A mixture of alpha-keto acids, the most important of which has been identified as alpha-ketoisocaproic acid, was found to be excreted in large quantities in the urine of beta-methyl-n-valeric acid were present in smaller amounts.

Their excretion would suggest an impairment of the degradative metabolism of the branched-chain amino acids as an important cause of the cerebral degeneration in this condition.

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