Leukoencephalomyelopathy

two rottweilers in the back of a car

A neurological disorder, termed leukoencephalomyelopathy (LEMP) has been described in Rottweiler and Leonberger dogs. LEMP is a recessively inherited neurodegenerative disorder that affects the white matter of the central nervous system (CNS).  Canine LEMP is characterized by slowly worsening gait abnormalities, especially spontaneous knuckling, dragging of the paws and hypermetria of the thoracic limbs, and a characteristic pattern on magnetic resonance imaging (MRI). Affected dogs show corresponding gross lesions in the cervical spinal cord white matter that may extend to the thoracic spinal cord, as well as to the brain; peripheral nerve and muscle biopsies are unremarkable.  Canine  LEMP often shows  a  juvenile  onset  and is characterized  by  a  generalized  progressive ataxia. Spinal reflexes of affected dogs are mostly normal.  In  the  progressive  clinical  course  of  the  disease,  affected  dogs may become  increasingly  immobile  within  a  few  months.  Like many diseases of the CNS, there is no effective treatment for LEMP. Since in most cases  the  dog  is  not  in  pain, but  is  strongly  restricted  in  its  quality  of  life,  owners  are  encouraged  to  ask a veterinarian for advice.

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Rottweilers & Great Danes

Research carried out at the University of Minnesota, the University of Bern, and Utrecht University has identified two LEMP mutations within the gene NAPEPLD, one in the Leonberger and the other in Rottweilers. The Rottweiler mutation has also been observed in Great Danes.


Rottweilers & Great Danes

LEMP in Rottweilers is a autosomal recessive central nervous system disease resulting from a 1 basepair insertion within the LEMP gene; clinical signs may develop as early as 1 year of age.  All studied Rottweilers homozygous affected (D/D) for this LEMP mutation have shown clinical signs of disease.  However, not all Rottweilers with suspected LEMP have tested affected for this mutation.  It is possible that these dogs may be suffering from a different form of LEMP or the other neurodegenerative diseases described in this breed, including - Neuroaxonal Dystrophy (NAD)

  • Population testing of >200 Rottweilers indicates that the carrier rate of this mutation is ~8%.
  • Although no affected Great Danes have yet been observed, population testing of >250 Great Danes indicates that the carrier rate of this mutation is ~8%.

Leonbergers

Research carried out at the University of Minnesota, the University of Bern, and Utrecht University has identified two LEMP mutations within the gene NAPEPLD, one in the Leonberger and the other in Rottweilers.  The Rottweiler mutation has also been observed in Great Danes.

Leonbergers

LEMP in Leonbergers is a partially penetrant autosomal recessive central nervous system disease resulting from an amino acid change within the LEMP gene.  All Leonbergers with confirmed LEMP have tested homozygous affected (D/D) for this mutation; however, not all dog that are homozygous for this mutation may show obvious clinical signs of disease within their lifetime.  Clinical signs may develop as early as 1 year of age.

  • Population testing of >5,000 Leonbergers indicates that the carrier rate of this mutation is ~14%.

For Leonberger dogs, we also offer genetic testing for two forms of Laryngeal Paralysis & Polyneuropathy (LPN1 & LPN2).

Scientific References

A missense mutation in the Vacuolar Protein Sorting 11 (VPS11) gene is associated with Neuroaxonal Dystrophy in Rottweiler dogs.
Lucot KL, Dickinson PJ, Finno CF, Mansour TA, Letko A, Minor KM, Mickelson JR, Drögemüller C, Brown CT,  and Bannasch DL. (2018)
G3: 8(8): 2773-2780. 

Canine NAPEPLD-associated models of human myelin disorders.
Minor KM, Letko A, Becker D, et al. (2018)
Scientific Reports volume 8, Article number: 5818 (2018) doi: 10.1038/s41598-018-23938-7

Magnetic resonance imaging and genetic investigation of a case of rottweiler leukoencephalomyelopathy.
Katrin Hirschvogel, Kaspar Matiasek, Katharina Flatz, Michaela Drögemüller, Cord Drögemüller, Bärbel Reiner and Andrea Fischer (2013)
BMC Veterinary Research 2013 9:57. doi: 10.1186/1746-6148-9-57

MRI findings in a Rottweiler with leukoencephalomyelopathy.
Joseph S. Eagleson, Marc Kent, Simon R. Platt, Raquel R. Rech, and Elizabeth W. Howerth (2013)
Journal of the American Animal Hospital Association: July/August 2013, Vol. 49, No. 4, pp. 255-261. doi: 10.5326/JAAHA-MS-5864

A novel leukoencephalomyelopathy of Leonberger dogs.
Oevermann, A., Bley, T., Konar, M., Lang, J. and Vandevelde, M. (2008) 
Journal of Veterinary Internal Medicine, 22: 467–471. doi: 10.1111/j.1939-1676.2008.0068.x

 


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