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4-repeat tauopathy sharing pathological and biochemical features of corticobasal degeneration and progressive supranuclear palsy

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Abstract

We report a 67-year-old man with 4-repeat (4R) tauopathy sharing both features of corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). Although CBD and PSP have a common pathological feature that 4R tau accumulates in neurons and glia, recent pathological studies have confirmed differences between the two disorders. Clinical features of the present case were asymmetrical apraxia, parkinsonism, memory disturbance, disorientation and left limb myoclonus with a 5-year history. Pathological features were the widespread occurrence of 4R tau-positive structures including pre-tangles, neurofibrillary tangles, astrocytic plaques, tufted astrocytes, coiled bodies and argyrophilic threads. Biochemically, immunoblotting of insoluble tau demonstrated the low molecular fragments of 37 kDa and 33 kDa observed in typical CBD and PSP, respectively, in addition to the presence of 4R tau isoforms. The present case shared tau-related pathological and biochemical features of CBD and PSP. These findings support that CBD and PSP are closely associated disorders having a pathogenesis common to 4R tauopathy.

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Acknowledgements

This research was supported by Grants-in-Aid from the Japan Ministry of Education, Science, Sports and Culture, and from the Japan Ministry of Health, Labor and Welfare. We are grateful to Dr. P. Davies and Dr. T. Ishii for the generous supply of antibodies. We also thank Dr. M. Goedert for providing tau cDNA.

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Authors and Affiliations

  1. Department of Psychiatry, Yokohama City University School of Medicine, 3-9 Fukuura, Kanazawa-ku, 236-0004, Yokohama, Japan

    Omi Katsuse, Eizo Iseki, Takashi Togo, Hirotake Uchikado & Kenji Kosaka

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

    Tetsuaki Arai & Haruhiko Akiyama

  3. Soga Hospital, Odawara, Japan

    Masanori Kato

  4. Reta Lila Weston Institute of Neurological Studies, University College London, London, UK

    Rohan de Silva & Andrew Lees

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  1. Omi Katsuse
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Correspondence to Eizo Iseki.

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Katsuse, O., Iseki, E., Arai, T. et al. 4-repeat tauopathy sharing pathological and biochemical features of corticobasal degeneration and progressive supranuclear palsy. Acta Neuropathol 106, 251–260 (2003). https://doi.org/10.1007/s00401-003-0728-8

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  • DOI: https://doi.org/10.1007/s00401-003-0728-8

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