Changes in saccadic intrusions over time as an objective biomarker to follow ALS disease progression

Amyotroph Lateral Scler Frontotemporal Degener. 2024 Nov;25(7-8):760-766. doi: 10.1080/21678421.2024.2376732. Epub 2024 Jul 8.

Abstract

Objective: Saccadic Intrusions (SIs) are abnormal eye movements during gaze fixation. Studies have indicated the clinical relevance of SIs, especially of square wave jerks (SWJ) in ALS. We used a software-based platform to extract SIs as a part of an interventional drug trial. The objective was to examine SIs' change over time as a potential biomarker of ALS disease progression. Methods: 28 ALS patients (61.95 ± 8.6 years) were assessed with the revised ALS Functional Rating Scale (ALSFRS-R) and with an oculometric test. Changes of SIs over time and correlations with ALSFRS-R and its bulbar subscale were calculated. A power calculation was conducted to understand the practical implications of results. Results: A significant increase of SWJ over trial duration was observed, with an increase in frequency (mean rise of 0.14 ± 0.28, p < 0.01), amplitude (0.001 ± 0.0016 degrees, p < 0.005), overall duration of SWJ (0.13 ± 0.25, in %, p < 0.01), and in their relative part out of all intrusions (0.18 ± 0.32, in %, p < 0.005). Negative correlations were found with the bulbar subscale (R=-0.43, -0.41, -0.39 and -0.47, respectively, p < 0.001). The required sample size for observing a 40% reduction in bulbar aspects when using the oculometric test (α = 0.05 and β = 0.8), was found to be 150 patients per arm, compared with 200 patients using the bulbar subscale. Conclusions: Evaluation of saccadic intrusions during fixation was able to detect disease progression over time, correlated with ALSFRS-R bulbar subscale. Eye movements can potentially serve as an objective biomarker in ALS clinical trials and reduce the required sample size to show clinical effect of therapies.

Keywords: Fixation; biomarkers; oculometrics; saccadic intrusions; square wave jerks.

MeSH terms

  • Aged
  • Amyotrophic Lateral Sclerosis* / diagnosis
  • Amyotrophic Lateral Sclerosis* / physiopathology
  • Biomarkers
  • Disease Progression*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Ocular Motility Disorders / diagnosis
  • Ocular Motility Disorders / etiology
  • Ocular Motility Disorders / physiopathology
  • Saccades* / physiology

Substances

  • Biomarkers