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Publication Detail
A Clinicopathologic Study of Movement Disorders in Frontotemporal Lobar Degeneration.
  • Publication Type:
    Journal article
  • Publication Sub Type:
    Article
  • Authors:
    de Pablo-Fernández E, González-Herrero B, Cerdán Santacruz D, Rossor MN, Schott JM, Lashley T, Holton JL, Fox NC, Revesz T, Warren JD, Jaunmuktane Z, Rohrer JD, Warner TT
  • Publication date:
    06/11/2020
  • Journal:
    Mov Disord
  • Status:
    Published
  • Country:
    United States
  • Language:
    eng
  • Keywords:
    corticobasal syndrome, frontotemporal dementia, frontotemporal lobar degeneration, movement disorders, parkinsonism
Abstract
BACKGROUND: Despite the considerable overlap with atypical parkinsonism, a systematic characterization of the movement disorders associated with frontotemporal lobar degeneration (FTLD) is lacking. OBJECTIVE: The aim of this study is to provide a detailed description of the phenomenology and neuropathologic correlations of movement disorders in FTLD. METHODS: In this cohort study, movement disorder clinical data were retrospectively collected from medical records of consecutive patients with a postmortem diagnosis of FTLD from the Queen Square Brain Bank between January 2010 and December 2018. At postmortem, neurodegenerative pathologies were systematically evaluated following consensus criteria. Degeneration of the substantia nigra was assessed as a marker of presynaptic dopaminergic parkinsonism using semiquantitative methods. RESULTS: A total of 55 patients (35 men [64%]) were included with median (interquartile range) age at diagnosis of 58.8 (52.6-63.9) years and a disease duration of 9.6 (6.2-12.9) years. Movement disorders were present in 19 (35%) patients without differences among disease subtypes. The most common syndromes were parkinsonism (9 patients [16%]), usually as an additional late feature, and corticobasal syndrome (CBS, 7 patients [13%]), commonly as a presenting feature. Substantia nigra degeneration was present in 37 (67%) patients although it did not show a good clinical correlation with movement disorders. Those with Pick's disease showed milder substantia nigra degeneration and better response to levodopa. CONCLUSIONS: Movement disorders can present in all FTLD subtypes, more commonly as a late additional feature (parkinsonism) or as a presenting symptom (CBS). The underlying pathophysiology is complex and likely to involve structures outside the presynaptic striatonigral system. © 2020 International Parkinson and Movement Disorder Society.
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UCL Queen Square Institute of Neurology
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