Multiple system atrophy
55 year old Malay woman presented with 2 year history of rapidly progressing dementia, parkinsonian features, and later pyramidal signs. Cerebellar involvement with ataxia and broad-base gait. No autonomic instability and unsure of any new incontinence (already with continence aids). She was sensitive to dopamine agonist and low dose atypical antipsychotics. Currently bed bound.
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There is atrophy of the pons with a cruciform T2 hyperintensity in keeping with the hot cross bun sign.
In most cases, provided the patient has been referred appropriately, the diagnosis of MSA-C is already suspected by the time such prominent pontine changes are seen.