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. 2024 Dec;39(12):2285-2291.
doi: 10.1002/mds.30019. Epub 2024 Sep 20.

Evaluation of Cerebrospinal Fluid α-Synuclein Seed Amplification Assay in Progressive Supranuclear Palsy and Corticobasal Syndrome

David P Vaughan  1   2 , Riona Fumi  1   2 , Marte Theilmann Jensen  1   2 , Megan Hodgson  1   2 , Tatiana Georgiades  1   2 , Lesley Wu  1   2 , Danielle Lux  1   2 , Ruth Obrocki  1   2 , Jennifer Lamoureux  3 , Olaf Ansorge  4 , Kieren S J Allinson  5 , Thomas T Warner  6   7 , Zane Jaunmuktane  6   7 , Anjum Misbahuddin  8 , P Nigel Leigh  9 , Boyd C P Ghosh  10 , Kailash P Bhatia  1   2 , Alistair Church  11 , Christopher Kobylecki  12 , Michele T M Hu  4 , James B Rowe  5 , Cornelis Blauwendraat  13   14 , Huw R Morris  1   2 , Edwin Jabbari  1   2
Affiliations

Evaluation of Cerebrospinal Fluid α-Synuclein Seed Amplification Assay in Progressive Supranuclear Palsy and Corticobasal Syndrome

David P Vaughan et al. Mov Disord. 2024 Dec.

Abstract

Background: Seed amplification assay (SAA) testing has been developed as a biomarker for the diagnosis of α-synuclein-related neurodegenerative disorders.

Objective: The objective of this study was to assess the rate of α-synuclein SAA positivity in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) and to analyze clinical and pathological features of SAA-positive and -negative cases.

Methods: A total of 96 cerebrospinal fluid samples from clinically diagnosed PSP (n = 59) and CBS (n = 37) cases were analyzed using α-synuclein SAA.

Results: Six of 59 (10.2%) PSP cases were α-synuclein SAA positive, including one case who was MSA-type positive. An exploratory analysis showed that PSP cases who were Parkinson's disease-type positive were older and had a shorter disease duration compared with SAA-negative cases. In contrast, 11 of 37 (29.7%) CBS cases were α-synuclein SAA positive, including two cases who were MSA-type positive.

Conclusions: Our results suggest that α-synuclein seeds can be detected in PSP and CBS using a cerebrospinal fluid α-synuclein SAA, and in PSP this may impact on clinical course.

Keywords: biomarker; diagnosis; neuropathology; synucleinopathies; tauopathies.

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