Category: Parkinsonism, Atypical: MSA
Objective: To develop a clinical score to distinguish between the parkinsonian variant of multiple system atrophy (MSA-P) and Parkinson’s disease (PD).
Background: The differential diagnosis between MSA-P and PD is often difficult, particularly early in the disease course.
Method: We compared patients with probable MSA-P and with PD with a disease duration of <3 years, selected from those who were enrolled in the Natural History Study of the Synucleinopathies, an international prospective observational study. Detailed clinical neurological, and autonomic parameters were assessed at enrollment using UMSARS part I, II and IV; Schrag quality of life (QoL) scale; burden of autonomic dysfunction by COMPASS-31 scale; smell function using the UPSIT; cardiovascular autonomic function using heart rate variability during deep-breathing, analysis of the Valsalva maneuver, orthostatic stress test, plasma catecholamine levels during supine rest and after head-up tilt; and cognitive evaluation using MoCA. Positive and negative likelihood ratios (LR) were obtained for each variable assessed. Multiple iterations of a composite score based on sequential addition of variables with the highest diagnostic accuracy were created by multiplying each variable’s LR and applying a logarithmic function.
Results: Fifty-eight MSA-P and 53 PD patients had a disease duration of less than 3 years. The vast majority of patients had been diagnosed within the last 12 months (81% MSA-P and 66% PD patients). MSA-P patients were more frequently female (53% vs. 30% p<0.05) and younger at diagnosis (63±8 years vs. 71±8 years, p<0.001). A 7-item score comprising the bladder weighted subscore of the COMPASS-31, UMSARS’s part 1, UPSIT, hyperreflexia, the motor subscore of Schrag’s MSA quality of life scale, falls within 3 years of diagnosis, and new or increased snoring resulted in a ROC curve AUC of 0.983, with excellent 93% sensitivity and 98% specificity to distinguish early MSA-P from PD.
Conclusion: We propose a scale of 7 clinical items to distinguish early stage MSA-P from PD. It considers urinary function, olfactory function, corticospinal signs, performance of activities of daily living, motor symptoms burden on quality of life, frequent early falls and sleep disordered breathing. We are now prospectively validating the scale to determine its predictive value in our prodromal cohort.
To cite this abstract in AMA style:P. Millar Vernetti, J.A Palma, L. Norcliffe-Kaufmann, M. Pérez, A. Fanciulli, F. Krismer, W. Singer, P. Low, M.T Pellecchia, H.J Kim, C. Shibao, A. Peltier, I. Biaggioni, M.J Martí, C. Terroba-Chambi, M. Merello, D. Goldstein, R. Freeman, C. Gibbons, S. Vernino, G. Wenning, H. Kaufmann. Towards a scoring system to distinguish early parkinsonian variant of multiple system atrophy from Parkinson’s disease [abstract]. Mov Disord. 2020; 35 (suppl 1). https://www.mdsabstracts.org/abstract/towards-a-scoring-system-to-distinguish-early-parkinsonian-variant-of-multiple-system-atrophy-from-parkinsons-disease/. Accessed December 4, 2023.
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