Disorder	Organ or system affected	Signs of metabolic instability	Neurodevelopmental effects (if not treated)
3-hydroxy-3-methyglutaryl-CoA lyase deficiency	CNS	Hypoglycemia	Developmental delay, hypotonia
3-methylcrotonyl-CoA carboxylase deficiency	CNS	Hypoglycemia	Developmental delay, hypotonia; many asymptomatic
Beta-ketothiolase deficiency	CNS	Hypoglycemia	Motor deficits, mental retardation
Glutaric acidemia type I	CNS	Acidosis	Motor deficits, severe dystonia; cognition often intact
Isovaleric acidemia	CNS	Acidosis, hyperammonemia, sweaty feet odor	Developmental delay, motor deficits, mental retardation
Methylmalonic acidemia	CNS	Acidosis, hyperammonemia	Developmental delay, mental retardation, movement disorders
Propionic acidemia	CNS	Acidosis, hyperammonemia	Developmental delay, mental retardation, speech defects