Multiple system atrophy (MSA-C)

Case contributed by Frank Gaillard
Diagnosis almost certain

Presentation

3 years of autonomic dysfunction (labile BP, bladder dysfunction) parkinsonism and cerebellar signs.

Patient Data

Age: 75 years
Gender: Female

Multiple FLAIR hyperintensities are seen throughout the supratentorial brain, confluent around the lateral ventricles. The SWI sequence shows innumerable punctate foci of previous hemorrhage in both the supra- and infratentorial brain, more pronounced in the basal ganglia and pons.

No abnormal diffusion restriction is seen. Atrophy of the brainstem and cerebellar hemispheres is more pronounced than that affecting the supratentorial brain; there is a "hot cross bun sign". MR Perfusion suggests generalized decreased cerebral blood flow, with possibly only relative sparing of the right peri-Sylvian region.

Conclusion:

1. Features of multiple system atrophy

2. Multiple cerebral microhemorrhage are predominantly in the basal ganglia and pons, and thus more likely related to hypertension rather than amyloid angiopathy.

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