Multiple system atrophy

Case contributed by Frank Gaillard
Diagnosis almost certain

Presentation

Progressive dysphasia and bulbar symptoms including dysphagia and dysphonia. Associated recent history of falls and urinary incontinence.

Patient Data

Age: 65 years
Gender: Female

MRI brain

mri

Largely unremarkable although there is relatively prominent loss of volume in the posterior fossa, and some high signal crossing the pons best seen on FLAIR. 

MRI brain - 6 months later

mri

The degree of atrophy has significantly progressed, seen both on midline sagittal and more easily on axial image. A hot cross bun sign is now evident on FLAIR.

Case Discussion

Multiple system atrophy can divided clinically into 2 forms: 

  1. MSA-C : predominance of cerebellar symptoms (olivopontocerebellar atrophy)
  2. MSA-P : predominance of parkinsonian signs and symptoms (striatonigral degeneration)

This case is the former (MSA-C) and striking cerebellar changes are evident. 

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