title: Young-onset multiple system atrophy.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurology, College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea.
authors.forename: Han-Joon
authors.initials: HJ
authors.lastname: Kim
authors.forename: Beom S
authors.initials: BS
authors.lastname: Jeon
authors.forename: Jee-Young
authors.initials: JY
authors.lastname: Lee
authors.forename: Ji Young
authors.initials: JY
authors.lastname: Yun
authors.forename: Young Eun
authors.initials: YE
authors.lastname: Kim
authors.forename: Sun Ha
authors.initials: SH
authors.lastname: Paek
mesh_terms: D000328:Adult; D017668:Age of Onset; D001921:Brain / Q000473:pathology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000175:diagnosis*