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tv   BBC News at Ten  BBC News  December 11, 2017 10:00pm-10:30pm GMT

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tonight at ten: a major breakthrough in the treatment of huntington's disease which could lead to new therapies for alzheimer's and other conditions. by correcting the defect that causes huntington's, the new experimental drug is potentially the biggest breakthrough in the field for half a century. we talk to one family affected by huntingtons, as researchers underline the significance of this major advance. for the first time we have the potential, we have the hope of a therapy that one day may slow or prevent huntington's disease completely. we'll have more details and we'll be looking at the potential for new treatments for other conditions. also tonight: snow and ice cause disruption on roads and at airports, with temperatures set to fall sharply overnight. police in salford have launched a murder inquiry after three children died in a fire in a house. new york police say a man detonated a bomb at the city's main bus terminal. four people were injured, including the bomber. and tributes to the broadcaster
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keith chegwin — once a familiar face who's died at the age of 60. and coming up on sportsday on bbc news: the full line up for the champions league — including a messi draw for chelsea who'll face barcelona in the last 16. good evening. scientists in london have reported a major breakthough in the treatment of huntington's disease and say it could lead to new therapies for alzheimer's and other conditions. it's been described as potentially the biggest breakthrough in the field for half a century by correcting the defect that causes huntington's —
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a disease which is incurable and kills most sufferers within 20 years of diagnosis. huntington's affects an estimated 10,000 people in the uk, with a further 25,000 thought to be at risk, as our health correspondent james gallagher reports. the allen family has been blighted by huntington's. they have seen their mother, stephanie, die from it. the last year of her life, every time we all went to visit her, she just held us and said, i want to die. the disease claimed their uncle keith and grant mother, 0live, too. they describe it as parkinson's, alzheimer's and motor neurone disease all rolled into one. when you have got something that is degenerative, you know that every day — you know the last day was probably better than the next one's going to be. frank, his sister sandy and also their brother peter's brains will all slowly degenerate from huntington's too. but now they have hope. the treatment is called gene—silencing. every cell in the body contains genes which hold the instructions
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for running the body. huntington's disease is the result of a corrupted gene that leads to the creation of a toxic protein which destroys the brain. a messenger carries the blueprints from the corrupted gene, this treatment sticks to the messenger, disabling and lowering the production of toxic brain protein. this will feel a little chilly. 46 patients had the experimental drug injected into the fluid that bathes the brain and spinal cord. the trial should prove if the therapy is safe and effective. it was led by scientists at the university college london, who say the results are of ground—breaking importance. for the first time we have the potential, we have the hope of a therapy that one day may slow or prevent huntington's disease completely. this is the experimental therapy.
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it is exciting, but it is not a cure, it will require far more research and following patients for years to come. this is a brain dieing from huntington‘s. doctors are starting longer trials to see whether targeting the protein families like the allens. if it works and it stops me getting any worse, than would be fantastic personally, i never really thought it would ever happen that that would happen. it's all about that you know can we stop it in other people, our children. this research also holds promise for other illnesses — similar toxic proteins are found in brain diseases including dementia and parkinson's. i really think that this is potentially the biggest
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break through in knew row degenerative diseases for the last 50 years. we have very similar situations in a at least some cases of these other diseases and if the over all mechanism is essentially the same, we should be able to use the same general approach. the allens have made a promise to their children that a treatment would be ready in time for them. research over the next four years will see if gene—silencing can fulfil that promise. and james gallagher is here. it is an important day, but as some people have said it is not a treatment or a cure, are they right to make all these ambitious claims? the thing about these diseases is that they're some of the most intractable problems in all medicine. if you have parkinson's, huntington's, dementia, there is no cure or a huntington's, dementia, there is no cure ora drug huntington's, dementia, there is no cure or a drug to slow the pace. i have been following the research for two years and it is the first hint
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ofa drug two years and it is the first hint of a drug that can slow the pace of one of the diseases. it needs more research. so it is generating real excitement, because it is targeting the fundamental cause of these disease and beyond huntington's it may show the way to more complicated diseases. thank you. snow and ice in some areas have led to the closure of hundreds of schools in wales and central england today, while hundreds of homes in the west midlands and 0xfordshire are facing power cuts for a second night. weather experts are warning that clear skies overnight will see temperatures fall sharply again and motoring organisations are warning about ice on the roads, as our correspondent sima kotecha reports. a blanket of snow covering large parts of the uk. it may look pretty but for many, it causes disruption, chaos and stress. an ideal playground for children, many of whom have had the day off,
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with hundreds of schools closed due to slippery roads and pavements. here in birmingham, the council—run schools are closed today. the local authority has faced some criticism as a result, with some saying there was no need because roads like this one have been cleared throughout the day. now, the council says that, tomorrow, it will be up to individual headteachers as to whether they open or remain closed, and that the priority for them is the safety of children. and it's notjust around england. schools in parts of northern ireland and wales have also been closed. 0n the roads, there's ice, making driving dangerous and difficult. some vehicle emergency services are claiming they've had almost 14,000 calls today. tonight, gritters are out in full force. the crews have done about 15 runs through the course of today. we've covered about 17,000 miles of gritting, which is from gloucester
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to australia and back. just trying to keep us moving. in south wales, lorries struggled with the conditions while in northern ireland, temperatures dipped to almost minus double figures. snowcapped rocks lining some of the belfast and londonderry route. in the highest village in surrey, the altitude combined with strong winds made it a challenge but some locals embraced the change. up here, we sometimes get a flurry but it never really settles, so i think this is the first time in about three years we have had some decent snowfall. in the west midlands, an nhs trust put out an appeal for four—wheel drive vehicles to help stranded nurses get to hospitals. well, we offered a number of them up to our health colleagues because obviously there is a lot of pressure on them at the moment in either getting them to patients or getting patients to hospital, so we put them at the disposal of the health colleagues we have got. we've got well trained drivers, a lot of good vehicles that we are happy to lend. and the problems aren't
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just on the road. a quarter of the flights from heathrow have been cancelled after the uk's busiest airport dealt with the fallout from the weekend's heavy snow. and there are still delays to ferry services between dover and calais. however, the port has now reopened after being closed for a few hours. the met office says it's going to be an extremely cold night, with some places experiencing temperatures of around —13 celsius. much of the snow will turn to ice, raising more concerns about travelling tomorrow. sima kotecha, bbc news. well, jon kay is at at a gritting station at stroud in gloucestershire. we have heard about some of the work, but what is going on tonight? they're working flat out right here at the moment. this giant dome stores salt and it was fall on friday, but half of it has gone.
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tonight it is expected to be their busiest night so far. a50 tonnes of salt will be spread. that is just in gloucestershire and just on the roads. that doesn't include the motorways. the latest we have from the met office is that it is going to be the coldest night of year so far. let's look at the figures. in the scottish borders, it could be down to minus nine. in the north—west of england, minus 10. the cold est pa rt north—west of england, minus 10. the coldest part of britain is mid wales where it could be minus 13. so gritting centres are trying to work out when is the best time to put the salt down. if it is too cold it won't work. if it is too wet it won't work. if it is too wet it won't work. if it is too wet it won't work. so they're using data from sensors in the roads to find what is the best time for the rush
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hour. thank you. greater manchester police have arrested five people, four of them on suspicion of murder, after three children died in a house fire in worsley. a 1a—year—old girl, named locally as demi pearson, was pronounced dead at the scene. an eight—year—old boy and a seven—year—old girl died later in hospital. their mother and another three—year—old child are being treated in hospital. 0ur correspondent danny savage reports. in a street of terraced houses on the edge of manchester, a family home, which was set alight early this morning, is now a major crime scene. neighbours who knew the victims and saw what happened have been left deeply upset. just a bang and all fireballs coming from the house. susan smith watched in horror as the children were rescued. they were carrying the children into the ambulance and that. an awful thing to happen on your own street? yes. the 1a—year—old girl who died at the scene has been named locally as demi pearson.
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an eight—year—old boy and seven—year—old boy died later in hospital. a three—year—old girl is in a critical condition and the mother of all the children, named locally as michelle pearson, is also seriously ill. the deaths of three children is heart—breaking. our thoughts are with the family, the little girl and her mum who are fighting for their lives. 0ur specially trained officers are now with the family to help them through this devastating time. tonight, several arrests have been made — the majority of them on suspicion of murder — as scores of detectives continue to work on this investigation. at the nearby church of stjohn the baptist, the doors were opened tonight for people to come in and remember the children who died and it was well attended — such is the effect of these awful events. police in new york say a man detonated a bomb
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close to the city's main bus terminal during the morning rush—hour. four people, including the bomber, were injured in a small explosion in central manhattan. a 27—year—old man, thought to be originally from bangladesh, has been arrested — as our correspondent nick bryant reports. 7.20 in the morning — the height of the rush hour — and security camera footage of an underpass at new york's busiest bus terminal. this low—tech bomb was detonated deliberately in the hope of killing monday morning commuters. the failed suicide bomber had strapped the home—made device to his body with velcro, but he was the only person that's seriously injured. coming as such a busy time in such a congested place, the intent appears to have been to cause mass casualties. the port authority bus terminal serves 65 million passengers a year, but only three other people were treated for minor injuries. what the authorities are calling a terror attack could have been so much worse. thank god the perpetrator did not
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achieve his ultimate goals. thank god our first responders were there so quickly to address the situation and to make sure people were safe. this is the suspect, akayed ullah, a 27—year—old immigrant from bangladesh who arrived here in 2011. he would never have made it into the country, claimed the white house, under president trump's proposed immigration limitations. we must protect our borders, we must ensure that individuals entering our country are not coming to do harm to our people and we must move a merit—based similar of immigration. back in new york, a quick round of instagrams and then the city moved on. what's remarkable is that within two hours of attack, new york city has pretty much returned to normal. there is a road closure here, but the subways are all open and people are going about their business. this attack failed to cause death and it failed to cause much disruption. the authorities believe the failed bomber acted alone.
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the question they haven't yet answered — was he inspired by the group calling itself islamic state? it got lawyers for survivors of the grenfell tower fire have called for a ‘more diverse panel‘ to advise the chairman of the inquiry into the disaster. they were speaking at the start of a two—day hearing into how the inquiry should proceed. 71 people died in the tragedy in june this year. six months on, the bbc has found that the cost of fire safety measures in tower blocks has already reached at least £600 million and could well be more than double that, as our home editor mark easton reports. across the country, the shock from grenfell can still be felt six months on. the question, "am i safe?", still echoes from hundreds of high—rise towers like these in greater manchester. the more i have been thinking about it, the less sleep i have been getting. i have been having broken sleep now for weeks and weeks and weeks. i just don't trust the electrics
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in these blocks any more. this is one that i have actually put in. john smith has been in this council block for 20 years. the cladding that clings to the wall outside john's flat failed safety tests and the council started to remove the panels, but then work stopped amid confusion over what should replace them. fire wardens now patrol day and night. if i had the money, i would not be living here. i'd be living somewhere else. thousands of residents of tower blocks like this one in greater manchester have been told that even now, without definitive guidance from central government, the local authorities cannot give them an absolute assurance of their safety. the current building regulations and fire safety rules have been described as not fit for purpose. we will chase that again over the
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next few days... greater manchester's high—rise task force says it is frustrated that six months after the tragedy in north kensington, so much uncertainty and anxiety remains. this has taken far too long, this is just simply not acceptable. what we have got here is an industrial crisis, as far as i am concerned and it is up to government to actually regulate this industry. with the government's review of building regulations and fire safety not due to publish its final report until spring next year, many housing providers across the uk are reluctant to commit to safety improvements until the post—grenfell rules are clear. bbc research suggests that the bill for work that councils and housing associations say must be done after grenfell has now reached at least £600 million and that is in the public sector alone. but for all the safety first promises made both nationally and locally, there is an argument raging over who should pay. the pipe is going through the wall
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here, they were not sealed properly. phil murphy is a former fire safety officer and after grenfell, he did checks on the manchester tower where he lives. he discovered the building's internal system to stop fire spreading had been completely compromised. they found holes in the floors, holes in the ceiling, holes between the flats, holes between the flats and the escape route. how dangerous was the block at that time? absolutely lethal. phil has now launched a campaign for sprinklers to be fitted to all high—rise towers and many of his neighbours are backing him. that would be a good idea. because all we have got is the holes there. someone is going to have to pay for that, that is the problem. the government says building owners should fund safety measures like sprinklers. even though many fire services say they are essential. where i want to get to is to fully cost this up, look at the potential economic impact here within greater manchester and take that to government and ask them to actually pay for it. do you think central government should foot the bill?
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absolutely. in the days after the grenfell tragedy, the prime minister said she would not and could not ask people to live in unsafe homes. six months on, and many high—rise residents are still worried that is exactly where they will be this christmas. mark easton, bbc news, greater manchester. a man has been arrested on suspicion of trespass after trying to climb into the buckingham palace. the police said the suspect was not carrying any offensive weapons and the incident they say is not now being treated as terror related. three women who claim they were sexually harassed by donald trump have called on the us congress to investigate allegations of his misconduct. the white house has repeatedly rejected the womens‘ accusations, which first came to light during last year's presidential race. mr trump's accusers are now demanding accountability for the president's actions, as rajini vaidyanathan reports. these three women are accusing the most powerful man in the world of sexual misconduct.
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they first spoke out last year, but in the wake of the harvey weinstein scandal, they are now calling on congress to investigate president trump. in an objective setting without question, a person with this record would have entered the graveyard of political aspirations, never to return, yet here we are with that man as president. jessica leeds, who was at today's news conference, says she was assaulted by mr trump decades ago while she was sitting next to him on a flight. the next thing i know, trump is over me like a wet blanket and he is kissing and fondling and everything, and the next thing i realised was that he was putting his hand up my skirt. i grabbed my purse and went to the back of the airplane. trump: and when you are a star, they let you do it. you can do anything. it was after the release of this tape, where mr trump can be heard bragging about groping women, that more than a dozen accusers came forward with allegations of sexual misconduct.
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all i can say it is totally fake news, it is fake, it is made—up stuff and it is disgraceful what happens — but that happens in the world of politics, john. but the women say they are telling the truth. apprentice contestant summer zervos says mr trump forcibly kissed her on several occasions a decade ago. she wants to sue him for defamation after he called her a liar. if the judge in new york decides the case should go ahead, her lawyer could call the president to testify. no man is above the law, including the president of the united states. in the past week alone, three members of congress have been forced to resign over accusations of sexual misconduct. in this current climate, many are asking why the same pressure hasn't been applied at the gates of the white house. but many voters simply aren't concerned. remember, donald trump won last year's election in spite is president of the united states.
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it left me feeling very gross, very dirty. they want to raise the profile of their allegations and hope, in some way, he will be held accountable. rajini vaidyanathan, bbc news, washington. the bbc has commissioned one of the largest surveys of the incidence of sexual harassment at work. the poll of six thousand men and women found that people working in the hospitality sector, self—employed workers, and those on zero—hours contracts, were more likely to face unwanted attention. among the other findings, a0 per cent of women and 18 per cent of men said they'd been sexually harassed in the workplace. nearly one in seven women, 1a per cent, said they've been sexually assaulted at work. and five per cent of women said they were told their career would be advanced in return for sexual favours. our special corrrespondent lucy manning has been listening to some of the women affected. i have had people ask me to send them nude photos in the workplace
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and you feel like nothing is going to be done about it. as a waitress, i experienced sexual harassment all the time. i almost think of my work as sex work. my boss was drunk and tries to come onto me, tries to come onto me, he's wearing this, like, bathrobe, it is disgusting. for many young working women, the question is not if they have been harassed doing theirjob, but how many times. it is one of those things, where there are so many examples of it, it is difficult to choose from. it is quite difficult to even pick one thing, the bbc‘s poll of 6000 people, the largest survey on harassment at work, found a0% of women said it has happened to them. i was a teenager at the time,
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i felt almost in a way, that this must be what it is like, to have a job. this is what it is like to be a young woman, this is normal. a work contact of mine who wasn't in my company, you know, a colleague from somewhere else, decided to stick his tongue down my throat. completely uninvited. this woman has the text of a senior colleague who lunged at her. lam like, i am like, you don't remember trying to kiss me. he said he was devastated, i have never done that in my life. the bbc poll shows that 10% of women have experienced harassment in the last year. is that something you find surprising? no. i don't think it's getting any better with time. ijust feel like it is more normalised. i think womenjust accept it more. people might think it is surprising that so many women are still being harassed in this day and age. ican't imagine...
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when the whole #metoo thing happened, there was not a single one of my girlfriends who was remotely surprised about how many people spoke out. the majority of my male friends just looked at me and said i had no idea that this was so common! female flexible workers are the most likely to suffer harassment at work with nearly 60% saying they have experienced it. i guess you just feel less worthy, in precarious work. i suppose there are more people in our generation who are working like this. this woman prepares to head to her waitressing job. she works in the industry with the most harassment. the poll shows women in hospitality have experienced the most harassment where more than half have suffered it, followed by the service industry. high rates of harassment were reported in the public sector and also in retail. i was pouring a bottle of wine for one guest and a bit of wine splashed out onto his hand and he told me to lick it off. the poll found few have spoken out.
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of those women who say they were harassed at work, just 25% reported it to anyone at all. i know that i never reported it. i left twojobs because i did not feel confident enough to report what was happening. do you think the high profile cases are going to change it for women in the workplace? the high profile cases at the moment are what inspired me to speak up for the first time, because i have never spoken about it before. if famous prominent women can be treated the way they have now revealed, the reality for women in offices, cafes and shops is still largely hidden. lucy manning, bbc news. 0ne one of the most important oil pipelines is being shut down after crack was discovered in aberdeenshire. it carries around a0%
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of northsea crude oil across land for processing at grangemouth. the brands of brent crude rose after news that the plant could be shut for around three weeks. some half a million children and young people are gambling every week. and for the first time, the gambling commission's annual survey, published tomorrow, has looked at gambling with virtual currencies, known as ‘skins betting'. the regulators say cracking down on the 3—billion pound industry is one of their top priorities, as our correspondent sian loyd explains. being a student, you get your student loan, some people would spend it on expensive clothes, you know, i spent it on gambling virtual items. there have been points where i struggled to buy food because itjust takes priority. four years ago, ryan's love of gaming spiraled into gambling. he has lost more than £2000. he is one of a growing number of gamers being drawn into the world of so—called skins betting. it is hard to ask your parents for £1000 to buy, like, like, a knife on cs—g0.
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it is a lot easier to ask for a tenner and try and turn it into 1000. these are those skins, knives and weapons. they are virtual items, used when playing computer games, like counterstrike global offensive, or as these young people call it, cs—go. they have a commercial value, some can be bought for pennies, but the rare ones cost thousands and are highly collectable. they exist within the game, but as these teenagers know, there are unlicensed sites where skins can be gambled and later i have gambled a bit myself. i have lost a bit of money. the concern is that through games like these, young people could be drawn into new forms of online
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gambling, which begs the question, is the regulator struggling to keep pace with the real problems of the virtual world? what we have identified now is a new front of unlicensed skin betting, that is particularly appealing in some cases to children and young people, that is a chief concern to us. they are running up bills, perhaps on their parents‘ paypal account or credit card, but the wider effect is the introduction and the normalisation of this kind of gambling among children and young people. i think it it is a huge emergence this year and i think it is going to get bigger and bigger. children's charities are also concerned. they say many parents are not even aware of the dangers and want to see the gambling commission doing more. gambling is changing it's shape, it is starting to occur in new and different forms and at the moment, regulators do not seem to be doing anything much about it. earlier this year, the gambling commission did
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prosecute one company operating an illegal skins betting site within the uk, but many are based abroad and are easy for young people to find via social media. you don't see an 11—year—old walk into a betting shop and betting 200 on a horse race, you know, but you can do it with this. there is no stopping that. some games providers have put more safeguards in place, but the challenge for the gaming industry and the regulator remains, to keep pace with those businesses who unscrupulously target young people. sian lloyd, bbc news. the broadcaster keith chegwin, known to millions of children
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