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.
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 and scientific benefit regarding Parkinson‘s disease and dementias which are also actively researched in my department.
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 and scientific benefit regarding Parkinson‘s disease and dementias which are also actively researched in my department.