Latest news with #CJD
Yahoo
24-05-2025
- Health
- Yahoo
A 58-year-old woman has died of a rare neurological condition. The cause of death? Being injected with hormones taken from a corpse.
When you buy through links on our articles, Future and its syndication partners may earn a commission. A woman in the U.S. has died from severe neurological symptoms that were caused by an abnormal protein — which she was unknowingly injected with nearly 50 years earlier. Having shown no neurological symptoms in the decades since the injection, the 58-year-old recently began experiencing tremors and changes in her ability to balance while walking. In the following weeks, she developed urinary incontinence, difficulty speaking and abnormal breathing. After being admitted to hospital, she entered a coma and later died, according to a case report published May 14 in the journal Emerging Infectious Diseases. A follow-up MRI scan while she was in hospital revealed damage to her brain, and further testing showed positive results for an abnormal protein called a prion. Prions are found in cell membranes, the wall that surrounds every one of our cells, and are thought to be one of the molecules involved in cellular communication and other interactions. Though prions can exist safely in the body, when these proteins accumulate in the brain they can cause surrounding proteins to contort and "misfold," damaging nerve cells. Infection with an abnormally 'folded' prion from an external source – like eating contaminated meat, as in the case of 'mad cow' disease – can also spark a chain of misfolding in the body. The brain damage caused by prion diseases is always fatal, with most patients dying within one year of first developing symptoms, according to Mayo Clinic. In this case, the woman was diagnosed with Creutzfeldt-Jakob Disease (CJD), an extremely rare and fatal brain wasting disease caused by prions. Related: Here's how dangerous, deadly prions spread to the brain It is thought the woman was given prion-infected hormones sometime between 1971 and 1980, while receiving treatment for a condition called panhypopituitarism. People with this condition have a lack of hormones produced by the pituitary gland, the organ that secretes a range of hormones including those involved in growth and sexual development. In the 1970s, panhypopituitarism was treated by injecting patients with human growth hormones extracted from the healthy pituitary glands of people who had died. At the time, this was common practice — as well as being used to treat pituitary conditions, hormones from cadavers were also given to women whose ovaries were not producing eggs. However, in 1985 researchers identified the first U.S. outbreak of CJD related to treatments with cadaver-derived growth hormones. The treatment was quickly suspended, and later a synthetic alternative hormone, produced by genetic engineering, was approved by the Food and Drug Administration (FDA). RELATED STORIES — Alzheimer's comes in at least 5 distinct forms, study reveals — Shingles vaccine may directly guard against dementia, study hints — 13 proteins tied to brain aging seem to spike at ages 57, 70 and 78 So far, 0.4% of those treated in the U.S with growth hormones taken from the organs of deceased individuals have gone on to develop CJD. How these hormones were infected with prions is still unknown, and scientists cannot yet explain why there is such a long latency period between the infection and the symptoms arising. There is currently no treatment available to those with CJD, or any other form of prion disease. However, ongoing research projects seek to uncover treatments for the conditions. The researchers involved in the recent case study said that while the number of people developing CJD as a result of growth hormone treatment has slowed over the years, it is still possible that new cases will arise.
Time of India
21-05-2025
- Health
- Time of India
American woman died of a mad-cow-like disease that lay dormant for 50 years: All you need to know
Image credits: Getty Images Sometimes life feels fine until the tragedy strikes and you realise that it was in the cards. An American woman recently died from an incurable prion disease that she had caught 50 years before. In a paper published on Wednesday in the journal Emerging Infectious Diseases, doctors revealed that the 58-year-old woman had developed a form of Creutzfeldt-Jakob disease (CJD) that she likely contracted through contaminated human growth hormone (HGH) treatments she received as a child. While the globally fatal disease is popular, the death presented the longest latency period ever documented for it. Creutzfeldt-Jakob disease Image credits: Getty Images Creutzfeldt-Jakob disease is a rare, fatal and rapidly progressive neurodegenerative disorder caused by abnormal prion proteins. Prions are proteinaceous infectious particles, a rogue protein that triggers normal proteins to misfold and form a clump and disturb brain function. Over time, the accumulation of bad prions destroys the brain and leads to symptoms such as dementia and death. What's concerning is that they are resistant to most standard methods of sterilisation that are used to kill other infectious agents like bacteria and viruses. How are prions caused? Prions can emerge sporadically with no clear reason. They can be caused by inherited mutations or can be transmitted through close contact with infected bodily fluids or brain matter. In the 1980s, doctors discovered that one could catch CJD through human growth hormone extracted from prion-infected cadavers. by Taboola by Taboola Sponsored Links Sponsored Links Promoted Links Promoted Links You May Like Villas Prices In Dubai Might Be More Affordable Than You Think Villas In Dubai | Search Ads Get Rates Undo A decade later, it was learned that one could catch the disease via tainted beef from cows infected with their own version of the prion disease, which is nicknamed the mad cow disease. What are the symptoms of CJD? Image credits: Getty Images According to the National Institute of Neurological Disorders and Stroke, the main symptoms of CJD include cognitive troubles leading to dementia, involuntary muscle jerks, and lack of coordination of movements. While it can be diagnosed through a neurological exam, EEG, MRI and CSE tests, it has no cure as of now. What happened to the woman? According to the report, the woman initially visited doctors with tremors and trouble balancing. Once the symptoms of the disease started, her condition worsened, she was hospitalised, fell into a coma and died. An autopsy revealed her death's cause as CJD but ruled out genetic causes. Since she had received HGH treatment, it was considered to be the root cause of her illness by doctors. HGH is used to treat severe growth-related conditions in children and has been used for many years. In this case, the woman received her first treatment 51.3 years before her symptoms appeared. Since the US switched the collection of HGH from cadavers in 1977, thus reducing the risk of prion contamination, the timeframe of the woman's infection is around 48.3 years earlier. According to the authors, people who have a history of HGH treatment before 1977 must look out for similar delayed cases. 'Although the US CJD outbreak has slowed substantially, the potential for new cases remains,' they wrote. One step to a healthier you—join Times Health+ Yoga and feel the change
Gizmodo
16-05-2025
- Health
- Gizmodo
U.S. Woman Dies From Mad Cow-Like Brain Disease That Lay Dormant for 50 Years
The grim reaper can sometimes take decades to catch up with its victims. In a report out this week, scientists describe a woman who died from an incurable prion disease caught roughly 50 years earlier. Doctors detailed the unusual case in a paper published Wednesday in the journal Emerging Infectious Diseases. The 58-year-old woman developed a form of Creutzfeldt-Jakob disease (CJD) likely contracted through contaminated human growth hormone (HGH) treatments she received as a child, they determined. The tragic death may represent the longest latency period ever documented for this universally fatal disease. Disease-causing prions are one of the strangest things that can sicken people. They're the misfolded form of the normal (but still very mysterious) prion protein found in our bodies and brains. When a rogue prion comes across its harmless counterpart, it converts the latter into a misfolded copy of itself, much like a zombie outbreak. Over time, the exponential accumulation of bad prions destroys the brain, leading to symptoms like dementia and death soon after. If that's not scary enough, prions are naturally resistant to most standard methods of sterilization used to kill off other infectious agents like bacteria or viruses. The one saving grace is that prion diseases in general remain very rare, though other prion-like diseases like Alzheimer's are more common. Prions can emerge sporadically for no clear reason, can be caused by inherited mutations, or they can be transmitted though close contact with infected bodily fluids or brain matter. In the 1980s, for instance, doctors discovered that people could catch CJD through human growth hormone extracted from prion-infected cadavers. A decade later, we learned that people could also catch CJD via tainted beef from cows infected with their own version of prion disease (nicknamed mad cow disease). The discovery of HGH-related CJD quickly led to the end of cadaver-sourced HGH in the U.S. and other countries (thankfully, synthetic HGH became available soon after). Still, over two hundred cases of iatrogenic CJD—iatrogenic meaning it was caused by a medical treatment—from tainted HGH have been documented worldwide. Most of these cases were reported between five and 10 years after people had used HGH. But prion disease can sometimes take much longer to manifest in certain people, even decades. According to this latest report, the woman first visited doctors with tremors and trouble balancing. As is often the case, once symptoms started, her condition rapidly worsened. She was hospitalized just four weeks later, and several days into her stay, she fell into a coma that she would never awaken from. An autopsy confirmed the woman's death from CJD but ruled out known genetic causes. And since she received HGH treatment prior to the cadaver ban, it was likely the root source of her illness, the doctors concluded. HGH is used to treat severe growth-related conditions in children, and is typically taken over many years, including in this case. That makes it impossible to know exactly when the woman contracted CJD. Her first treatment was 51.3 years prior to her symptoms appearing, for instance, while her last treatment was 42.1 years earlier. But the U.S. switched to a different method of collecting HGH from cadavers in 1977, one that inadvertently and greatly reduced the risk of prion contamination. Based on that, the most likely timeframe for her infection is around 48.3 years earlier, the authors calculated. There are known cases of people dying from CJD infections caught over 30 years ago, as well as reports of other prion diseases emerging over a half century later. But this appears to be the longest case of HGH-related CJD documented so far, though it might not be the last. The researchers note that genetics can influence a person's chances of developing prion disease. And the woman possessed a mutation in the gene that codes for the prion protein linked to longer latency periods. So while tainted human growth hormone hasn't caused CJD for a very long time, some people out there may still one day succumb to their earlier exposure (it's estimated that around 7,700 people in the U.S. took HGH prior to the cadaver ban). The authors say that doctors treating neurological illness should at least be on the lookout for these delayed cases, especially if their patients have a history of HGH treatment before 1977. 'Although the US iCJD outbreak has slowed substantially, the potential for new cases remains,' they wrote.
Metro
09-05-2025
- Health
- Metro
'People will die before they get compensation from the Infected Blood Scandal'
Cressida Haughton (left) lost her father Derek and Deborah Dennis her husband Barrie after they were infected by blood plasma (Picture: Jeff Moore/PA Wire) A victim of the Infected Blood Scandal has accused the government of 'playing a waiting game' in paying compensation. Steve Nicholls, 58, from Surrey, is one of the about 122 infected schoolboys who went to Treloars, a school for disabled children in Hampshire. He was left with hepatitis A, B, C and Creutzfeldt-Jakob disease (CJD) as a result of the injections he was given. Eighty of the boys have died, he said. He is now calling on the Government to speed up its payouts to the victims and families of those who have died, adding that he fears people will die before they get compensation. More than 30,000 people in the UK were infected with hepatitis and HIV after they were given contaminated blood products in the 1970s and 1980s without their knowledge, now known as the Infected Blood Scandal. The school said it was unaware that the blood products were infected with HIV and hepatitis C and has since apologised to the victims and their families. To view this video please enable JavaScript, and consider upgrading to a web browser that supports HTML5 video Up Next Previous Page Next Page Over 3,000 infected people have died since, and the number keeps rising after one of the biggest scandals affecting the NHS. The victims included boys with haemophilia attending Treloars school, where they were infected without their parents' consent. The service engineer, who went to the school when he was nine, told Metro: 'I got all the infections from Treloars, but I didn't find out until I was 25. I managed to hold down a full-time job, as all the boys did. ' However, at about 40, his health 'started to crash.' Meanwhile, his mum and dad – like hundreds of other parents of the infected – felt 'tremendously guilty' as they unknowingly 'could have signed us up for medical research' and his mum gave him the injections prescribed by doctors. Steve, who is married and has grown-up children, said he feels 'fatigued' and 'very tired, but I will not let this go until I see justice' after 40 years. Steve Nicholls, 58, from Surrey, was infected with hepatitis A, B and C and a blood clotting disease by blood products given to him at the Treloars School (Picture: PA) He described the delay in compensation as 'completely unacceptable' He continued: 'If people pass away before they make a claim for compensation, in many cases that compensation claim dies with them. 'It's hard to draw any conclusion that the government is playing a waiting game. It should be much simpler and faster.' He said the infection scandal has had 'a massive impact' on this family and other victims who 'continue to suffer today.' Following years of tireless campaigning by victims and their loved ones, the scandal made headlines, leading to an official inquiry led by Sir Brian Langstaff. Bombshell documents previously revealed how children were compared to chimpanzees by government-funded agencies as part of their plans to use blood plasma known as Factor VIII. Stuart Cantrill lost his dad (pictured) to AIDS at the age of 15 after his father was infected with HIV he got from blood products (Picture: Stuart Cantrill) The inquiry heard that documents from 1970 reveal how the scientists working on the research knew that the Factor blood products 'have been found to transmit this form of hepatitis to chimpanzees,' yet they still sent the products to the school and did not inform them. The infected plasma was bought from the US, where pharmaceutical companies had sourced the blood by mixing it from prisoners and paid donors. It was then sold to hospitals and schools for haemophiliac children in the UK. To make matters worse, the details of the infections were widely covered up, the a report found last year. While the inquiry concluded last May and found there were 'catalogue of failures' with 'catastrophic' consequences, the gruelling wait for compensation continues for many. Danielle Mullan holds a picture of her mum Marie Cromie, who died on July 4, 2023 after being infected with hepatitis C from infected blood products (Picture: Liam McBurney/PA Wire) Last year, former Prime Minister Rishi Sunak described the inquiry's findings as a 'day of shame' for the state. Further hearings on the government's response to compensation were held this week, with victims and campaigners speaking on Wednesday and yesterday. Nick Thomas-Symonds, a Cabinet Office minister, was heckled when he gave evidence. He apologised and told the victims and families in the room that the 'government has failed you over decades on behalf of the state. I am sorry.' When he was asked whether both victims and affected people will die before compensation, he said 'yes.' The government has said it has paid £80,000,000 in compensation out of the £11,800,000,000 in total. Steve, who has been campaigning for the infected, said the morale is 'at an all-time low.' Former students of Treloars School (from left to right): Adrian Goodyear, Richard Warwick, Steve Nicholls, and Gary Webster (Picture: PA) He said they could 'see the light at the end of the tunnel' last year when the initial inquiry concluded – but the relief was short-lived. Speaking from the hearing during a break, he told Metro: 'We felt validated and recognised, we have fought for over 40 years to be recognised, and we thought we had achieved it and the rest would follow very quickly. 'But within two days, the government had called the General Election, and we knew we were in trouble and could see delays.' 'Our numbers are diminishing fast,' he added. The relatives of victims of the blood scandal posed with the final report last year (Picture: AFP/Images) What happens next for victims? The compensation service is set to open up to around 100 people each week, the independent compensation body told Metro. A spokesperson for the Infected Blood Compensation Authority said: 'Those impacted by the infected blood scandal have waited decades for recognition and compensation, and that's why our priority remains paying as many people as soon as possible. We thank the Infected Blood Inquiry and all those who gave evidence across the two days of further hearings. 'We have started small, learning from each person making a claim, and have continued to build these learnings into our claim service as we have grown. So far, 677 people have been asked to start their compensation claim and this number continues to grow, with more than £90 million offered in compensation. To view this video please enable JavaScript, and consider upgrading to a web browser that supports HTML5 video Up Next Previous Page Next Page 'We are now opening our service to around 100 people every week. We expect that every living and infected person registered with a support scheme will be able to start their claim by the end of 2025, and we are working through these as quickly as possible.' Treloars School said in a statement: 'With additional hearings being held this week to address the timeliness and adequacy of the Government's response to compensation following the publication of the Infected Blood Inquiry's report 12 months ago, we would like to reiterate the call we made at that time to implement compensation plans without further delay. 'The Inquiry's report laid bare the full extent of this horrifying national scandal. We are deeply saddened that some of our former pupils were so tragically infected and their families affected, and we share our former students' frustration with the time it is taking for compensation to be paid. 'Separately, we are actively working with our former students and their families to deliver a lasting memorial to those who received infected blood products.' Get in touch with our news team by emailing us at webnews@ For more stories like this, check our news page. Arrow MORE: Leeds United fan, 23, in coma after falling from tree during club's promotion parade Arrow MORE: Woman quit over results of 'which Star Wars character are you?' personality quiz Arrow MORE: US warned to prepare as bird flu 'absolutely could be the next pandemic'
BBC News
07-05-2025
- Health
- BBC News
Infected blood scandal: Farnham man criticises compensation speed
'We fear we will die before infected blood justice' 9 minutes ago Share Save Julia Gregory BBC News, Farnham Patrick Barlow and Craig Buchan BBC News, South East Share Save Julia Gregory / BBC Steve Nicholls says he fears he will die before seeing justice after being injected with infected blood in the 1970s and 1980s. A Surrey man who was injected with infected blood at a boarding school says he worries he will die before seeing justice. Steve Nicholls, 58, who lives near Farnham, became infected with hepatitis C and Creutzfeldt-Jakob disease (CJD) after unknowingly taking part in medical trials at Treloar's School in Alton, Hampshire, in the 1970s and 1980s. Further hearings of the Infected Blood Inquiry, beginning on Wednesday, will focus on the government's response to compensation for those affected by the scandal, with Mr Nicholls adding there had been "very little progress" on the scheme. A government spokesperson said infected blood victims had suffered "unspeakably" and that it remains committed to cooperating with the inquiry. Mr Nicholls, a retired service engineer, went to Treloar's School while being treated for haemophilia alongside about 120 children who were also infected. About 80 of those infected have died, with Mr Nicholls saying he is representing them and their families. He added: "We knew that this was a tragedy that was no accident and we would fight to get the truth told. Whoever was left would fight and fight and fight." Mr Nicholls said: "We all feel very, very scared that we are going to die before we see justice or any compensation." Students at the school were unknowingly included in secret trials of blood products imported from America, some of which were infected with HIV and hepatitis. Mr Nicholls said that, following the publishing of Sir Brian Langstaff's report into the scandal in May 2024, victims felt "recognised and validated" but there had been little progress in paying out compensation since. A government spokesperson says it has paid £80 million in compensation out of a total £11.8 billion earmarked for compensation having given itself until the end of 2029 to make payments. More than 30,000 people contracted HIV and hepatitis from contaminated blood products in the 1970s and 80s – and 3,000 people have since died. PA Media Infected blood campaigners called for recognition of victims of the scandal The government spokesperson added: "The victims of this scandal have suffered unspeakably. "We remain fully committed to cooperating with the Inquiry, are acting on its recommendations, and are grateful for its work to date." A spokesperson for Treloar's School says it "sincerely apologises to our former students and their families who were so devastatingly infected and affected by the infected blood scandal". They added: "The inquiry's report lays bare the full extent of this horrifying national scandal. We are deeply saddened that some of our former pupils were so tragically infected and their families affected and we expect the government to implement its compensation plans without further delay." The school has also backed plans for a public memorial to former pupils who died as a result of the infected blood scandal. Follow BBC Surrey on Facebook, on X, and on Instagram. Send your story ideas to southeasttoday@ or WhatsApp us on 08081 002250.
