L-2-hydroxyglutaric Aciduria - L-2-HGA



L-2-HGA is a neurometabolic disorder; it causes progressive deterioration of the central nervous system function. The disease is characterized by elevated levels of L-2-hydroxyglutaric acid in urine, plasma and cerebrospinal fluid. This produces neurological defects including psychomotor retardation, altered behaviour, seizures and ataxia.

Severity - 5

L-2-HGA has a high degree of severity due to the excessive affects on the affected dog. The degeneration of psychomotor skills often results in a decreased quality of life for both you and your dog.


Clinical signs that are usually apparent between 6 months and a year, and can include:
- Epileptic seizures
- ‘Wobbly’ stance/walk
- Tremors
- Muscle stiffness; particularly as a result of exercise or excitement
- Altered behaviour.

Genetic Testing

Animal Network provides a DNA test that determines the genetic predisposition to L-2-HGA.

L-2-Hydroxyglutaric Aciduria is an autosomal recessive disease. A recessive phenotype (trait or disease) will only be expressed when two copies of the recessive gene variant are present. A direct gene test can detect whether a dog is clear, carrier or is affected by the disease.

A dog with two copies of the recessive gene variant is affected, they will express the phenotype and will pass a copy of the gene variant onto their offspring 100% of the time. A dog with one copy of the recessive gene variant is a carrier, they do not express the phenotype themselves, however they are will pass the gene variant onto their offspring 50% of the time. A dog that has does not have any copies of the recessive gene variant is clear, and will never produce affected offspring.

For an explanation of possible genetic status of offspring, please refer to our fact sheet on Breeding strategies. ORDER A TEST

Breeds Affected

Staffordshire Bull Terrier

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