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<p>Multiple system atrophy (MSA) is a rare, progressive neurodegenerative disorder with high mortality and diagnostic uncertainty. We re-evaluated 80 cases from the Catalan MSA Registry (CMSAR), applying the 2022 MDS diagnostic criteria and cerebrospinal fluid biomarkers, including α-synuclein seeding assays (asyn-SAA) and neurofilament light chain (NfL). Clinical and biomarker reassessment was performed in 2022–2023. At re-evaluation, 20 patients were alive; 14 were reclassified as non-MSA and three as unclassifiable. MSA cases had shorter survival (8.8 vs 14.8 years, <i>p</i> = 0.001), and dysphagia predicted poorer outcomes. The 2022 criteria showed higher specificity (94% for clinically established, 71% for probable) than the 2008 criteria. NfL levels were significantly higher in MSA (p = 0.005) and predicted mortality. Asyn-SAA was negative in confirmed MSA cases and positive in three suspected Parkinson’s disease cases. These findings support the added diagnostic value of integrating updated criteria and CSF biomarkers to improve MSA classification and long-term monitoring.</p>

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An eight-year follow-up of the CMSAR: assessing how the new MDS criteria and biomarkers impact diagnostic accuracy

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年1月10日

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  • Alexandra Pérez Soriano,
  • Cèlia Painous,
  • Manel Fernandez,
  • Jesica Pérez,
  • Raquel Ruiz-Garcia,
  • Laura Naranjo,
  • Ana Camara,
  • Iban Aldecoa,
  • Laura Molina,
  • Yaroslau Compta,
  • Esteban Muñoz,
  • Maria J. Martí,
  • Javier Pagonabarraga,
  • Francesc Valldeoriola,
  • Jorge Hernández-Vara,
  • Serge Jauma,
  • Victor Puente,
  • Claustre Pont,
  • Núria Caballol,
  • Eduardo Tolosa,
  • Angels Bayes,
  • Jaume Campdelacreu,
  • Oriol de Fàbregues,
  • Asunción Ávila,
  • Matilde Calopa,
  • Pau Pastor,
  • Montserrat Pujol,
  • Carles Gaig,
  • Lluís Planellas,
  • Ana Cámara

摘要

Multiple system atrophy (MSA) is a rare, progressive neurodegenerative disorder with high mortality and diagnostic uncertainty. We re-evaluated 80 cases from the Catalan MSA Registry (CMSAR), applying the 2022 MDS diagnostic criteria and cerebrospinal fluid biomarkers, including α-synuclein seeding assays (asyn-SAA) and neurofilament light chain (NfL). Clinical and biomarker reassessment was performed in 2022–2023. At re-evaluation, 20 patients were alive; 14 were reclassified as non-MSA and three as unclassifiable. MSA cases had shorter survival (8.8 vs 14.8 years, p = 0.001), and dysphagia predicted poorer outcomes. The 2022 criteria showed higher specificity (94% for clinically established, 71% for probable) than the 2008 criteria. NfL levels were significantly higher in MSA (p = 0.005) and predicted mortality. Asyn-SAA was negative in confirmed MSA cases and positive in three suspected Parkinson’s disease cases. These findings support the added diagnostic value of integrating updated criteria and CSF biomarkers to improve MSA classification and long-term monitoring.