Spinocerebellar Ataxia, KCNJ10-associated (SAMS)

Spinocerebellar Ataxia (SCA/SAMS) appears to have different variations of symptoms and ages of onset in the Russell group of Terriers. One subtype is associated with symptoms that usually appear between 2-12 months of age, and includes progressive difficulty coordinating fine muscle movements and loss of balance. Myokymia, with sudden muscle contractions or tremors, affects more than half of the dogs in this group; seizures may also be present. For this reason, the disease may also be known as Spinocerebellar Ataxia with Myokymia, Seizures or both (SAMS). Most dogs are humanely euthanized because of poor quality of life.