Alaskan Husky encephalopathy is an inherited neurological disease affecting Alaskan Huskies. Affected dogs are unable to produce a protein important in transporting the essential nutrient, thiamine (vitamin B1) into cells of the body and typically present with neurological symptoms between 6 months and 2 years of age. Cells of the nervous system are particularly dependent upon thiamine for the nutrient’s role in energy metabolism. As seen in this disorder, severe thiamine deficiency leads to death of neurons in the brain and subsequent neurological disease. Symptoms include weakness in limbs, altered mental state, loss of balance, abnormal gait, decreased facial pain sensitivity, difficulties eating or swallowing, seizures and blindness. The progression of disease is variable with some dogs going through periods of static disease progression, but most dogs die due to disease complications or humane euthanasia within several months of initial clinical presentation.

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