In patients with organic acidemia and aminoacidopathy (including methyl malonicacidemia, Propionic acidemia, isovalericacidemia, glutaricaciduria I and II, MSUD, NKH, tyrosinemia, galactosemia, biotinidase deficiency, PKU, hemocysteinuria and MTHFR); in 33% of patients, brain imaging was normal; 28.9% of patients had brain atrophy; 24.5% of patients had white matter involvement; the rest of patients had basal ganglia along with other area involvement. The gene discussed is MTHFR; the disease is isovaleric acidemia.