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Multiple System Atrophy — RACP Adult Medicine MCQ

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ModerateNeurologyMultiple System AtrophyRACP Adult Medicine

A 60-year-old woman presents with parkinsonism, early severe autonomic failure (orthostatic hypotension, urinary incontinence), and cerebellar ataxia. She has inspiratory stridor during sleep. MRI shows the 'hot cross bun' sign in the pons. What is the most likely diagnosis?

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Correct answer: B — Multiple system atrophy

MSA presents with parkinsonism plus early severe autonomic failure and/or cerebellar ataxia. Stridor is a red-flag feature. The hot cross bun sign (cruciform pontine hyperintensity on MRI) is characteristic. MSA-P predominates with parkinsonism; MSA-C with cerebellar features. Levodopa response is typically poor.

Reference: MDS – 2024 – MSA Diagnostic Criteria