UCL Institute of Neurology

Scientists in the Department of Neurodegenerative Disease, Dementia Research Centre are beginning an important study to shed new light on the workings of a rare form of Alzheimer’s disease, made possible by a major grant from the Alzheimer’s Research Trust, the UK’s leading dementia research charity.

Researchers in the Dementia Research Centre want to know more about the causes of posterior cortical atrophy (PCA) – a variant of Alzheimer’s that affects only a few thousand people in the UK, including the much-loved Discworld author Sir Terry Pratchett, who is also a patron of the Alzheimer’s Research Trust.

Unlike typical Alzheimer’s, where people experience memory loss in the early stages, PCA takes hold at the back of the brain, which is responsible for interpreting what we see. The first symptoms include visual problems such as difficulty seeing what or where things are – for example, difficulty recognising faces.

Dr Sebastian Crutch, a leading expert on PCA, will compare 50 PCA patients, 50 Alzheimer’s patients and 50 healthy people over a four-year period, in an effort to pinpoint the causes of PCA and the way it progresses. The study, funded by a £329,947 grant from the Alzheimer’s Research Trust, will see his team use a range of techniques to compare people with PCA at different points in time – and they hope their work could lead to new ways of diagnosing the disease, as well as helping predict how it will take hold and how it might respond to possible treatments.

As part of the project, the team will devise a set of visual tests to describe more accurately what people with PCA actually see, with the aim of developing new tools such as reading aids.

Dr Crutch said:
“Because the visual problems associated with PCA are caused by effects on the brain rather than a problem with the eyes, it is difficult for us to simulate exactly what patients see – for example, many people say they see ‘pieces’ of the everyday world but can’t see it as one big picture. Very few studies have looked at the nature of the condition in detail, and we hope this project could tell us much more about how it works. However, there is a huge amount of work for us to do, so we are very lucky to have the Alzheimer’s Research Trust supporting us.”

Dr Simon Ridley, Head of Research at the Alzheimer’s Research Trust, said: “This is an exciting study that should tell us much more about the workings of PCA and the way it differs from other forms of dementia, and we are very pleased to be supporting it. With more than 820,000 people now living with dementia in the UK, it is more important than ever that we act to tackle the condition, and we can only do that through research projects such as this one.”

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