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In vivo comparison of Richardson’s syndrome and progressive supranuclear palsy-parkinsonism

  • Movement Disorders - Original Article
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Abstract

Richardson’s syndrome (RS) and progressive supranuclear palsy-parkinsonism (PSP-P) are the most common subtypes of PSP. Post-mortem data suggests that the clinical presentation of the two subtypes differs especially in the first 2 years of disease and then converges. This hypothesis has, to our knowledge, never been confirmed in a living cohort. Medical history was used to define subtypes retrospectively in 23 consecutive PSP patients from our outpatient clinic specialized in movement disorders. 14 patients suffered from RS, and 9 from PSP-P. Using a prospective cross-sectional approach, clinical, cognitive, behavioral, speech and biochemical (cerebrospinal fluid tau levels) features were compared. RS patients showed shorter time from disease onset to diagnosis and more neuropsychological and neurobehavioral deficits than PSP-P patients, but differed not significantly with regard to clinical and biochemical features. RS and PSP-P show considerable symptoms overlap during the disease course when using routine assessments, with persisting differences regarding non-motor symptoms. Shorter disease duration of the comparably affected RS patients indicates that this subtype has an accelerated disease progression at early disease stages.

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Acknowledgments

We thank all patients who participated in the study, and Susanne Wagner for reviewing the manuscript. W.M. has been supported by a Forschungskolleg Geriatrie Grant from the Robert Bosch Foundation, Stuttgart, Germany (Nr. 32.5.1141.0019.0). In the previous 12 months, GE received honoraria for consultancies, advisory boards and as a speaker from Axxonis Pharma, Cephalon, Desitin, Boehringer Ingelheim, Glaxo Smith Kline, Valeant, Orion, Solvay und Schwarz Pharma (UCB). DB received honoraria for lectures from UCB, Glaxo Smith Kline, TEVA and Lundbeck and for serving on scientific advisory boards for Novartis, UCB, Glaxo Smith Kline and TEVA. DB has received grants from the Michael J. Fox Foundation, the Bundesministerium für Bildung und Forschung, Janssen Pharmaceuticals, TEVA Pharma GmbH, Solvay and the German Parkinson’s disease Association.

Conflict of interest

All authors report no conflict of interest.

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Authors and Affiliations

  1. Department of Neurodegeneration, Hertie Institute for Clinical Brain Research and German Center for Neurodegenerative Diseases, University of Tuebingen, Hoppe-Seyler-Strasse 3, 72076, Tuebingen, Germany

    Karin Srulijes, Sarah Bauer, Elisabeth Dietzel, Adriane Gröger, Daniela Berg & Walter Maetzler

  2. Movement Disorder Clinic, Beelitz-Heilstaetten, Germany

    Grit Mallien & Georg Ebersbach

  3. Department of Clinical Gerontology, Robert-Bosch-Hospital, Stuttgart, Germany

    Walter Maetzler

Authors
  1. Karin Srulijes
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  2. Grit Mallien
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  3. Sarah Bauer
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  4. Elisabeth Dietzel
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  5. Adriane Gröger
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  6. Georg Ebersbach
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  7. Daniela Berg
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  8. Walter Maetzler
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Correspondence to Karin Srulijes.

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Srulijes, K., Mallien, G., Bauer, S. et al. In vivo comparison of Richardson’s syndrome and progressive supranuclear palsy-parkinsonism. J Neural Transm 118, 1191–1197 (2011). https://doi.org/10.1007/s00702-010-0563-8

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  • DOI: https://doi.org/10.1007/s00702-010-0563-8

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