Diagnosis and Prognosis in Progressive Supranuclear Palsy

Lead Research Organisation: University of Cambridge
Department Name: Neurology

Abstract

Progressive supranuclear palsy (PSP) is a disabling, incurable disease, where people have frequent falls and have difficulty moving their eyes. It is however very variable and patients can have many additional problems, such as problems with thinking or speaking.

This variability in the disease can make it difficult to diagnose or to predict how the disease will develop in time. At Cambridge we are combining several methods of research to try to help.

We are carrying out a range of tests including psychology, measurement of eye movements and monitoring of electromagnetic brain waves early in the disease. As we follow patients through their illness, we will see whether these tests help one to understand the differences between patients’ symptoms and importantly whether they can help to predict how the disease will develop.

This would help us to inform patients and their families of the types of problem they are more likely to face in the future. To understand the variation in PSP between different patients would also help us develop better treatments for this disease in the future.

Technical Summary

Progressive supranuclear palsy (PSP) is a devastating neurodegenerative disease of middle age and older life, which remains essentially untreatable. I will study brain structures and functions that may be used to confirm the diagnosis and associated social cognitive deficits, to predict prognosis and disease type, and be potential surrogate markers for future candidate therapies.

The objectives are to study patients using a tailored set of neuropsychological tests, measurements of eye movements, and functional brain imaging with Magnetoencephalography (MEG). A secondary objective is to study these patients again at 1 year, to determine the rate of progression of cognitive problems, in relation to baseline characteristics.

Patients will be recruited from the ‘Disorders of Movement and Cognition‘ clinic which has a large group of patients with PSP. Patients and comparison groups will be studied using psychology tests (memory, organisation, mental flexibility, emotional and social skills). I will then study eye movements because the brain systems eye movement control have alot in common with cognitive brain systems: eye movements may show which patients will develop an earlier dementia. I will study brain function and connectivity in brain networks using MEG. I predict that connectivity is impaired early in the disease before brain shrinkage (atrophy) is significant, and that this could be a sensitive measure of potential new treatments.

Some of our patients will die from their disease during the 3 years (typically 2-4/year). Many patients have declared their intention to donate to the Cambridge Brain Bank. This pathology information will be used to reinforce the findings from the main research methods.

Our research has specific aims to improve the assessment, care and treatment of patients with PSP. However, PSP is an archetype of diseases that give problems with both mental functions and movement. There is therefore a broader potential medical andentific benefit regarding Parkinson‘s disease and dementias which are also actively researched in my department.

Publications

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Ghosh BC (2009) Emotion recognition in progressive supranuclear palsy. in Journal of neurology, neurosurgery, and psychiatry

 
Description Member of PSP association advisory panel
Geographic Reach National 
Policy Influence Type Participation in advisory committee
 
Description Sackler Fellowship
Amount £1,500 (GBP)
Organisation Raymond and Beverly Sackler Foundation 
Sector Charity/Non Profit
Country United Kingdom
Start 10/2007 
End 10/2010
 
Description Sackler Fellowship
Amount £1,250 (GBP)
Organisation Raymond and Beverly Sackler Foundation 
Sector Charity/Non Profit
Country United Kingdom
Start 10/2007 
End 10/2010
 
Description Sackler Fellowship
Amount £1,250 (GBP)
Organisation Raymond and Beverly Sackler Foundation 
Sector Charity/Non Profit
Country United Kingdom
Start 10/2007 
End 10/2010
 
Title HSB 
Description Database of patients with movement disorders such as progressive supranuclear palsy with collection of biological materials for analysis. 
Type Of Material Database/Collection of Data/Biological Samples 
Year Produced 2009 
Provided To Others? Yes  
Impact Articles to be submitted. 
 
Description CBSU collaboration 
Organisation Medical Research Council (MRC)
Department MRC Cognition and Brain Sciences Unit
Country United Kingdom 
Sector Public 
PI Contribution Our team recruited a patient population to investigate the presence of emotion recognition and theory of mind deficits.
Collaborator Contribution Research experience in emotion recognition and theory of mind enabling broad based research within the field, amongst a novel patient population.
Impact This is a multidisciplinary collaboration involving psychologists, computer scientists and clinicians. Publications of papers are ongoing but include Brain as detailed.
Start Year 2007
 
Description Advisory panel on care pathway 
Form Of Engagement Activity A formal working group, expert panel or dialogue
Part Of Official Scheme? Yes
Type Of Presentation Keynote/Invited Speaker
Geographic Reach National
Primary Audience Health professionals
Results and Impact Care pathway was published nationally with local events to roll out to publicise it amongst PD nurses and other health professionals.

Generated some media interest.
Year(s) Of Engagement Activity 2011,2012
 
Description Lecture for PD masterclass 
Form Of Engagement Activity A talk or presentation
Part Of Official Scheme? No
Type Of Presentation Keynote/Invited Speaker
Geographic Reach National
Primary Audience Health professionals
Results and Impact British Geriatric society hold a Parkinson's disease masterclass which I was invited to speak at. My role initially was to disseminate the care pathway to PD professionals but later evolved into teaching about PSP and other Parkinson's plus conditions.

Continuing interest in care pathway for PSP association.
Year(s) Of Engagement Activity 2012,2013
 
Description PSP association symposium 
Form Of Engagement Activity A talk or presentation
Part Of Official Scheme? No
Type Of Presentation Keynote/Invited Speaker
Geographic Reach National
Primary Audience Participants in your research and patient groups
Results and Impact Carers for patients with progressive supranuclear palsy and health care professionals attended a symposium organised by the PSP association. I talked about the cognitive and behavioural aspects of the disease which led to extended discussion during and after the presentation.

Health care paractitioners asked to be included in a PSP network.
Year(s) Of Engagement Activity 2010,2011