Spongiform leukoencephalomyelopathy (SLEM)*

A genetic variant has been found to cause a degenerative neurological disease in Border Terriers, which is called spongiform leucoencephalomyelopathy (SLEM) or shaking puppy syndrome. \nDue to changes of the insulating myelin sheaths around the nerve fibres of the white matter of the brain, poor transmission of nerve impulses results in neurological symptoms of incoordination, tremors and seizures. First symptoms usually could be observed at the time when the puppies begin to stand and walk, normally at the age of two weeks. First tremors and shaking are seen in their hind limbs, later the shaking affects the entire body and they show poor coordination. Moreover, affected puppies are reported to be smaller and weigh less than their unaffected littermates.\nHowever, some affected pups do not suffer from incoordination and shaking until 10-12 weeks of age and the severity of the clinical symptoms is variable. Therefore it is suspected that other gene mutations or environmental factors might influence the progression of the myelin defect. \nAt this time there is no known effective therapy for puppies suffering from SLEM and they usually have to be euthanized due to a poor quality of life. \n