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Familial Parkinsonism with Apathy, Depression and Central Hypoventilation (Perry’s Syndrome)

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Part of the Advances in Behavioral Biology book series (ABBI,volume 51)

Abstract

A rare and unique form of familial parkinsonism with autosomal dominant inheritance that is characterized by all cardinal features of parkinsonism, apathy, depression, central hypoventilation, weight loss, and rapid progression was first described by Perry et al.1 Thereafter, four additional families from different ethnic background have been reported (Table 1).2–6 In most of these affected individuals, disease presented between ages of 40–55, with symptoms of apathy, psychomotor slowness and/or depression, usually accompanied or followed by moderate degrees of parkinsonism and weight loss. Central type of hypoventilation developed eventually and caused death in most of the patients due to sudden apnea or complications of respiratory insufficiency. Pathology differs from idiopathic Parkinson’s disease by severe neuronal loss and gliosis in substantia nigra with no or very few Lewy-body formation,1,2,4,5 and in some patients, by extension of neuronal loss to locus ceruleus, caudate, pallidum and medulla. 2’4

Keywords

  • Frontotemporal Dementia
  • Progressive Supranuclear Palsy
  • Locus Ceruleus
  • Familial Parkinsonism
  • Progressive Weight Loss

These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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© 2002 Kluwer Academic / Plenum Publishers, New York

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Elibol, B. et al. (2002). Familial Parkinsonism with Apathy, Depression and Central Hypoventilation (Perry’s Syndrome). In: Mizuno, Y., Fisher, A., Hanin, I. (eds) Mapping the Progress of Alzheimer’s and Parkinson’s Disease. Advances in Behavioral Biology, vol 51. Springer, Boston, MA. https://doi.org/10.1007/978-0-306-47593-1_48

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  • DOI: https://doi.org/10.1007/978-0-306-47593-1_48

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4757-0973-5

  • Online ISBN: 978-0-306-47593-1

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