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Clinical outcomes of two main variants of progressive supranuclear palsy and multiple system atrophy: a prospective natural history study

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Abstract

Progressive supranuclear palsy (PSP) and parkinsonian subtype of multiple system atrophy (MSA-P) are, after Parkinson’s disease (PD), the most common forms of neurodegenerative parkinsonism. Clinical heterogeneity of PSP includes two main variants, Richardson syndrome (PSP-RS) and PSP-parkinsonism (PSP-P). Clinical differentiation between them may be impossible at least during the first 2 years of the disease. Little is known about the differences in natural course of PSP-RS and PSP-P and, therefore, in this study we prospectively followed the clinical outcomes of consecutive, pathologically unconfirmed patients with the clinical diagnoses of PSP-RS (51 patients), PSP-P (21 patients) and MSA-P (49 patients). Estimated mean survival time was 11.2 years for PSP-P, 6.8 years for PSP-RS, and 7.9 years for MSA-P, where a 5-year survival probabilities were 90, 66 and 78 %, respectively. More disabling course of PSP-RS compared to PSP-P was also highlighted through the higher number of milestones reached in the first 3 years of the disease, as well as in the trend to reach all clinical milestones earlier. We found that PSP-P variant had a more favorable course with longer survival, not only when compared to PSP-RS, but also when compared to another form of atypical parkinsonism, MSA-P.

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Acknowledgments

This work has been funded by the Ministry of Science, Republic of Serbia (project no. 175090).

Conflicts of interest

MJL, IP and TP declare no conflict of interests. VK has served on the scientific advisory board for Pfizer and has received speaker honoraria from Novartis, Boehringer Ingelheim, Pfizer, Hemofarm-Stada, and GlaxoSmithKline; and receives research support from the Ministry of Science and Technology of the Republic of Serbia (project no. 175090). He also received research grants from Hemopharm-Stada, Pharmaswiss, Boehringer Ingelheim, Lundbeck, Novartis,Glaxo, and Bayer.

Ethical standard

The research presented in this study was approved by local ethics committees, and written informed consent was obtained from each patient prior to their inclusion in this study. The authors declare that they acted in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.

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Authors and Affiliations

  1. Clinic of Neurology, School of Medicine, University of Belgrade, Ul. Dr Subotića 6, 11000, Belgrade, Serbia

    Milica Jecmenica-Lukic, Igor N. Petrovic, Tatjana Pekmezovic & Vladimir S. Kostic

  2. Institute of Epidemiology, School of Medicine, University of Belgrade, Belgrade, Serbia

    Tatjana Pekmezovic

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  1. Milica Jecmenica-Lukic
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  2. Igor N. Petrovic
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  3. Tatjana Pekmezovic
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  4. Vladimir S. Kostic
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Correspondence to Vladimir S. Kostic.

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Jecmenica-Lukic, M., Petrovic, I.N., Pekmezovic, T. et al. Clinical outcomes of two main variants of progressive supranuclear palsy and multiple system atrophy: a prospective natural history study. J Neurol 261, 1575–1583 (2014). https://doi.org/10.1007/s00415-014-7384-x

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  • DOI: https://doi.org/10.1007/s00415-014-7384-x

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