Clinically affected dogs are often young (3 - 18 months
of age) and present with poor weight gain, progressive peripheral neuropathy including ataxia, leg crossing, tremors and paresis which
worsens with exercise.Behavioral changes, blindness, dementia, anorexia, cachexia, urinary incontinence and
muscle weakness affecting all four limbs are usually present in terminal stages
of the disease with death occurring 2 - 6 months after onset
of clinical signs, necessitating euthanasia.