Gluteric acidemia I (GA I) is an inherited disorder in which the body cannot properly process specific proteins due to defects in the enzyme glutaryl-CoA dehydrogenase (GCDH). GCDH is required to break down certain building blocks of proteins. Accumulation of these products causes damage to the brain and other organs. Build up is more significant during times of stress, such as illness or fasting. Damage to the brain can cause affected children to exhibit delays in motor skills, such as crawling and walking, and intellectual impairment. Other symptoms include unusually large heads, decreased muscle tone, seizures, rigidity, spasms, and sometimes abnormal bleeding in the brain. Symptoms usually appear in infancy or early childhood, sometimes not until adulthood. Life expectancy is generally shortened, but with prompt and appropriate treatment during periods of stress and strict diet management, children with GA I may have completely normal growth and development. GA I is caused by pathogenic variants in the GCDH gene.