Leukoencephalomyelopathy

Other Names: Canine Leukoencephalomyelopathy, LEMP
Affected Genes: NAPEPLD
Inheritance: Autosomal Recessive
Mutation: chr18:16987327-16987328 (canFam3): 1 bp insertion (ins C)

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Common Symptoms

Leukoencephalomyelopathy is an inherited progressive neurological disease affecting Rottweilers. Affected dogs experience degeneration of a fatty tissue layer, known as Myelin, which forms an insulating sheath around nerves and increases the speed of nerve signal transmission. Dogs with Leukoencephalomyeopathy present as early as one year of age with slowly progressive signs of neurological dysfunction including incoordination, high stepping gait, and weakness in all four limbs. A wide-based stance with occasional involuntary crossing of limbs is common. Inappropriate placement of feet while walking may result in severe toenail wear. Affected dogs are often humanely euthanized by five years of age due to increasingly severe neurological dysfunction and a lack of effective treatment options.

Breed-Specific Information for the Rottweiler

The Mutation of the NAPEPLD gene associated with leukoencephalomyelopathy has been identified in Rottweilers. Though the exact frequency in the overall rottweiler population is unknown, approximately 7% out of 233 Rottweilers tested were carriers of the mutation and 2% were at-risk.

Testing Tips

Genetic testing of the NAPEPLD gene in Rottweilers will reliably determine whether a dog is a genetic Carrier of leukoencephalomyelopathy. Leukoencephalomyelopathy is inherited in an Autosomal Recessive manner in dogs meaning that they must receive two copies of the mutated gene (one from each parent) to develop the disease. In general, carrier dogs do not have features of the disease but when bred with another carrier of the same Mutation, there is a risk of having affected pups. Each pup that is born to this pairing has a 25% chance of inheriting the disease and a 50% chance of inheriting one copy and being a carrier of the NAPEPLD gene mutation. Reliable genetic testing is important for determining breeding practices. Because symptoms do not appear until adulthood, genetic testing should be performed before breeding. In order to eliminate this mutation from breeding lines and to avoid the potential of producing affected pups, breeding of known carriers to each other is not recommended. Rottweilers that are not carriers of the mutation have no increased risk of having affected pups.

There may be other causes of this condition in dogs and a normal result does not exclude a different mutation in this gene or any other gene that may result in a similar genetic disease or trait.

References

  • Gamble DA and Chrisman CL. A Leukoencephalomyelopathy of Rottweiler Dogs. Vet Pathol. 1984 May;21(3):274-80. doi: 10.1177/030098588402100302. [PubMed: 6730216]
  • Hirschvogel K, Matiasek K, Flatz K, Drogemuller M, Drogemuller C, Reiner B, Fischer A. Magnetic resonance imaging and genetic investigation of a case of rottweiler leukoencephalomyelopathy. BMC Vet Res. 2013 Mar 26;9:57. doi: 10.1186/1746-6148-9-57. [PubMed: 23531239]
  • Minor KM, Letko A, Becker D, Drogemuller M, Mandigers PJJ, Bellekom SR, Leegwater PAJ, Stassen QEM, Purschbach K, Fischer A, Flegel T, Matiasek K, Ekenstedt KJ, Furrow E, Patterson EE, Platt SR, Kelly PA, Cassidy JP, Shelton GD, Lucot K, Bannasch DL, Martineau H, Muir CF, Priestnall SL, Henke D, Oevermann A, Jagannathan V, Mickelson JR, and Drogemuller C. Canine NAPEPLD-associated models of human myelin disorders. Sci Rep. 2018 Apr 11;8(1):5818. doi: 10.1038/s41598-018-23938-7. [PubMed: 29643404]