Multiple system atrophy

Case contributed by Dr Jan Frank Gerstenmaier

Presentation

This patient initially presented with tremor, and now has progressive cognitive decline

Patient Data

Age: 60
Gender: Male
Nuclear medicine

I-123 Ioflupane SPECT

Uptake of I-123 Ioflupane is absent in the putamen of both hemispheres and greatly reduced in both caudate nuclei, more marked on the right.

MRI

MRI

There is brainstem and cerebellar atrophy. 

MRI

MRI follow up 8 months later

There has been a rapid progression of brainstem and cerebellar atrophy in only 8 months. Hot cross bun sign is particularly evident on the T2.

Case Discussion

In this case, signs and symptoms of both striatonigral degeneration and olivopontocerebellar atrophy were present. The I-123 Ioflupane scan showed grossly abnormal radiotracer uptake which is a non-specific finding and can be seen in Parkinson disease and well as in a range of "Parkinson-plus" syndromes. Similarly, the hot cross bun sign is non-specific and can be seen in a number of neurodegenerative diseases including vCJD.

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Case information

rID: 23273
Case created: 1st Jun 2013
Last edited: 25th Feb 2016
Inclusion in quiz mode: Included

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