PATHOLOGY N* Q-7

[Roxanita]

A 7-month-old female infant is brought to the physician's office after having a tonic-clonic seizure. Findings include ataxia, hypotonia, hearing loss, hair loss, and a rash. Laboratory studies show normal plasma amino acid concentrations but abnormal urinary organic acid concentrations consistent with multiple carboxylase deficiency. The administration of which of the following is the most appropriate treatment of this patient?

A) Biotin

B) Vitamin B2 (riboflavin)

C) Vitamin B6 (pyridoxine)

D) Vitamin B12 (cyanocobalamin)

E) Vitamin C

[BlakeH]

Answer is B6, which is needed for the activity of the B6-dependant Glutamate decarboxylase , which leads to the production of GABA from Glutamate.