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Spongiform leukoencephalomyelopathy (SLEM) of the Border terrier

Neurological · Dog

Spongiform leukoencephalomyelopathy described in Border terrier puppies, also known as 'shaking puppy syndrome' due to the characteristic generalized tremor. It manifests in the neonatal period with spongy degeneration of the white matter of the central nervous system. Autosomal recessive inheritance is suspected.
Inheritance patternAutosomal recessive (suspected; no gene identified)
Gene / MutationNot identified. No gene or causal variant has been described for spongiform leukoencephalomyelopathy of the Border terrier; diagnosis is based on clinical signs and histopathology of the central nervous system.
PenetranceComplete penetrance in affected animals, with neonatal expression. Heterozygotes are apparently healthy.
Codemosd
Turnaround time42 days
Price61,11 €

Incidence

Border terrier. Limited data on carrier frequency in the general population.

Breeder management

- Monitor litters with a history of affected puppies
- Do not repeat matings that have produced affected puppies
- Identify carrier breeding dogs in suspected lines when a genetic test is available
- Avoid matings between individuals from affected lines
- Exclude affected animals from breeding

Specialist notes

Differential diagnosis with other neonatal tremor syndromes (hypomyelination, hereditary tremor of other breeds) and with leukoencephalomyelopathies of other breeds. Histopathology of the central nervous system is diagnostic.

References

1. Martin-Vaquero P et al. 2012. A novel spongiform leukoencephalomyelopathy in Border Terrier puppies. J Vet Intern Med. PMID: 22269031

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