Journal Article

DZNE-2026-00317

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Midsagittal Midbrain Area and Midbrain-to-Pons-Ratio Cannot Distinguish Overlap Syndromes Between Amyotrophic Lateral Sclerosis and Progressive Supranuclear Palsy.

Cantré, D. ; König, J. ; Makowsky, C. ; Dyrba, M.DZNE* ; Prudlo, J.Extern*

2026
Urban & Vogel München

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Please use a persistent id in citations: doi:10.1007/s00062-025-01564-x

Abstract: 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.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).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).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.

Keyword(s): Humans (MeSH) ; Amyotrophic Lateral Sclerosis: diagnostic imaging (MeSH) ; Amyotrophic Lateral Sclerosis: pathology (MeSH) ; Supranuclear Palsy, Progressive: diagnostic imaging (MeSH) ; Supranuclear Palsy, Progressive: pathology (MeSH) ; Diagnosis, Differential (MeSH) ; Male (MeSH) ; Female (MeSH) ; Mesencephalon: diagnostic imaging (MeSH) ; Mesencephalon: pathology (MeSH) ; Aged (MeSH) ; Middle Aged (MeSH) ; Magnetic Resonance Imaging: methods (MeSH) ; Pons: diagnostic imaging (MeSH) ; Pons: pathology (MeSH) ; Sensitivity and Specificity (MeSH) ; Amyotrophic lateral sclerosis ; Frontotemporal dementia ; Morphometric MRI ; Progressive supranuclear palsy

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Contributing Institute(s):

1. Clinical Dementia Research (Rostock /Greifswald) (AG Teipel)

Research Program(s):

1. 353 - Clinical and Health Care Research (POF4-353) (POF4-353)

Appears in the scientific report 2026

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Database coverage:
; ; ; Clarivate Analytics Master Journal List ; DEAL Springer ; Essential Science Indicators ; IF < 5 ; JCR ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection

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