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Purpose <p>When amyotrophic lateral sclerosis (ALS), a&#xa0;TDP-43 proteinopathy, and progressive supranuclear palsy (PSP), a&#xa0;tauopathy, are associated with frontotemporal dementia (ALS-FTD or PSP-FTD), clinical differentiation can be challenging. There are no established imaging biomarkers to differentiate ALS-FTD from PSP-FTD.</p> Methods <p>We evaluated the midsagittal midbrain area (MBA) and the midbrain-to-pons-(MB/P)-ratios in T1 MPRAGE MRI of 36&#xa0;PSP cases (<i>n</i> = 14&#xa0;PSP-FTD), 77&#xa0;ALS cases (<i>n</i> = 10&#xa0;ALS-FTD), and 72&#xa0;healthy controls (HC).</p> Results <p>In ALS, both parameters were indistinguishable from HC. Patients with ALS-FTD had low MBA-values and MB/P-ratios not significantly different from cases of PSP. While ROC-analyses provided an excellent diagnostic accuracy of both parameters for differentiating PSP from HC (AUC<sub>MBA</sub> = 0.974) as well as PSP from ALS (AUC<sub>MBA</sub> = 0.982), midbrain morphometry provided poor diagnostic accuracy for distinguishing ALS-FTD from PSP-FTD (AUC<sub>MBA</sub> = 0,614).</p> Conclusion <p>The MBA and the MB/P-ratio are morphometric parameters that have proven reliable in atypical Parkinsonian syndromes. Both can distinguish between PSP and ALS in their typical clinical forms. However, they cannot differentiate between PSP-FTD and ALS-FTD.</p>

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Midsagittal Midbrain Area and Midbrain-to-Pons-Ratio Cannot Distinguish Overlap Syndromes Between Amyotrophic Lateral Sclerosis and Progressive Supranuclear Palsy

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年9月12日

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  • Daniel Cantré,
  • Jochem König,
  • Caroline Makowsky,
  • Martin Dyrba,
  • Johannes Prudlo

摘要

Purpose

When amyotrophic lateral sclerosis (ALS), a TDP-43 proteinopathy, and progressive supranuclear palsy (PSP), a tauopathy, are associated with frontotemporal dementia (ALS-FTD or PSP-FTD), clinical differentiation can be challenging. There are no established imaging biomarkers to differentiate ALS-FTD from PSP-FTD.

Methods

We evaluated the midsagittal midbrain area (MBA) and the midbrain-to-pons-(MB/P)-ratios in T1 MPRAGE MRI of 36 PSP cases (n = 14 PSP-FTD), 77 ALS cases (n = 10 ALS-FTD), and 72 healthy controls (HC).

Results

In ALS, both parameters were indistinguishable from HC. Patients with ALS-FTD had low MBA-values and MB/P-ratios not significantly different from cases of PSP. While ROC-analyses provided an excellent diagnostic accuracy of both parameters for differentiating PSP from HC (AUCMBA = 0.974) as well as PSP from ALS (AUCMBA = 0.982), midbrain morphometry provided poor diagnostic accuracy for distinguishing ALS-FTD from PSP-FTD (AUCMBA = 0,614).

Conclusion

The MBA and the MB/P-ratio are morphometric parameters that have proven reliable in atypical Parkinsonian syndromes. Both can distinguish between PSP and ALS in their typical clinical forms. However, they cannot differentiate between PSP-FTD and ALS-FTD.