National Reyes Syndrome Foundation UK

Second level navigation header

Inherited Metabolic Disorders Which Can Simulate Reye's Syndrome

Defects in Metabolism of Branched Chain Amino Acids

Disorders of Branched Chain Amino Acids
(valine, leucine, isoleucine & related organic acidaemias)

  • 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency (3)

  • Isovaleric Acidaemia (IVA)

  • Maple Syrup Disease
       also known as:
       Maple Syrup Urine Disease(MSUD)

  • 3-Methylglutaconic Aciduria
    see note iii
  • Methylmalonic Acidaemia

  • Mitochondrial Acetoacetyl-CoA Thiolase Deficiency
       also known as:
       Beta-Ketothiolase Deficiency

  • Multiple Carboxylase Deficiency (MCD)

  • Propionic Acidaemia

Note iii

Methylglutaconic Aciduria (3) is listed on the CLIMB website individually under types 1, 2, 3 and 4. 

Next review January 2020