Rapidly progressive familial parkinsonism with central hypoventilation, depression and weight loss (Perry syndrome)-A literature review

Christian Wider, Zbigniew K. Wszolek

Research output: Contribution to journalReview article

45 Scopus citations

Abstract

Autosomal dominant parkinsonism, hypoventilation, depression and weight loss (Perry syndrome) has been reported in only seven families worldwide. It is a rapidly progressive disease leading to death from respiratory insufficiency within a few years. Parkinsonism is usually mild, with bradykinesia, rigidity, rest and postural tremor, and axial signs. Response to levodopa is poor although transient response has been occasionally observed. The early signs include parkinsonism, depression and weight loss, whereas hypoventilation is a late feature. Neuropathology shows severe neuronal loss in the substantia nigra, less prominent neuronal loss in the locus coeruleus, and no or few Lewy bodies. In this review, we also propose diagnostic criteria for this condition.

Original languageEnglish (US)
Pages (from-to)1-7
Number of pages7
JournalParkinsonism and Related Disorders
Volume14
Issue number1
DOIs
StatePublished - Jan 1 2008

Keywords

  • Depression
  • Familial
  • Genetic
  • Hypoventilation
  • Parkinsonism
  • Perry
  • Weight loss

ASJC Scopus subject areas

  • Neurology
  • Geriatrics and Gerontology
  • Clinical Neurology

Fingerprint Dive into the research topics of 'Rapidly progressive familial parkinsonism with central hypoventilation, depression and weight loss (Perry syndrome)-A literature review'. Together they form a unique fingerprint.

  • Cite this