Abstract
Background
Multiple system atrophy (MSA) rarely begins before the age of 40 and detailed descriptions
of young-onset MSA are lacking.
Methods
Among 455 patients included in our MSA cohort, four developed disease before the age
of 40. We reviewed the medical records of these patients.
Results
Case 1 and 2 presented with cerebellar symptoms. Case 1 had clinical features and
a course typical of MSA. Case 2 had a rapid course and died 3 years after onset. Case 3 and Case 4 presented with levodopa-responsive parkinsonism.
Both developed motor fluctuations and peak-dose limb dyskinesias. Subthalamic deep
brain stimulation (DBS) resulted in some improvements in motor symptoms, but they
became totally dependent within a few years.
Discussion
Young-onset MSA is rare but does exist. Young-onset MSA with predominant parkinsonism
may closely resemble Parkinson disease at onset and is likely to develop motor complications.
Attention should be given to the possibility of young-onset MSA in selecting DBS candidates.
Keywords
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Article info
Publication history
Published online: May 21, 2012
Accepted:
April 13,
2012
Received in revised form:
April 9,
2012
Received:
October 31,
2011
Identification
Copyright
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
