Multiple system atrophy (MSA) rarely begins before the age of 40 and detailed descriptions of young-onset MSA are lacking.
Among 455 patients included in our MSA cohort, four developed disease before the age of 40. We reviewed the medical records of these patients.
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.
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.
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- Second consensus statement on the diagnosis of multiple system atrophy.Neurology. 2008; 71: 670-676
- Progression of multiple system atrophy (MSA): a prospective natural history study by the European MSA Study Group (EMSA SG).Mov Disord. 2006; 21: 179-186
- Survival of Korean patients with multiple system atrophy.Mov Disord. 2011; 26: 909-912
- Potential outcome measures and trial design issues for multiple system atrophy.Mov Disord. 2007; 22: 2371-2377
- Survival in multiple system atrophy.Mov Disord. 2008; 23: 294-296
- Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients.Brain. 2002; 125: 1070-1083
- MSA-C is the predominant clinical phenotype of MSA in Japan: analysis of 142 patients with probable MSA.J Neurol Sci. 2006; 249: 115-121
- Clinical outcomes of progressive supranuclear palsy and multiple system atrophy.Brain. 2008; 131: 1362-1372
- Pallidal stimulation reduces treatment induced dyskinesias in “minimal change” multiple system atrophy.Mov Disord. 2005; 20: 1042-1047
- Parkinson's disease like presentation of multiple system atrophy with poor response to STN stimulation: a clinicopathological case report.Mov Disord. 2004; 19: 973-977
- Adverse effects of subthalamic nucleus DBS in a patient with multiple system atrophy.Neurology. 2003; 61: 247-249
- What clinical features are most useful to distinguish definite multiple system atrophy from Parkinson's disease?.J Neurol Neurosurg Psychiatry. 2000; 68: 434-440
Published online: May 21, 2012
Accepted: April 13, 2012
Received in revised form: April 9, 2012
Received: October 31, 2011
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.