Inferior olivary hypertrophy is uncommon in progressive supranuclear palsy

Omi Katsuse, Dennis W. Dickson

Research output: Contribution to journalArticlepeer-review

14 Scopus citations

Abstract

Inferior olivary hypertrophy (IOH) is an uncommon disorder attributed to transsynaptic degeneration and characterized clinically by palatal myoclonus and histopathologically by swollen, fenestrated neurons and bizarre astrocytic gliosis. IOH is usually associated with the interruption of the dentato-olivary tract, most often from cerebrovascular lesions in the dentate nucleus or red nucleus. In progressive supranuclear palsy (PSP), the dentato-olivary tract is consistently affected, and there are reports suggesting that IOH may be relatively common in PSP. To address this issue, the frequency of IOH was investigated in 264 PSP brains. To determine if there was a relationship between IOH and neurofibrillary degeneration in the dentato-olivary pathway, the severity of neurofibrillary degeneration was assessed in the inferior olivary nucleus, cerebellar dentate nucleus and red nucleus in PSP cases with and without IOH. IOH was uncommon in PSP, being found in only 4 of 264 (1.5%) brains, which was not significantly different from the frequency in non-PSP controls (8 of 862; 0.9%). In all non-PSP cases IOH was associated with cerebrovascular lesions, but not in any of the PSP cases with IOH. The severity of neurofibrillary degeneration in the inferior olivary nucleus was significantly greater in PSP cases with IOH than in PSP cases without IOH, whereas there were no significant differences in the cerebellar dentate nucleus and red nucleus. These findings suggest that direct neurofibrillary degeneration in the inferior olivary nucleus may be related to IOH in PSP rather than transsynaptic degeneration.

Original languageEnglish (US)
Pages (from-to)143-146
Number of pages4
JournalActa neuropathologica
Volume108
Issue number2
DOIs
StatePublished - Aug 2004

Keywords

  • Dentato-olivary tract
  • Inferior olivary hypertrophy
  • Neurofibrillary degeneration
  • Progressive supranuclear palsy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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