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    SubjectsBiochemistryMaple Syrup Urine Disease — Pathophysiology and Management
    Pre-clinicalBiochemistry

    Maple Syrup Urine Disease — Pathophysiology and Management

    1 MCQs in Biochemistry for NEET PG

    1 Hard
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    Sample Questions

    hard

    A 3-year-old boy with a known diagnosis of maple syrup urine disease (MSUD) is brought to the emergency department with acute encephalopathy, ataxia, and a characteristic sweet-smelling urine. His plasma branched-chain amino acid (BCAA) levels are markedly elevated. Which of the following pathophysiological mechanisms BEST explains the acute neurological deterioration in this patient?

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