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Leukoencephalomyelopathy

Leukoencephalomyelopathy (LEMP) is an inherited neurodegenerative disorder affecting the white matter of the central nervous system in certain dog breeds.

Affected Genes: NAPEPLD

Inheritance: Autosomal Recessive

Variant(canFam6):
chr18:16641602-16641603 1 bp insertion C

Breed: Danoodle
Great Dane
Rottweiler

General Information: Leukoencephalomyelopathy (LEMP) primarily affects the central nervous system's white matter, manifesting as a progressive neurologic condition in breeds like Rottweilers, Leonbergers, and potentially Great Danes. The disease is characterized by the degeneration of myelin, the protective sheath surrounding nerve fibers, which crucially aids in the rapid transmission of nerve signals. Symptoms typically emerge by one year of age and progressively worsen, including incoordination, a high-stepping gait, limb weakness, and an unusual stance where limbs may involuntarily cross. Dogs may also show significant toenail wear due to dragging their feet. Although pain is not usually a symptom, the progressive loss of mobility and quality of life often leads to euthanasia by the age of five. Diagnosis is supported by MRI findings and clinical signs, with affected dogs exhibiting characteristic spinal cord changes.

How to Read Your Dog's Test Results for this Genetic Variant:

Two Variants Detected: Dog Likely Affected

One Variant Detected: Dog Unlikely Affected

No Variants Detected: No Effect

Gene / Testing Information: Genetic testing for Leukoencephalomyelopathy (LEMP) identifies mutations in the NAPEPLD gene, essential for determining carrier status and aiding in responsible breeding practices. LEMP is transmitted via an autosomal recessive pattern, requiring a dog to inherit two copies of the mutated gene—one from each parent—to develop the disease. While carriers do not show symptoms, breeding two carriers greatly increases the risk of producing affected offspring. Each offspring from such a pairing has a 25% chance of being affected and a 50% chance of being a carrier. Given the adult onset of symptoms, genetic testing is recommended before breeding to prevent the propagation of this debilitating condition. Breeding of known carriers is strongly discouraged to reduce the incidence of the disease and maintain the health of future generations. It is important to note that a clear genetic test does not rule out other neurological conditions, as other genes may also cause similar symptoms.

References:
Gamble DA and Chrisman CL. A Leukoencephalomyelopathy of Rottweiler Dogs. Vet Pathol. 1984 21(3):274-280.

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 9:57.

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 8(1):5818.