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Introduction <p>This study aimed to identify neuropsychiatric, motor, and cognitive features associated with accelerated disease progression in behavioral variant frontotemporal dementia (bvFTD).</p> Methods <p>192 participants were classified as having mild or moderate disease severity based on CDR + NACC FTLD global scores, with scores of 0.5 or 1.0 defined as mild (n = 106) and 2.0 as moderate (n = 86). Participants were further categorized as having accelerated (mild: n = 35; moderate: n = 32) or non-accelerated (mild: n = 71; moderate: n = 54) progression rates based on change in CDR + NACC FTLD-SB sum of boxes (SB) scores (≥ 3.5 points) between Visit 1 and 2. Random forest modeling and logistic regression identified features most predictive of accelerated progression within each group.</p> Results <p>In mild bvFTD, episodic memory impairment and presence of frontal-behavioral neuropsychiatric symptoms were predictive of accelerated progression, whereas in moderate bvFTD, language impairments and motor signs were the strongest predictors. Identified features improved prediction of accelerated progression beyond demographic and clinical factors in mild (∆R2 = .22, p &lt; 0.001) and moderate bvFTD (∆R2 = 0.11, p = 0.08) but did not achieve statistical significance in the moderate group.</p> Discussion <p>Distinct clinical profiles predict accelerated progression in mild versus moderate bvFTD, underscoring the importance of stage-specific clinical markers for prognosis and care.</p>

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Factors influencing accelerated progression in behavioral variant frontotemporal dementia

  • 期刊论文

  • 发表日期：

    2026年4月30日

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  • Molly Split,
  • Zachary J. Kunicki,
  • Daliah Ross,
  • Rachel Keszycki,
  • Sarah Prieto,
  • Masood Manoochehri,
  • Alyssa N. De Vito,
  • Brian Appleby,
  • Chadwick M. Hales,
  • Chiadi Onyike,
  • Corey T. McMillan,
  • David Clark,
  • David Irwin,
  • David Knopman,
  • Douglas R. Galasko,
  • Dylan Wint,
  • Erik D. Roberson,
  • Gabriel C. Léger,
  • Ging-Yuek Robin Hsiung,
  • Hilary W. Heuer,
  • Howard J. Rosen,
  • Ian M. Grant,
  • Ian R. Mackenzie,
  • Irene Litvan,
  • John Kornak,
  • Justin Kwan,
  • Katya Rascovsky,
  • Kimiko Domoto-Reilly,
  • Lauren Massimo,
  • Lawrence S. Honig,
  • Leah K. Forsberg,
  • M. Carmela Tartaglia,
  • Mario F. Mendez,
  • Neill Graff-Radford,
  • Nupur Ghoshal,
  • Peter Ljubenkov,
  • R. Ryan Darby,
  • Sami Barmada,
  • Sandra Weintraub,
  • Suzee Lee,
  • A. Campbell Sullivan,
  • Adam Boxer,
  • Adam M. Staffaroni,
  • Allison Snyder,
  • Andrea Bozoki,
  • Belen Pascual,
  • Bradford C. Dickerson,
  • Bradley F. Boeve,
  • Edward D. Huey,
  • Megan Barker

摘要

Introduction

This study aimed to identify neuropsychiatric, motor, and cognitive features associated with accelerated disease progression in behavioral variant frontotemporal dementia (bvFTD).

Methods

192 participants were classified as having mild or moderate disease severity based on CDR + NACC FTLD global scores, with scores of 0.5 or 1.0 defined as mild (n = 106) and 2.0 as moderate (n = 86). Participants were further categorized as having accelerated (mild: n = 35; moderate: n = 32) or non-accelerated (mild: n = 71; moderate: n = 54) progression rates based on change in CDR + NACC FTLD-SB sum of boxes (SB) scores (≥ 3.5 points) between Visit 1 and 2. Random forest modeling and logistic regression identified features most predictive of accelerated progression within each group.

Results

In mild bvFTD, episodic memory impairment and presence of frontal-behavioral neuropsychiatric symptoms were predictive of accelerated progression, whereas in moderate bvFTD, language impairments and motor signs were the strongest predictors. Identified features improved prediction of accelerated progression beyond demographic and clinical factors in mild (∆R2 = .22, p < 0.001) and moderate bvFTD (∆R2 = 0.11, p = 0.08) but did not achieve statistical significance in the moderate group.

Discussion

Distinct clinical profiles predict accelerated progression in mild versus moderate bvFTD, underscoring the importance of stage-specific clinical markers for prognosis and care.