What is CJD?
Understanding CJD and Prion Diseases
Creutzfeldt-Jakob Disease (CJD) and prion diseases are rare but invariably fatal neurodegenerative disorders of the brain, which can remain silent for years before causing rapid and irreversible physical and mental decline, inevitably leading to death.
CJD causes prion proteins in the brain to become misshapen and stick to each other, which multiply and spread throughout the brain.
This process kills brain cells, leading to dementia, problems with speech and movement and, invariably, death.
CJD accounts for 1 in every 5,000 deaths in the UK.
Most patients die within just one year after the onset of symptoms, some within weeks of diagnosis, and others degenerate over several years.
What happens in the brain?
What are the different types of prion diseases?
There are three main types of prion disease.
The most common form is Sporadic CJD, which occurs at random in the population as an unlucky chance event. It causes 1 in 5000 deaths in the UK.
Inherited prion diseases (CJD, GSS – Gerstmann-Sträussler-Scheinker syndrome, FFI – Fatal Familial Insomnia) are caused by a faulty gene passed down in families and affect several hundred people in the UK.
Acquired forms of prion disease (known as iatrogenic CJD) are rarer and caused by accidental transfer of prions during medical or surgical procedures or in the past from eating food contaminated with prions from BSE-infected cattle (in this case also called Variant CJD).
All forms are untreatable and invariably fatal.
What are the symptoms?
In the early stages, CJD causes a variety of symptoms, some of which are commonly associated with other conditions, meaning CJD is often misdiagnosed or diagnosed late. Symptoms include personality change, memory loss, impaired thinking, anxiety, insomnia, depression, trouble speaking and jerky movements. These symptoms usually get worse quickly, and the patient often dies within a few months or even weeks after diagnosis. When suspected, the disease can usually be diagnosed with a combination of a brain scan and a test on spinal fluid.
A specialist clinical service, the NHS National Prion Clinic, can assist with rapid diagnosis and supportive care and is referred most patients with suspected prion disease in the UK.
Is there hope for a treatment?
There is currently no treatment or cure for CJD, but world-leading research at the MRC Prion Unit at UCL developed an experimental antibody treatment called PRN100. This was designed to stop prion proteins from malfunctioning in the brain.
A small number of patients were given PRN100 in an experimental treatment programme in 2018/2019 and PRN100 showed remarkably positive early-stage test results in humans.
The results of the experimental treatment programme were written up in an article published in The Lancet Neurology in April 2022. Read the full article here.
It is hoped that PRN100 could also be used to completely prevent onset of disease in healthy individuals who carry one of the many genetic mutations that cause prion disease during adult life or those who have been accidentally exposed to prions during past medical treatments.
Why is funding so important?
The next crucial step is the Phase II clinical trial, which aims to demonstrate the treatment’s efficacy in patients with CJD.
After 20 years of dedicated research, just a few final steps remain to turn hope into reality — to transform the lives of patients, at-risk populations, families, carers and society as a whole.
£10 million is needed to take PRN100 through clinical trial phase II, a fraction of the cost of comparable research.
The three main types of prion diseases
Sporadic CJD
Inherited
Acquired
Benefits beyond prion diseases: possible future role for Alzheimer's and other neurodegenerative diseases
PRN100 might not just cure CJD, but it could also be used in research for other neurological diseases causing dementia, including Alzheimer’s disease where closely similar processes leading to formation and spread of amyloids formed from different brain proteins is central to the disease process, and Parkinson’s disease.
WITH YOUR SUPPORT, WE CAN CURE CJD.
Finding a treatment for CJD
PRN100 is a potential treatment
A breakthrough for CJD and beyond
World leading research at the MRC Prion Unit at UCL developed an experimental antibody treatment called PRN100. This was designed to stop prion proteins from malfunctioning in the brain.
Positive results were shown in laboratory testing and a small number of patients were given PRN100 in an experimental treatment programme in 2018/2019.
PRN100 is being developed as a potential treatment for CJD and other prion diseases. It may also help advance research into Alzheimer’s and Parkinson’s diseases, which share similar protein misfolding processes in the brain.
The Cure CJD Campaign is working to raise £10 million to fund the research needed to take PRN100 into Phase 2 clinical trials. With your support, we can move one step closer to a treatment for CJD while unlocking discoveries that could benefit many more people affected by neurodegenerative diseases.
PRN100 might not just cure CJD, but it could also be used in research for other neurological diseases causing dementia, including Alzheimer’s disease where closely similar processes leading to formation and spread of amyloids formed from different brain proteins is central to the disease process, and Parkinson’s disease.
WITH YOUR SUPPORT, WE CAN CURE CJD.
Finding a treatment for CJD
World leading research at the MRC Prion Unit at UCL developed an experimental antibody treatment called PRN100. This was designed to stop prions from malfunctioning in the brain.
Positive results were shown in laboratory testing and a small number of patients were given PRN100 in an experimental treatment programme in 2018/2019.