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

I-123 Ioflupane SPECT

Modality: Nuclear medicine

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

Modality: MRI

There is brainstem and cerebellar atrophy. 

MRI follow up 8 months later

Modality: MRI

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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