UCL HUNTINGTON’S DISEASE RESEARCH

INFORMATION FOR SCIENTISTS, CLINICIANS, PATIENTS, RELATIVES AND CARERS

white-logo_win-HD

 

What’s the study about?

The WIN-HD is an MRC funded collaborative project between the Huntington’s Disease Research Centre at UCL and ICM, Pitié-Salpêtrière University Hospital in Paris.  The project aims to understand the white matter changes that occur in HD in the years prior to the onset of visible clinical signs, known as the pre-manifest stage.

 

What is white matter?

White matter is made up of bundles of nerve fibres (axons) which are extensions of nerve cells (neurons). Many of these nerve fibers are surrounded by a type of sheath, or covering, called myelin. This myelin is white, which gives white matter its colour.  These myelinated fibres are like the motorways between brain regions, speeding up transmission of electrical nerve signals and protecting nerve fibers from injury.  Degeneration of this white matter may play a big role in the clinical symptoms we see in HD.

 

How do we measure ‘white matter’ with MRI?

Whereas previous magnetic resonance imaging (MRI) studies have detected volumetric changes in the brain, it is not known whether white matter changes are directly due to neuron loss or other processes that occur before this neurodegeneration.  Using MRI we are using novel methods called Diffusion-Weighted NMR spectroscopy (DW-MRS) and diffusion MRI analysed using Neurite Orientation Dispersion and Density Imaging (NODDI) in people carrying the HD gene as well as non-carriers.  DW-MRS offers the unique opportunity measure the movement of metabolites inside the neurons non-invasively, which will allow us to better distinguish between the different pathological processes that lead to the changes observed in pre-manifest HD.  NODDI is a way of analysing diffusion imaging data that allows us to estimate the complexity of neurites in white matter, by estimating their density and spatial configuration in the brain.

 

What’s the bigger picture?

WIN-HD is part of a larger project (taking place at the ICM, Pitié-Salpêtrière University Hospital in Paris) that will combine clinical research, preclinical modelling, mouse biology, brain imaging in human pre-manifest HD gene carriers, post-mortem brain samples, and rodent models to investigate early white-matter changes in Huntington’s Disease.  Imaging in humans will hopefully help us to untangle the relationship between the clinical and MRI measures seen in the human brain, with the cellular and molecular changes seen in post-mortem brain tissue. Due to the certainty of disease onset in pre-manifest HD gene-mutation carriers this will allow us to investigate the early interactions of cell sub-types long before clinical diagnosis.

 

How can I find out more?

Please contact Hannah Furby on h.furby.11@ucl.ac.uk if you want to receive more information.

Updated December 18, 2017