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Young onset Parkinson's disease.

N Quinn1, P Critchley, C D Marsden

  • 1University Department of Neurology, Institute of Psychiatry, London, England.

Movement Disorders : Official Journal of the Movement Disorder Society
|January 1, 1987
PubMed
Summary

Young onset Parkinson's disease (YOPD) is proposed for patients aged 21-40. While levodopa is effective, early dyskinesias are common. Juvenile parkinsonism (onset <21) is invariably familial and likely genetic.

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Area of Science:

  • Neurology
  • Neurodegenerative Diseases
  • Movement Disorders

Background:

  • Parkinsonism onset under 40 is rare.
  • Secondary causes like Wilson's disease and encephalitis were excluded.
  • Two distinct groups emerged: onset >21 years (idiopathic) and onset <21 years (familial).

Purpose of the Study:

  • To characterize parkinsonism with onset under 40 years.
  • To differentiate between idiopathic and familial forms.
  • To evaluate treatment response and disease progression in young onset Parkinson's disease.

Main Methods:

  • Analysis of a personal series of 60 parkinsonism cases with onset <40 years.
  • Exclusion of known secondary causes.
  • Classification into two groups based on age of onset and hereditary factors.
  • Assessment of associations with other disorders and response to levodopa therapy.

Main Results:

  • Young onset Parkinson's disease (YOPD, onset 21-40) showed no association with hypertension, diabetes, or thyroid disorders.
  • Twenty percent of YOPD patients had a family history of parkinsonism, but prevalence in relatives was similar to the general population.
  • YOPD patients responded well to levodopa, but experienced early and frequent dyskinesias and response fluctuations. Dementia was negligible in patients <65 years.
  • Juvenile parkinsonism (onset <21) was invariably familial, suggesting a genetic basis. These patients did not show dementia, and CT scans were normal.

Conclusions:

  • Idiopathic Parkinson's disease can present in young adults (21-40 years) and is termed "young onset Parkinson's disease."
  • Juvenile parkinsonism (<21 years) is likely a distinct genetic disorder.
  • Levodopa treatment for YOPD is effective but associated with early motor complications. Most YOPD cases are likely degenerative Lewy body Parkinson's disease.
  • Further research is needed to identify environmental factors potentially accelerating disease onset.

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