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Cognitive Development During Adulthood01:30

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Cognitive development continues throughout adulthood, undergoing significant shifts across early, middle, and late stages. Individual transition occurs from adolescent idealism to pragmatic and adaptable thinking in early adulthood. During this period, individuals learn to integrate personal beliefs with the recognition that other perspectives are equally valid. Exposure to the complexities of modern society, diverse experiences, and higher education contribute to this adaptive thought process,...
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Updated: Jun 2, 2025

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Total functioning capacity scale in Huntington's disease: natural course over time.

K F van der Zwaan1, S Feleus2,3, O M Dekkers3

  • 1LUMC Department of Neurology, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands. k.f.van_der_Zwaan@lumc.nl.

Journal of Neurology
|January 15, 2025
PubMed
Summary
This summary is machine-generated.

Huntington's disease (HD) patients show significant functional capacity changes, with many improving TFC scores. Longer CAG repeats correlate with earlier functional decline in HD progression.

Keywords:
Disease progressionFunctional statusHuntington's diseaseMovement disorders

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Area of Science:

  • Neuroscience
  • Genetics
  • Clinical Research

Background:

  • The Total Functioning Capacity (TFC) assessment is crucial for monitoring Huntington's disease (HD) progression in research and clinical settings.
  • Understanding the natural course of functional decline and improvement in HD is essential for effective trial design and patient care.

Purpose of the Study:

  • To investigate the natural progression of functional capacity in individuals with Huntington's disease (HD).
  • To analyze how age and CAG-repeat length influence changes in Total Functioning Capacity (TFC) scores.
  • To evaluate the strengths and limitations of the TFC assessment in longitudinal HD studies.

Main Methods:

  • Analysis of clinical data from the Enroll-HD platform (version 5, including Registry-3) involving 21,079 participants.
  • Focus on 15,527 participants with 52,457 visits and TFC scores ranging from 0 to 13.
  • Examination of TFC score changes over time in relation to age and CAG-repeat length.

Main Results:

  • Most individuals maintained maximum functional capacity; however, 3505 participants experienced TFC score changes.
  • Within 4 years, 64.1% of those with changing scores declined, while 18.6% improved.
  • Age-related functional decline followed a sequence: occupation, finances, daily living, and care needs; longer CAG repeats correlated with earlier decline.

Conclusions:

  • A substantial number of Huntington's disease patients show regained functional capacity, highlighting TFC's utility despite potential interrater variability.
  • The TFC assessment accurately reflects intended changes across functional domains in HD.
  • Analysis of the reduced penetrance group suggests potential biases in healthcare-seeking behavior unrelated to HD progression.