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This is a Brain and Spinal Cord condition.

L-2-Hydroxyglutaricaciduria, L2HGA

What is L2HGA?

A rare metabolic disorder, L2HGDH encodes the enzyme L-2-hydroxyglutarate dehydrogenase, which breaks down L-2-hydroxyglutarate. Buildup of L-2-hydroxyglutarate is known to induce oxidative stress, though the exact mechanism by which it causes the neurologic symptoms of L2HGA is unknown.

What are the signs & symptoms that develop in affected dogs?

Affected dogs can experience seizures, behavior changes, or both. Behavior changes can range from loss of trained behavior to hyperactivity to increased aggression, while seizures can vary in severity and duration.

When do signs and symptoms develop?

Affected Staffordshire Bull Terriers present as early as 4 months and as old as 6 years with progressive neurologic symptoms.

How do vets diagnose this condition?

Definitive diagnosis of L2HGA involves testing blood, urine, cerebrospinal fluid, and/or other fluids and tissues for elevated levels of L-2-hydroxyglutaric acid.

How is this condition treated?

Currently there is no definitive treatment for L2HGA; rather, the condition is managed with anti-seizure and anti-anxiety medications.

What actions should I take if my dog is affected?

  • Please follow your veterinarian's advice and give medication as appropriate. If your dog has evidence of behavioral changes, make sure to take precautions with regard to who can interact with your dog.
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