Clinical Case Database / Category: Patient Management

Supranuclear palsy

Publication details

Dr Khaled Amar, Dr Ryan Aguiar, Dr. Jonathan Hewitt
Foundation Years Journal, volume 4, issue 3, p.19 (123Doc Education, London, March 2010)


Progressive supranuclear palsy (PSP), also known as Steele-Richardson-Olszewski syndrome, is an uncommon neurodegenerative disease which often starts in middle and late life, and is frequently misdiagnosed as Parkinson's disease (1). PSP is characterised by a predominantly axial Parkinsonism with head retraction and a poor response to levodopa. Other classic features include an early onset of postural instability and falls together with paralysis of downward gaze. Cognitive impairment and dementia is well documented in PSP but a presentation with dementia prior to the motor features is rare. We present a case of PSP that presented with cognitive impairment progressing to a dementia picture for 4 years before the onset of the typical motor features of PSP.

Access the Clinical Cases Database

A subscription is required to read the full article. Please subscribe using one of the options below.

Foundation Years Clinical Cases Database£29.006 months
Add to cart
Foundation Years Clinical Cases Database£39.0012 months
Add to cart


Dr Khaled Amar

Consultant Physician
Royal Bournemouth Hospital, Dorset

Dr Ryan Aguiar

Clinical psychologist
King's Park Unit
Bournemouth, Dorset

Dr. Jonathan Hewitt

Consultant in old age Psychiatry
Barrow Hospital, Bristol


1. Steel JC, Richardson JC, Olszewski J (1964) Progressive supranuclear palsy. Arch Neurol, 10:333–359.
2. Fung KM, Forman MS, Cucchiara B, et al. (2001) A 70 year old with rigidity, decreased ocular movements and dementia. Brain Pathol, 11:119–120.
3. Esmonde T, Giles E, Xuereb J, Hodges J (1996) Progressive supranuclear palsy presenting with dynamic aphasia. J Neurol Neurosurg Psychiatry, 60:403–410.
4. Davis PH, Bergeron C, Mclaghlan DR (1985) Atypical presentation of progressive supranuclear palsy. Ann Neurol. 17:337–343.
5. Morris HR, Gibb G, Katzenschlager R, et al. (2002) Pathological, clinical and genetic heterogeneity in progressive supranuclear palsy. Brain, 125:969–975.
6. Albert ML, Feldman RG, Willis AL, et al. (1974) The subcortical dementia of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry, 37:121–130.
7. Bigio E, Brwin DF, White CL, et al. (1999) Progressive supranuclear palsy with dementia: cortical pathology. Neurol, 58:359–365.
8. Cambier J, Masson M, Viader F, et al. (1985) Frontal syndrome of progressive supranuclear palsy. Rev Neurol, 141:528–536.
9. Goffinet AM, DeVolder AG, Gillian C, et al. (1989) Positron tomography demonstrates frontal hypometabolism in progressive supranuclear palsy. Ann Neurol, 25:131–139.
10. Josephs KA, Ishizawa T, Tsuboy Y, et al. (2002) A clinicopathological study of vascular progressive supranuclear palsy: a multi-infarct disorder presenting as progressive supranuclear palsy. Arch Neurol, 59:1597–1601.
11. Nakashima H, Terada S, Ishizu H, et al. (2003) An autopsied case of dementia with lewy bodies with supranuclear gaze palsy. Neurol Res, 25:533–537.
12. Litvan I, Grimes DA, Lang AE, et al. (1999) Clinical features differentiating patients with post-mortem confirmed progressive supranuclear palsy and corticobasal degeneration. J Neurol, 246(S2):1–5.
13. Bergeron C, Davis A, Lang AE. (1998) Corticobasal ganglionic degeneration and progressive supranuclear palsy presenting with cognitive decline. Brain Pathol, 8:355–365.
14. Baker M, Litvan I, Hoilden H, et al. (1999) Association of an extended haplotype in the tau gene with progressive supranuclear palsy. Hum Mol Genet, 8:711–715.
15. Litvan I, Agid Y, Calne D, et al. (1996) Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski): report of the NINDS-SPSP international workshop. Neurol, 47:1–9.
16. Litvan I, Agid Y, Jankovic J, et al. (1996) Accuracy of clinical criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome). Neurol, 46:922–930.
17. Josephs KA, Dickson DW (2003) Diagnostic accuracy of progressive supranuclear palsy in the society for progressive supranuclear palsy brain bank. Mov Disord, 18:1018–1026.


Conflict Of Interest

The Journal requires that authors disclose any potential conflict of interest that they may have. This is clearly stated in the Journal’s published “Guidelines for Authors”. The Journal follows the Guidelines against Conflict of Interest published in the Uniform Requirements for Manuscripts Submitted to Biomedical Journals (

Financial Statement

The authors of this article have not been paid. The Journal is financed by subscriptions and advertising. The Journal does not receive money from any other sources. The decision to accept or refuse this article for publication was free from financial considerations and was solely the responsibility of the Editorial Panel and Editor-in-Chief.

Patient Consent statement

All pictures and investigations shown in this article are shown with the patients’ consent. We require Authors to maintain patients’ anonymity and to obtain consent to report investigations and pictures involving human subjects when anonymity may be compromised. The Journal follows the Guidelines of the Uniform Requirements for Manuscripts ( The Journal requires in its Guidelines for Authors a statement from Authors that “the subject gave informed consent”.

Animal & Human Rights

When reporting experiments on human subjects, the Journal requires authors to indicate whether the procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the HelsinkiDeclaration of 1975, as revised in 2008.

About the Clinical Cases Database

T​he Foundation Years Clinical Cases Database is​ a selection of 600 peer-reviewed clinical cases in the field of patient safety and clinical practice, specifically focused on the clinical information needs of junior doctors, based around the Foundation Year Curriculum programme (MMC). The cases have been chosen to align with the Foundation Year Curriculum.

The database is fully searchable, or can be browsed by medical specialty. Abstracts can be read free of charge, however a subscription is required in order to read the complete cases.