Acta Neuropathologica

, Volume 106, Issue 3, pp 251–260 | Cite as

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

  • Omi Katsuse
  • Eizo IsekiEmail author
  • Tetsuaki Arai
  • Haruhiko Akiyama
  • Takashi Togo
  • Hirotake Uchikado
  • Masanori Kato
  • Rohan de Silva
  • Andrew Lees
  • Kenji Kosaka
Regular Paper


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.


4-repeat tauopathy Corticobasal degeneration Progressive supranuclear palsy Astrocytic plaque Tufted astrocyte 



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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Copyright information

© Springer-Verlag 2003

Authors and Affiliations

  • Omi Katsuse
    • 1
  • Eizo Iseki
    • 1
    Email author
  • Tetsuaki Arai
    • 2
  • Haruhiko Akiyama
    • 2
  • Takashi Togo
    • 1
  • Hirotake Uchikado
    • 1
  • Masanori Kato
    • 3
  • Rohan de Silva
    • 4
  • Andrew Lees
    • 4
  • Kenji Kosaka
    • 1
  1. 1.Department of PsychiatryYokohama City University School of MedicineYokohamaJapan
  2. 2.Department of PsychogeriatricsTokyo Institute of PsychiatryTokyoJapan
  3. 3.Soga HospitalOdawaraJapan
  4. 4.Reta Lila Weston Institute of Neurological StudiesUniversity College LondonLondonUK

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