Constant and severe involvement of Betz cells in corticobasal degeneration is not consistent with pyramidal signs: A clinicopathological study of ten autopsy cases

Kuniaki Tsuchiya, Shigeo Murayama, Kazuko Mitani, Tatsuro Oda, Kunimasa Arima, Masaru Mimura, Hiroshi Nagura, Chie Haga, Haruhiko Akiyama, Hiroshi Yamanouchi, Hidehiro Mizusawa

Research output: Contribution to journalArticle

28 Citations (Scopus)


This report concerns a clinicopathological study of three additional patients with corticobasal degeneration (CBD), described here for the first time, and a clinicopathological correlation between pyramidal signs and upper motor neuron involvement, in ten autopsy cases of CBD, including seven cases reported by us previously. We investigated pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and involvement of the primary motor cortex and pyramidal tract, focusing on the astrocytosis of the fifth layer of the primary motor cortex. Pyramidal signs were observed in six (60%) of the ten cases. Hyperreflexia was evident in six patients (60%), with spasticity being observed in three patients (30%). Loss of Betz cells associated with prominent astrocytosis and presence of ballooned neurons in the fifth layer of the primary motor cortex was observed in all ten cases. In all cases, involvement of the pyramidal tract was obvious in the medulla oblongata, without involvement of the pyramidal tract in the midbrain. Constant and severe involvement of the fifth layer of the primary motor cortex, including the Betz cells, has not previously been reported in CBD. We suggest that the pyramidal signs in CBD have been disregarded.

Original languageEnglish
Pages (from-to)353-366
Number of pages14
JournalActa Neuropathologica
Issue number4
Publication statusPublished - 2005 Apr 1
Externally publishedYes



  • Betz cells
  • Charcot-Rebeiz disease
  • Corticobasal degeneration
  • Pyramidal signs
  • Pyramidal tract degeneration

ASJC Scopus subject areas

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

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