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Pathological Validation of the MDS Criteria for the Diagnosis of Multiple System Atrophy
Sasivimol Virameteekul1,2, Tamas Revesz1, Zane Jaunmuktane1
1Queen Square Brain Bank for Neurological Disorders, UCL Queen Square Institute of Neurology, London, United Kingdom.
Background:
The recent International Parkinson and Movement Disorder Society diagnostic criteria for multiple system atrophy (MDS-MSA) have been developed to improve diagnostic accuracy although their diagnostic properties have not been evaluated.
Objectives:
The aims were to validate the MDS-MSA diagnostic criteria against neuropathological diagnosis and compare their diagnostic performance to previous criteria and diagnosis in clinical practice.
Methods:
Consecutive patients with sporadic, progressive, adult-onset parkinsonism, or cerebellar ataxia from the Queen Square Brain Bank between 2009 and 2019 were selected and divided based on neuropathological diagnosis into MSA and non-MSA. Medical records were systematically reviewed, and clinical diagnosis was documented by retrospectively applying the MDS-MSA criteria, second consensus criteria, and diagnosis according to treating clinicians at early (within 3 years of symptom onset) and final stages. Diagnostic parameters (sensitivity, specificity, positive/negative predictive value, and accuracy) were calculated using neuropathological diagnosis as gold standard and compared between different criteria.
Results:
Three hundred eighteen patients (103 MSA and 215 non-MSA) were included, comprising 248 patients with parkinsonism and 70 with cerebellar ataxia. Clinically probable MDS-MSA showed excellent sensitivity (95.1%), specificity (94.0%), and accuracy (94.3%), although their sensitivity at early stages was modest (62.1%). Clinically probable MDS-MSA outperformed diagnosis by clinicians and by second consensus criteria. Clinically established MDS-MSA showed perfect specificity (100%) even at early stages although to the detriment of low sensitivity. MDS-MSA diagnostic accuracy did not differ according to clinical presentation (ataxia vs. parkinsonism).
Conclusions:
MDS-MSA criteria demonstrated excellent diagnostic performance against neuropathological diagnosis and are useful diagnostic tools for clinical practice and research. © 2023 International Parkinson and Movement Disorder Society.
Insights
The new Multiple System Atrophy (MSA) diagnostic criteria show high accuracy in identifying MSA against neuropathology. These criteria are valuable tools for clinical practice and research, though early-stage sensitivity varies.
Area of Science:
- Neurology
- Neuroscience
- Clinical Diagnostics
Background:
- The International Parkinson and Movement Disorder Society (MDS) developed new diagnostic criteria for multiple system atrophy (MSA) to enhance accuracy.
- The diagnostic properties of these recent MDS-MSA criteria require thorough evaluation.
Purpose of the Study:
- To validate the MDS-MSA diagnostic criteria by comparing them to neuropathological diagnoses.
- To assess the diagnostic performance of MDS-MSA criteria against prior criteria and clinical practice diagnoses.
Main Methods:
- Retrospective review of medical records for patients with parkinsonism or cerebellar ataxia (2009-2019).
- Application of MDS-MSA, second consensus, and clinician diagnoses at early and final stages.
- Calculation and comparison of diagnostic parameters (sensitivity, specificity, accuracy) using neuropathological diagnosis as the gold standard.
Main Results:
- Clinically probable MDS-MSA demonstrated high sensitivity (95.1%), specificity (94.0%), and accuracy (94.3%).
- MDS-MSA criteria outperformed previous consensus criteria and clinician diagnoses.
- Early-stage sensitivity for probable MDS-MSA was modest (62.1%), while established MDS-MSA had perfect specificity (100%) but lower sensitivity.
Conclusions:
- The MDS-MSA criteria exhibit excellent diagnostic performance when validated against neuropathological findings.
- These criteria serve as effective tools for both clinical practice and research in multiple system atrophy.
- Diagnostic accuracy was consistent across different clinical presentations (ataxia vs. parkinsonism).
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