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

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Abstract

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.

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Acknowledgements

We wish to express our gratitude to former Prof. H. Tsukagoshi (Department of Neurology, Tokyo Medical and Dental University) for his valuable advice. We also wish to thank to Mr. Y. Shoda and Ms E. Matsui for their photographic assistance.

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Authors and Affiliations

  1. Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital, 2-1-1, Kamikitazawa, Setagaya-ku, 156-0057, Tokyo, Japan

    Kuniaki Tsuchiya

  2. Department of Neuropathology, Tokyo Institute of Psychiatry, Tokyo, Japan

    Kuniaki Tsuchiya, Chie Haga & Haruhiko Akiyama

  3. Department of Neurology, Tokyo Medical and Dental University, Tokyo, Japan

    Kuniaki Tsuchiya & Hidehiro Mizusawa

  4. Department of Neuropathology, Tokyo Metropolitan Gerontology, Tokyo, Japan

    Shigeo Murayama

  5. Department of Neurology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan

    Kazuko Mitani, Hiroshi Nagura & Hiroshi Yamanouchi

  6. Department of Psychiatry, National Shimofusa Sanatorium, Chiba, Japan

    Tatsuro Oda

  7. Department of Laboratory Medicine, National Center Hospital for Mental, Nervous and Muscular Disorders, Tokyo, Japan

    Kunimasa Arima

  8. Department of Neuropsychiatry, Showa University School of Medicine, Tokyo, Japan

    Masaru Mimura

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  1. Kuniaki Tsuchiya
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Tsuchiya, K., Murayama, S., Mitani, K. et al. Constant and severe involvement of Betz cells in corticobasal degeneration is not consistent with pyramidal signs: a clinicopathological study of ten autopsy cases. Acta Neuropathol 109, 353–366 (2005). https://doi.org/10.1007/s00401-004-0966-4

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