This review examined treatments for specific syndromes associated with ASD and seizures, including genetic syndromes such as TSC and Fragile X and metabolic disorders such as mitochondrial disease and dysfunction, urea cycle disorder, succinic semialdehyde dehydrogenase, branched-chain ketoacid dehydrogenase kinase, creatine and biotinidase deficiency, Smith–Lemli–Opitz syndrome, pyridoxine-dependent and responsive seizures, organic acidemias, and abnormalities of folate and cobalamin metabolism. This evidence concerns the gene BCKDK and inborn organic aciduria.