L-2-Hydroxyglutaricaciduria, L2HGA

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.

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

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

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

Talk to your vet about your dog’s L2HGA result so you can plan ongoing care and discuss any medication that may help manage symptoms.

Watch for changes such as unsteady movement, tremors, seizures, or behavioral differences, and contact your vet if you notice any of these.

If your dog shows confusion, disorientation, or aggression, supervise interactions carefully and ensure your dog is in a calm, secure environment.

Keep your dog’s routine predictable and their surroundings quiet to help reduce stress and make them feel more comfortable at home.

L2HGA was first identified in Staffordshire Bull Terriers and so is also known as "Staffy Cramp."

Penetrance: This disease is inherited in an autosomal recessive manner, meaning that affected dogs must have two copies of the mutation to show clinical signs.

Gene

L2HGDH

Inheritance type

recessive

Clinical category

Neurologic

Citations

1. Penderis et al 2007

• American Bully
• American Pit Bull Terrier
• American Staffordshire Terrier
• Alapaha Blue Blood Bulldog
• Olde English Bulldogge
• Staffordshire Bull Terrier
• Staffordshire Terrier