Constant involvement of the Betz cells and pyramidal tract in amyotrophic lateral sclerosis with dementia: A clinicopathological study of eight autopsy cases

K. Tsuchiya, K. Ikeda, M. Mimura, M. Takahashi, H. Miyazaki, M. Anno, H. Shiotsu, H. Akabane, K. Niizato, T. Uchihara, I. Tominaga, I. Nakano

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36 Citations (Scopus)

Abstract

We investigated clinicopathologically pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and the involvement of the primary motor cortex and pyramidal tract, in eight Japanese autopsy cases of amyotrophic lateral sclerosis (ALS) with dementia. Pyramidal signs were observed in seven (88%) of the eight autopsy cases. Hyperreflexia and Babinski sign were evident in seven (88%) and three (38%) patients, respectively, but spasticity was not observed in any of the eight patients. Loss of Betz cells in the primary motor cortex was evident in the seven cases in which this structure was examined. Astrocytosis in the fifth layer of the primary motor cortex was noticed in three cases. In all eight cases, involvement of the pyramidal tract was obvious in the medulla oblongata, but no involvement of the pyramidal tract was found in the midbrain. Involvement of the pyramidal tract in the spinal cord, particularly of large myelinated fibers, was observed in all six cases in which the spinal cord was examined. In ALS with dementia, pyramidal signs were shown to be present more frequently than previously believed, and the clinicopathological correlation between pyramidal signs and involvement of the pyramidal tract was obvious. Constant involvement of Betz cells and the pyramidal tract in ALS with dementia has not been reported. Our clinicopathological findings may make a contribution to the understanding of the clinicopathological hallmarks of this disorder. Furthermore, we believe that this study will also contribute to the elucidation of the nosological status of ALS with dementia.

Original languageEnglish
Pages (from-to)249-259
Number of pages11
JournalActa Neuropathologica
Volume104
Issue number3
Publication statusPublished - 2002 Dec 1
Externally publishedYes

Keywords

  • Amyotrophic lateral sclerosis
  • Betz cells
  • Degeneration
  • Dementia
  • Pyramidal signs
  • Pyramidal tract

ASJC Scopus subject areas

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

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    Tsuchiya, K., Ikeda, K., Mimura, M., Takahashi, M., Miyazaki, H., Anno, M., Shiotsu, H., Akabane, H., Niizato, K., Uchihara, T., Tominaga, I., & Nakano, I. (2002). Constant involvement of the Betz cells and pyramidal tract in amyotrophic lateral sclerosis with dementia: A clinicopathological study of eight autopsy cases. Acta Neuropathologica, 104(3), 249-259.