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Multiple system atrophy

Case contributed by Chee Kok Yoon
Diagnosis almost certain

Presentation

This 55 year old presented with a 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.

Patient Data

Age: 55 years
Gender: Female
mri

There is atrophy of the pons with a cruciform T2 hyperintensity in keeping with the hot cross bun sign.

Case Discussion

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. 

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