L-2- hydroxyglutaric aciduria (L-2-HGA) is an inherited neurometabolic disorder affecting dogs. Affected dogs have a mutation in the enzyme that breaks down L-2-hydroxyglutaric acid resulting in increased levels of L-2-hydroxyglutaric acid in urine, blood and cerebrospinal fluid and progressive damage to the brain. Affected dogs typically present between 4 months and one year of age with symptoms of neurologic disease including “wobbly” gait, tremors, seizures, muscle stiffness with exercise or excitement and changes in behavior. In some cases, affected dogs do not present with disease until later in life.

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