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Short communication| Volume 319, ISSUE 1-2, P168-170, August 15, 2012

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Young-onset multiple system atrophy

  • Han-Joon Kim
    Affiliations
    Department of Neurology, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Department of Movement Disorder Center, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Parkinson Disease Study Group, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Neuroscience Research Institute, BK21, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea
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  • Beom S. Jeon
    Correspondence
    Correspondence to: B.S. Jeon, Department of Neurology, College of Medicine, Seoul National University, 28 Yongon-dong, Chongno-gu, Seoul, 110-744, Republic of Korea. Tel.: +82 2 2072 2876; fax: +82 2 3672 7553.
    Affiliations
    Department of Neurology, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Department of Movement Disorder Center, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Parkinson Disease Study Group, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Neuroscience Research Institute, BK21, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea
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  • Jee-Young Lee
    Affiliations
    Department of Neurology, Seoul National University Boramae Hospital, Seoul, Republic of Korea
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  • Ji Young Yun
    Affiliations
    Department of Neurology, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Department of Movement Disorder Center, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Parkinson Disease Study Group, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Neuroscience Research Institute, BK21, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea
    Search for articles by this author
  • Young Eun Kim
    Affiliations
    Department of Neurology, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Department of Movement Disorder Center, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Parkinson Disease Study Group, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Neuroscience Research Institute, BK21, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea
    Search for articles by this author
  • Sun Ha Paek
    Correspondence
    Correspondence to: S.H. Paek, Department of Neurosurgery, College of Medicine, Seoul National University, 28 Yongon-dong, Chongno-gu, Seoul, 110-744, Republic of Korea. Tel.: +82 2 2072 3993; fax: +82 2 744 8459.
    Affiliations
    Department of Movement Disorder Center, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Neuroscience Research Institute, BK21, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea

    Department of Neurosurgery, BK21, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea
    Search for articles by this author

      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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