Brain, Vol. 124, No. 7, 1438-1449,
July 2001
© 2001 Oxford University Press
The prevalence of progressive supranuclear palsy (Steele–Richardson–Olszewski syndrome) in the UK
1 Department of Neurology and 2 Department of Epidemiology and Public Health, School of Health Sciences, University of Newcastle upon Tyne, 3 Department of Social Medicine, Bristol University, 4 Reta Lila Weston Institute of Neurological Sciences and Institute of Neurology and 5 Neurogenetics Department, Institute of Neurology, London, UK
Correspondence to:
Dr D. J. Burn, Consultant and Senior Lecturer in Neurology, Regional Neurosciences Centre, Newcastle General Hospital, Westgate Road, Newcastle upon Tyne NE4 6BE, UK E-mail: d.j.burn{at}ncl.ac.uk
We performed a study to estimate the point prevalence of progressive supranuclear palsy (PSP) in the UK at national, regional and community levels. A `Russian doll' design was used in which the population denominator for each of the three studies was successively smaller, whilst the method of case ascertainment became increasingly more rigorous. The NINDS-SPSP (National Institute of Neurological Disorders and the Society for Progressive Supranuclear Palsy) diagnostic criteria for PSP were applied throughout the study for case definition. The national study identified cases using passive referral mechanisms [e.g. the British Neurological Surveillance Unit (BNSU), PSP (Europe) Association patient register]. We identified 577 cases of PSP, giving a national prevalence estimate of 1.0 per 100 000 [95% confidence interval (CI) 0.9–1.1]. The North of England regional study used active `multiple source' case ascertainment from a collaborative network of neurologists and non-neurologists. We identified 80 cases of PSP in this study, giving a crude and age-adjusted prevalence of 3.1 (95% CI 2.4–3.8) and 2.4 (1.9–3.0) per 100 000, respectively. Of these 80 cases, 51 patients (65%) were referred initially to non-neurologists and 10 patients (13%) had not seen a neurologist at any stage of their illness. The proportion of female cases was significantly greater in the regional than in the national study (61% versus 44%; P < 0.02). Cases referred to non-neurologists were significantly older than those referred to neurologists in the regional study (median age 73 versus 69.5 years; P < 0.01). Patients in the community study were identified via diagnostic and therapeutic registers from a representative sample of general practices in Newcastle upon Tyne. We identified 17 cases of PSP, yielding crude and age-adjusted prevalences of 6.5 (95% CI 3.4–9.7) and 5.0 (95% CI 2.5–7.5) per 100 000, respectively. Seven of the 17 cases (41%) had not previously been diagnosed as PSP. This study suggests that PSP is more common than previously considered, is commonly misdiagnosed and that the majority of cases are not initially referred to neurologists. The study also confirms the importance of active and detailed case ascertainment in ensuring reliable prevalence estimates.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  G. Bensimon, A. Ludolph, Y. Agid, M. Vidailhet, C. Payan, P. N. Leigh, and for the NNIPPS Study Group Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: The NNIPPS Study Brain, January 1, 2009; 132(1): 156 - 171. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Schrag, C. Selai, N. Quinn, A. Lees, I. Litvan, A. Lang, Y. Poon, J. Bower, D. Burn, and J. Hobart Measuring quality of life in PSP: The PSP-QoL. Neurology, July 11, 2006; 67(1): 39 - 44. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. Zampieri and R. P Di Fabio Progressive Supranuclear Palsy: Disease Profile and Rehabilitation Strategies Physical Therapy, June 1, 2006; 86(6): 870 - 880. [Full Text] [PDF]
|
|
|
|
|
|
D. C. Paviour, S. L. Price, M. Jahanshahi, A. J. Lees, and N. C. Fox Longitudinal MRI in progressive supranuclear palsy and multiple system atrophy: rates and regions of atrophy Brain, April 1, 2006; 129(4): 1040 - 1049. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. J. Burn Commentary on: Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism, by D. Williams, R. de Silva, D. Paviour, et al. (Brain-2004-01045.R1) Brain, June 1, 2005; 128(6): 1235 - 1236. [Full Text] [PDF]
|
|
|
|
|
|
N. J. Cordato, A. J. Duggins, G. M. Halliday, J. G. L. Morris, and C. Pantelis Clinical deficits correlate with regional cerebral atrophy in progressive supranuclear palsy Brain, June 1, 2005; 128(6): 1259 - 1266. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 U Nath, R Thomson, R Wood, Y Ben-Shlomo, A Lees, C Rooney, and D Burn Population based mortality and quality of death certification in progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) J. Neurol. Neurosurg. Psychiatry, April 1, 2005; 76(4): 498 - 502. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D C Paviour, J M Schott, J M Stevens, T Revesz, J L Holton, M N Rossor, A J Lees, and N C Fox Pathological substrate for regional distribution of increased atrophy rates in progressive supranuclear palsy J. Neurol. Neurosurg. Psychiatry, December 1, 2004; 75(12): 1772 - 1775. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M B Muzaimi, J Thomas, S Palmer-Smith, L Rosser, P S Harper, C M Wiles, D Ravine, and N P Robertson Population based study of late onset cerebellar ataxia in south east Wales J. Neurol. Neurosurg. Psychiatry, August 1, 2004; 75(8): 1129 - 1134. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
U. Nath, Y. Ben-Shlomo, R.G. Thomson, A.J. Lees, and D.J. Burn Clinical features and natural history of progressive supranuclear palsy: A clinical cohort study Neurology, March 25, 2003; 60(6): 910 - 916. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. Caparros-Lefebvre, N. Sergeant, A. Lees, A. Camuzat, S. Daniel, A. Lannuzel, A. Brice, E. Tolosa, A. Delacourte, and C. Duyckaerts Guadeloupean parkinsonism: a cluster of progressive supranuclear palsy-like tauopathy Brain, April 1, 2002; 125(4): 801 - 811. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N. J. Cordato, C. Pantelis, G. M. Halliday, D. Velakoulis, S. J. Wood, G. W. Stuart, J. Currie, M. Soo, G. Olivieri, G. A. Broe, et al. Frontal atrophy correlates with behavioural changes in progressive supranuclear palsy Brain, March 1, 2002; 125(4): 789 - 800. [Abstract] [Full Text] [PDF]
|
|