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Publication Detail
MAPT-Associated Familial Progressive Supranuclear Palsy with Typical Corticobasal Degeneration Neuropathology: A Clinicopathological Report
  • Publication Type:
    Journal article
  • Authors:
    Cullinane PW, Fumi R, Theilmann Jensen M, Jabbari E, Warner TT, Revesz T, Morris HR, Rohrer JD, Jaunmuktane Z
  • Publisher:
    Wiley
  • Publication date:
    11/03/2023
  • Journal:
    Movement Disorders Clinical Practice
  • Status:
    Accepted
  • Print ISSN:
    2330-1619
  • Language:
    English
  • Keywords:
    MAPT, corticobasal degeneration, progressive supranuclear palsy, pathology
  • Notes:
    © 2023 The Authors. Movement Disorders Clinical Practice published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License (https://creativecommons.org/licenses/by-nc-nd/4.0/), which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
Abstract
Corticobasal degeneration (CBD) is a rare, 4-repeat (4R) tauopathy characterized by astrocytic plaque neuropathology. Although ~25% of sporadic CBD cases present with progressive supranuclear palsy-Richardson's syndrome (PSP-RS),1 only one other case of microtubule-associated protein tau gene (MAPT)- related CBD with a PSP-like phenotype has been reported.2 We aim to highlight important issues regarding the classification of MAPT-associated tauopathies and the implications for clinical research.
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