Q:

Brown-Vialetto-Van Laere syndrome (BVVLS)

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Brown-Vialetto-Van Laere syndrome (BVVLS), a neurologic disorder characterized by progressive neurologic deterioration, sensorineural hearing loss,and pontobulbar palsy usually responds to treatment with high doses of


  1. niacin
  2. biotin
  3. riboflavin
  4. pyridoxine
  5. thiamin

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(C). Mutations in genes coding for riboflavin transporter proteins have been identified in children with BVVLS.

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