Neurologic

This is a Brain and Spinal Cord condition.

L-2-Hydroxyglutaricaciduria, L2HGA

What is L2HGA?

Currently there is no definitive treatment for L2HGA; rather, the condition is managed with anti-seizure and anti-anxiety medications. A rare metabolic disorder, this can cause seizures and behavior changes in affected dogs and usually requires medical management of these symptoms. Behavior changes can range from loss of trained behavior to hyperactivity to increased aggression, while seizures can vary in severity and duration. Affected dogs can experience seizures, behavior changes, or both. Definitive diagnosis of L2HGA involves testing blood, urine, cerebrospinal fluid, and/or other fluids and tissues for elevated levels of L-2-hydroxyglutaric acid. L2HGA was first identified in Staffordshire Bull Terriers and so is also known as "Staffy Cramp;" affected Staffordshire Bull Terriers present as early as 4 months and as old as 6 years with progressive neurologic symptoms. 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.

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