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Young-onset multiple system atrophy: Clinical and pathological features.

Amit Batla1, Eduardo De Pablo-Fernandez2,3, Roberto Erro1,4

  • 1University College London (UCL) Institute of Neurology, London, UK.

Movement Disorders : Official Journal of the Movement Disorder Society
|August 29, 2018
PubMed
Summary

Young-onset multiple system atrophy (MSA) has distinct clinical and pathological features compared to Parkinson's disease and late-onset MSA. Identifying these differences aids in early diagnosis and understanding of this rare condition.

Keywords:
Multiple system atrophymyoclonusolivopontocerebellar degenerationstriatonigral degeneration

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Area of Science:

  • Neurology
  • Pathology

Background:

  • Young-onset multiple system atrophy (MSA) is defined as onset before age 40.
  • Distinguishing young-onset MSA from young-onset Parkinson's disease (PD) and late-onset MSA is clinically challenging.

Purpose of the Study:

  • To identify clinical and pathological characteristics of young-onset MSA.
  • To differentiate young-onset MSA from young-onset PD and late-onset MSA for improved early diagnosis.

Main Methods:

  • Retrospective review of clinical and pathological data from 22 patients with young-onset MSA.
  • Comparison of clinical features with 16 autopsy-confirmed young-onset PD cases and a large series of late-onset MSA.

Main Results:

  • Young-onset MSA patients (mean age 36.7 years) showed more dystonia, levodopa responsiveness, and levodopa-induced dyskinesia than late-onset MSA.
  • Myoclonus and pyramidal signs were less common in young-onset PD compared to young-onset MSA.
  • The minimal-change pathological variant was more frequent in young-onset MSA.

Conclusions:

  • Clinical and pathological differences identified can improve diagnostic accuracy for young-onset MSA.
  • Findings assist clinicians in the early diagnosis and understanding of young-onset MSA.
  • This research contributes to understanding the pathological basis of early-onset neurodegenerative diseases.