variable phenotypic expression ranging from severely affected to subclinical forms, which has to be considered in future gene analysis studies.
authors.affiliation: Tierärztliche Praxis für Neurologie, Mainfrankenpark 16b, 97337 Dettelbach. info@tierneurologie.de
authors.forename: K
authors.initials: K
authors.lastname: Rentmeister
authors.forename: T
authors.initials: T
authors.lastname: Bilzer
authors.forename: S
authors.initials: S
authors.lastname: Petri
authors.forename: G
authors.initials: G
authors.lastname: Schanen
authors.forename: M
authors.initials: M
authors.lastname: Fehr
authors.forename: O
authors.initials: O
authors.lastname: Distl
authors.forename: A
authors.initials: A
authors.lastname: Tipold
mesh_terms: D000818:Animals; D001947:Breeding; D004283:Dog Diseases / Q000175:diagnosis / Q000235:genetics*; D004285:Dogs; D004576:Electromyography / Q000662:veterinary; D005260:Female; D008297:Male; D008957:Models, Genetic; D009132:Muscles / Q000473:pathology / Q000503:physiopathology; D009460:Neurologic Examination / Q000662:veterinary; D010375:Pedigree; D011115:Polyneuropathies / Q000175:diagnosis / Q000235:genetics / Q000662:veterinary*; D011446:Prospective Studies