Abstract
Maple syrup urine disease (MSUD) is a rare autosomal recessive metabolic disorder with neonatal-onset feeding difficulties, movement disorder, encephalopathy and seizures. If not treated in a timely manner, fatal progressive neurological deterioration and respiratory failure ensue [1,2]. This serious condition is caused by various degrees of deficiency in the branched-chain alpha-ketoacid dehydrogenase leading to the accumulation of the branched-chain amino acids, leucine, isoleucine, and valine and their toxic by-products in the brain.
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