Which of the Following Statements About Maple Syrup Urine Disease $\alpha$

Which of the following statements about maple syrup urine disease is FALSE?
(I) It is caused by a genetic deficiency in the enzyme BCKDH.
(II) The disease is fatal unless promptly treated.
(III) There is a buildup of the branched-chain $\alpha$ -keto acids.
(IV) Patients with the disease excrete urine that smells like maple syrup.

A) I only
B) II only
C) I,III
D) III,IV
E) All of the statements are true.