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.
Similar content being viewed by others
References
Aarsland D, Litvan I, Larsen JP (2001) Neuropsychiatric symptoms of patients with progressive supranuclear palsy and Parkinson’s disease. J Neuropsychiatry Clin Neurosci 13(1):42–49
Agosta F, Kostic VS, Galantucci S, Mesaros S, Svetel M, Pagani E, Stefanova E, Filippi M (2010) The in vivo distribution of brain tissue loss in Richardson’s syndrome and PSP-parkinsonism: a VBM-DARTEL study. Eur J Neurosci 32(4):640–647
Birdi S, Rajput AH, Fenton M, Donat JR, Rozdilsky B, Robinson C, Macaulay R, George D (2002) Progressive supranuclear palsy diagnosis and confounding features: report on 16 autopsied cases. Mov Disord 17(6):1255–1264
Boeve BF, Lang AE, Litvan I (2003) Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol 54(Suppl 5):S15–S19
Brusa A, Mancardi GL, Bugiani O (1980) Progressive supranuclear palsy 1979: an overview. Ital J Neurol Sci 1(4):205–222
Cambier J, Masson M, Viader F, Limodin J, Strube A (1985) Frontal syndrome of progressive supranuclear palsy. Revue neurologique 141(8–9):528–536
Cummings JL, Mega M, Gray K, Rosenberg-Thompson S, Carusi DA, Gornbein J (1994) The Neuropsychiatric Inventory: comprehensive assessment of psychopathology in dementia. Neurology 44(12):2308–2314
Dubois B, Slachevsky A, Litvan I, Pillon B (2000) The FAB: a Frontal Assessment Battery at bedside. Neurology 55(11):1621–1626
Fahn S, Elton RL (1987) Committee MotUD Unified Parkinson’s Disease Rating Scale. In: Fahn S, Marsden CD, Calne DB (eds) Recent developments in Parkinson’s disease, vol 2. MacMillan Health Care Information, New York, pp 153–164
Folstein MF, Folstein SE, McHugh PR (1975) “Mini-mental state”. A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 12(3):189–198
Gibb WR, Lees AJ (1988) The relevance of the Lewy body to the pathogenesis of idiopathic Parkinson’s disease. J Neurol Neurosurg Psychiatry 51(6):745–752
Gilman S, Low PA, Quinn N, Albanese A, Ben-Shlomo Y, Fowler CJ, Kaufmann H, Klockgether T, Lang AE, Lantos PL, Litvan I, Mathias CJ, Oliver E, Robertson D, Schatz I, Wenning GK (1999) Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci 163(1):94–98
Golbe LI, Ohman-Strickland PA (2007) A clinical rating scale for progressive supranuclear palsy. Brain 130(Pt 6):1552–1565
Grafman J, Litvan I, Stark M (1995) Neuropsychological features of progressive supranuclear palsy. Brain Cogn 28(3):311–320
Jellinger KA (2008) Different tau pathology pattern in two clinical phenotypes of progressive supranuclear palsy. Neuro-degener Dis 5(6):339–346
Kaat LD, Boon AJ, Kamphorst W, Ravid R, Duivenvoorden HJ, van Swieten JC (2007) Frontal presentation in progressive supranuclear palsy. Neurology 69(8):723–729
Kluin KJ, Foster NL, Berent S, Gilman S (1993) Perceptual analysis of speech disorders in progressive supranuclear palsy. Neurology 43(3 Pt 1):563–566
Kluin K, Gilman S, Foster N, Sima A, D’Amato C, Bruch L, Bluemlein L, Little R, Johanns J (2001) Neuropathological correlates of dysarthria in progressive supranuclear palsy. Arch Neurol 58(2):265–269
Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC, Goetz CG, Golbe LI, Grafman J, Growdon JH, Hallett M, Jankovic J, Quinn NP, Tolosa E, Zee DS (1996) Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology 47(1):1–9
Litvan I, Bhatia KP, Burn DJ, Goetz CG, Lang AE, McKeith I, Quinn N, Sethi KD, Shults C, Wenning GK (2003) Movement Disorders Society Scientific Issues Committee report: SIC Task Force appraisal of clinical diagnostic criteria for parkinsonian disorders. Mov Disord 18(5):467–486
Maetzler W, Keller S, Michelis J, Koehler N, Stransky E, Becker C, Schulte C, Melms A, Gasser T, Berg D (2009) No differences of butyrylcholinesterase protein activity and allele frequency in Lewy body diseases. Neurobiol Dis 35(2):296–301
Marin RS, Biedrzycki RC, Firinciogullari S (1991) Reliability and validity of the Apathy Evaluation Scale. Psychiatry Res 38(2):143–162
Nath U, Ben-Shlomo Y, Thomson RG, Lees AJ, Burn DJ (2003) Clinical features and natural history of progressive supranuclear palsy: a clinical cohort study. Neurology 60(6):910–916
O’Sullivan SS, Massey LA, Williams DR, Silveira-Moriyama L, Kempster PA, Holton JL, Revesz T, Lees AJ (2008) Clinical outcomes of progressive supranuclear palsy and multiple system atrophy. Brain 131(Pt 5):1362–1372
Oswald W, Fleischmann U (1997) Das Nürnberger Altersinventar. Hogrefe, Göttingen
Richardson JC, Steele J, Olszewski J (1963) Supranuclear Ophthalmoplegia, Pseudobulbar Palsy, Nuchal Dystonia and Dementia. A clinical report on eight cases of “heterogenous system degeneration”. Trans Am Neurol Assoc 88:25–29
Sachin S, Shukla G, Goyal V, Singh S, Aggarwal V, Behari M (2008) Clinical speech impairment in Parkinson’s disease, progressive supranuclear palsy, and multiple system atrophy. Neurology India 56(2):122–126
Schrag A, Ben-Shlomo Y, Quinn NP (1999) Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet 354(9192):1771–1775
Skodda S, Visser W, Schlegel U (2010) Acoustical analysis of speech in progressive supranuclear palsy. J Voice (epub ahead of print)
Williams DR, Lees AJ (2009) Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol 8(3):270–279
Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, Holton JL, Revesz T, Lees AJ (2005) Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Brain 128(Pt 6):1247–1258
Williams DR, Holton JL, Strand C, Pittman A, de Silva R, Lees AJ, Revesz T (2007a) Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson’s syndrome. Brain 130(Pt 6):1566–1576
Williams DR, Holton JL, Strand K, Revesz T, Lees AJ (2007b) Pure akinesia with gait freezing: a third clinical phenotype of progressive supranuclear palsy. Mov Disord 22(15):2235–2241
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.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
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
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00702-010-0563-8