#MARTIN HOULDEN

[manfrommars2049]

Elite Dangerous Space Station by Martin Houlden via ImaginaryStarships

[planetejobn]

:Bring Back Adventure: by Martin Houlden

[listingmovies]

TOP 5 ACTORS stuck in their characters.

In television, film and theater, typecasting is the process by which a particular actor becomes strongly identified with a specific character. It usually happens when the actor was introduced to the audience with that role, or when the character was so strong that it is fixed in the public's imagination. And those actors often try to shake off their key roles by doing something radically different. But does that ever work? Could the audience bear to see the man who was once a religious figure, living a romantic drama in a different movie? Or better yet could we ever accept that these roles were just a performance? And that these actors are actually radically different from how we’ve seen them on-screen?

5-      Jim Caviezel in “The Passion of the Christ” (2004) & Robert Powell in “Jesus of Nazareth” (1977)

Most people don’t know his name and refer to him as “the Jesus in that Mel Gibson movie”. Did you know he also played in The Count of Monte Christo and The Thin Red Line? Actors that had a Jesus Role are usually bound to be remembered for that part only. Apparently, playing Jesus is a big deal – knowing he is not the only religious character movies have been made about. However, he remains the most represented historical character whether in books, paintings, movies etc. So how can you rebound from Jesus to another role? In fact, prior to filming, Gibson precisely warned Caviezel that playing Jesus would hurt his acting career. The actor later admitted that good roles had been hard to come by since, but stated he had no regrets about taking the role. He even starred in an advertisement while incarnating Jesus.

If Willem Dafoe managed to get out of Jesus’ robe after The Last Temptation of Christ by Martin Scorsese, it was a struggle for Robert Powell. His role in Jesus of Nazareth came almost by luck. The producers considered choosing a well-known star such as Dustin Hoffman or Al Pacino but chose Powell as he matched the popular perception of Jesus. He was subject to severe criticism from religious groups for 'living in sin' with his companion so the couple had to marry shortly before production began. But Powell's portrayal has since become an often-used image in popular devotional art, and historian James Houlden even claimed that the actor “defined the visual image of Christ in the minds of the audience... Perhaps more than any other Jesus film.”

4-      Audrey Tautou in “Le Fabuleux destin d’Amélie Poulain” (2001)

Tautou achieved international recognition for her lead role in the 2001 film Le fabuleux destin d’Amélie Poulain, which met with critical acclaim and was a major box-office success. If the character in Jean Pierre Jeunet’s movie was quite surreal and unique, Tautou seems to have dragged this weirdo attitude into all her roles. Even when she took on rather sexy parts, the little Amélie always seems to be right around the corner. Maybe it’s because the audience recognized that spirit in Tautou and doesn’t want to see her in something radically different since she achieved to be wonderfully weird. From a shy new actress, to an acclaimed star with this one film, Amélie’s unique character - that ate Tautou’s own personality - got Luc Besson to say: “I wish I had been the producer of that movie”.

3-      Tom Hulce in “Amadeus” (1984)

Amadeus is a fictionalized biography of Wolfgang Amadeus Mozart. Antonio Salieri, an Italian composer contemporary of Mozart is so driven by jealousy of his talent and his success as a composer that he plans to kill him. He plots to pass off a Requiem - which he secretly commissioned from Mozart - as his own. Mozart is pictured as a very irrational and childish genius. It was imperative to give him a unique personality to contrast his talent with his foolish manners. Kenneth Brannagh was one of the finalists for the role of Mozart, but was dropped from consideration when Forman decided to make the film with an American cast. Hulce used many sources of inspiration to portray Mozart as an unpredictable genius with a lot of mood swings. And he put so much of his soul into this role that it’s hard to remember him in anything else. The unique and very special Mozart he was able to play gave the script an essential upturn. He may have lost the Oscar for best actor to his partner F. Murray Abraham who played Salieri but remains linked in our minds to the famous musician more than any other role.

2-      Clint Eastwood in Spaghetti Westerns (1960’s)

Now I realize I’m analyzing a giant here. Clint Eastwood, film actor, director, producer, and composer. has appeared in over 50 movies of the most several genres. But what really gets stuck in our heads with time is the Western Eastwood. In fact, I’m sure we all have the same picture of him in mind: him, his poncho, his hat, his cigarette, pulling out a fireproof vest from under his clothes and a zoom in on his squinted eyes. It’s a bit like the fast food/McDonald’s association. Anyway, so it’s pretty impressive how this guy spent over 50 years trying to make various movies, but still ends up being the Western guy. Some people even believe that Eastwood’s time is the authentic Western Movies era. While we all know (if you didn’t, I just saved you) that the real western movement started in the early 1900’s and was prominent in the time of director John Ford. What Sergio Leone did was making westerns only by changing their rules. The hero becomes for instance an anti-hero, that is far from perfect. But somehow, despite all that, Eastwood has been holding the “cool guy” label for decades now. It is a notable reference for Westerns, a lot more than John Wayne – John Ford’s favorite actor. In Back to the future 3, Marty goes back to the year 1885 and calls himself Eastwood because it’s the first Western name that pops into his head. Would you have thought of John Wayne?

1-      Carry Fisher in the ‘’Star Wars” series (started in 1977)

Carrie Frances Fisher actress, writer and humorist first became known for playing Princess Leia in the Star Wars film series. Princess Leia has been called a 1980s icon, a feminist hero and "an exemplary personification of female empowerment." The character has been referenced or parodied in several TV shows and films, and celebrated in cosplay. The scene where Fisher appeared in the Leia golden bikini has become a mythical moment. Legendary enough that Ross (in Friends) has fantasies over that outfit making his girlfriend Rachel forced to grant his wish and wear it in bed. Leia has also been used in a wide range of very popular Star Wars merchandise. No wonder Fisher could not assert her independence from the character with the bagel hair. In a 2011 interview, Fisher said:

“No one could have known the extent of the franchise. Not that I don’t think I’m cute or anything, but when I looked in the mirror, I didn’t think I was signing away anything of value. Lately I feel like I’m Minnie Mouse—the identity of Princess Leia so eclipses any other identity that I’ve ever had.” The world praised her when she died in 2016 of cardiac arrest. The devastated fans wrote all over social media “So long Princess Leia’’.

[swimmersdaily]

Six years after his last individual title, Patrick Hausding made it again. The 30-year old German won the 1m final with a magical last dive to claim his 15th European gold, the second here in Kyiv. Italy also enjoyed a fine afternoon, Noemi Batki and Chiara Pellacani claimed gold in the 10m synchro, followed by a bronze by Lorenzo Marsaglia in the 1m.

(L to R) BANKS PHOEBE, MARTIN EMILYGBR Great BrItain Silver Medal, BATKI NOEMI, PELLACANI CHIARA ITA Italy Gold Medal, BELIAEVA EKATERINA, TIMOSHININA IULIIA RUS Russia BRONZE Medal Kyiv, Ukraine UKR 07/08/2019 Diving 10 meters platform synchro women podium Len European Diving Championships 2019 Sport Arena Liko Kyiv, Ukraine Photo © Giorgio Scala / Deepbluemedia / Insidefoto

(L to R) KOLODIY OLEG UKR Ukraina silver medal, HAUSDING PATRICK GER Germany Gold Medal, MARSAGLIA LORENZO ITA Italy bronze medal Kyiv, Ukraine UKR 07/08/2019 Diving 1 meter springboard men podium Len European Diving Championships 2019 Sport Arena Liko Kyiv, Ukraine Photo © Giorgio Scala / Deepbluemedia / Insidefoto

HAUSDING PATRICK GER Germany Gold Medal Kyiv, Ukraine UKR 07/08/2019 Diving 1 meter springboard men podium Len European Diving Championships 2019 Sport Arena Liko Kyiv, Ukraine Photo © Giorgio Scala / Deepbluemedia / Insidefoto

BATKI NOEMI, PELLACANI CHIARA Italy ITA Gold Medal Kyiv, Ukraine UKR 07/08/2019 Diving 10 meters platform synchro women podium Len European Diving Championships 2019 Sport Arena Liko Kyiv, Ukraine Photo © Giorgio Scala / Deepbluemedia / Insidefoto

KOLODIY OLEG UKR Ukraina Men – 1m Springboard – Preliminary Kyiv, Ukraine UKR 07/08/2019 Diving Len European Diving Championships 2019 Sport Arena Liko Kyiv, Ukraine Photo © Giorgio Scala / Deepbluemedia / Insidefoto

HAUSDING PATRICK GER Germany Men – 1m Springboard – Preliminary Kyiv, Ukraine UKR 07/08/2019 Diving Len European Diving Championships 2019 Sport Arena Liko Kyiv, Ukraine Photo © Giorgio Scala / Deepbluemedia / Insidefoto

BELIAEVA EKATERINA RUS Russia, TIMOSHININA IULIIA RUS Russia Women – Synchronised Platform – Final Kyiv, Ukraine UKR 07/08/2019 Diving Len European Diving Championships 2019 Sport Arena Liko Kyiv, Ukraine Photo © Giorgio Scala / Deepbluemedia / Insidefoto

BATKI NOEMI ITA Italy, PELLACANI CHIARA ITA Italy Women – Synchronised Platform – Final Kyiv, Ukraine UKR 07/08/2019 Diving Len European Diving Championships 2019 Sport Arena Liko Kyiv, Ukraine Photo © Giorgio Scala / Deepbluemedia / Insidefoto

BANKS PHOEBE GBR Great BrItain, MARTIN EMILY GBR Great Britain Women – Synchronised Platform – Final Kyiv, Ukraine UKR 07/08/2019 Diving Len European Diving Championships 2019 Sport Arena Liko Kyiv, Ukraine Photo © Giorgio Scala / Deepbluemedia / Insidefoto

MARSAGLIA LORENZO ITA Italy Men – 1m Springboard – Final Kyiv, Ukraine UKR 07/08/2019 Diving Len European Diving Championships 2019 Sport Arena Liko Kyiv, Ukraine Photo © Giorgio Scala / Deepbluemedia / Insidefoto

HAUSDING PATRICK GER Germany Men – 1m Springboard – Final Kyiv, Ukraine UKR 07/08/2019 Diving Len European Diving Championships 2019 Sport Arena Liko Kyiv, Ukraine Photo © Giorgio Scala / Deepbluemedia / Insidefoto

Medallists, Day 3

Women’s 10m synchro: 1. Noemi Batki, Chiara Pellacani (ITA) 290.34, 2. Phoebe Banks, Emily Martin (GBR) 284.40, 3. Ekaterina Beliaeva, Iulia Timoshinina (RUS) 277.50

Men’s 1m: 1. Patrick Hausding (GER) 388.85, 2. Oleg Kolodiy (UKR) 381.50, 3. Lorenzo Marsaglia (ITA) 380.15

Italy opened its golden account in Kyiv by a fine win in the 10m synchro, courtesy of Noemi Batki and Chiara Pellacani. As sometimes happens in this event, fewer mistakes earn titles for those who don’t drop under a certain level. That was exactly the case with the Italians: while their dives were not of top quality (received 6.0-6.5s to their last two 3.2DD attempts respectively), but the synchronisation was the best in the field, a series of 7.5-8.0s arrived in each round. Since the two divers had less practice together than usual as Pellacani focuses on her 3m Olympic qualification campaign, their efforts deserve even higher credits – and, ultimately, deserved a gold medal.

Great Britain’s rookies Phoebe Banks and Emily Martin caused a pleasant surprise as they finished runners-up, only 5.94 points shy of the Italians. They also used the chance ‘created’ by the Russians who led after two rounds but then struck the third dive, which seemed to be a cursed one for Ekaterina Beliaeva. Just like a day earlier, when she failed to hold her armstand in the individual final and with a zero-pointer she fell from top to bottom in that very round, this time she missed her dive dreadfully, received 1.0 and 2.0 and the synchro marks were also ruined. They bounced back then, came up with two dives earning the highest scores respectively but had to settle for the bronze.

The men’s 1m final ended up in a real thriller as the lead was changing constantly and no one could gain any bigger advantage. Before the last round home favourite Oleg Kolodiy topped the ranks but had a low DD dive to perform so the following four could aim for something bigger while they were separated by as little as 1.05 points.

Kolodiy came up with a fine last attempt but only with a DD of 2.6. Still, he kept the lead ahead of Jordan Houlden – the Brit had a modest one and finished 1.40 shy of the Ukrainian. Poland’s Kacper Lesiak also eyed the podium but couldn’t perform his most difficult dive as nice as the first three which had put him in the third place back then. The bronze landed in the neck of Lorenzo Marsaglia, winner of the prelims, whose middle two dives didn’t go as well as he planned (under-60s) but especially his final one was a brilliant 72-pointer, enough to edge out Houlden by 0.35 points for the third place.

By then the battle for the title had been over. Patrick Hausding used all his experience and knowledge to pull off the best attempt of the whole evening for 75.20 points with plenty of 8.0s among the marks. It was a kind of magic from the German great who clinched his last individual golds at the home, at the 2014 Europeans in Berlin when he won the 1m and the 3m as well. Six years later, aged 30, he had it again and added another gold to his unique treasury, the second one after the team event: now he possesses 31 medals including 15 golds – and counting…

Quotes

Noemi Batki, Italy, gold, 10m synchro

“When the Russians missed their dive in the third round, we were like, hey, what’s going on here? – but then we had to refocus and concentrate on our dives. I think we managed to do a good job despite we couldn’t train that much together this year. Chiara is more a springboard diver and she wants to qualify for the Olympics in 3m so she rather prepares for that, we could not even practice every dive here before this competition so it was a kind of all-in event from our side but it worked.”

Emily Martin, Great Britain, silver, 10m synchro

“We are quite happy with this medal. We kept our balance for the whole competition, our dives were on a constantly good level. We performed the last one for the first time in competition, it was a kind of gambling but paid off at the end.”

Ekaterina Beliaeva, Russia, bronze, 10m synchro

“We were hoping for a better result, indeed to claim the gold medal. But in the third round I couldn’t hold my legs, I was unable to complete the move, and this bad dive cost us the gold medal.”

Patrick Hausding, Germany, gold, 1m springboard

“The competition was not perfect. I had my reserves here and there, but the others didn’t make perfect jumps either while I could finish the competition with my best dive.

You have to keep your nerves. I always do the two and a half summersaults at the end, because this is my parade jump, that’s how I secured silver here two years ago and gold in Berlin 2014. This is a jump that causes a bit of a stir all the time. If that works really well, then you might receive some extra marks.

Even though this is not an Olympic event, I love to do it. I don’t jump from 10m any more so I have the time to practice it and the level of this competition is just the same you have in the 3m, these are real title battles at the European Championships so I’m keen to be part of it and I really value to have this gold added to my collection.”

Oleg Kolodiy, Ukraine, silver, 1m springboard

“I’m glad since this is my second medal at these European Championships. It was so exciting again to compete in front of our fans and it was a really thrilling final. I’m really happy now.”

Lorenzo Marsaglia, Italy, bronze, 1m springboard

“I’m really satisfied with this bronze medal. This is the first big result I’ve achieved at European Championships. I know that the top four were really close to each other, but I made it, I’m on the podium, so I’m really happy now.”

For detailed results please visit:

http://divingkyiv2019.microplustiming.com/index_web.php

Press release from LEN, photos courtesy of Deepbluemedia / Giorgio Scala

European Diving Championships, Kyiv (UKR) – Day 3: Patrick’s magic works again, gold and bronze for Italy Six years after his last individual title, Patrick Hausding made it again. The 30-year old German won the 1m final with a magical last dive to claim his 15th European gold, the second here in Kyiv.

[mikemortgage]

Huawei CFO extradition ‘casts a pall’ over Canada’s business relationship with China

Ottawa’s move to formally authorized the extradition process for Huawei Technologies Co. chief financial officer Meng Wanzhou could throw a wrench in Canada’s overall business dealings with China, its second-largest trade partner after the United States, say experts in Canada-China relations.

Meng is facing extradition to the United States on charges of bank fraud, wire fraud and conspiracies to commit bank and wire fraud over accusations she lied about Huawei’s business dealings in Iran. Canadian authorities arrested her in December in Vancouver, where she is living under supervision after being released on $10-million bail. China subsequently detained of two Canadians and the upgraded a Canadian’s prison term to a death sentence.

On Friday, Department of Justice officials issued an authority to proceed that enables Meng’s case to move to an extradition hearing at which a judge will determine whether the charges qualify as offences under Canadian law and, if so, whether there is sufficient evidence to extradite her to the U.S. A date for the hearing will be scheduled at a British Columbia Supreme Court appearance on Wednesday.

Canada allows extradition case against Huawei CFO to proceed

Huawei's founder breaks silence to deny U.S. spying claims, calls Trump 'a great president'

'They are retaliating': Canadian canola hit by unusual delays in China after arrest of Huawei executive

In a statement, Meng’s legal team maintained her innocence and expressed disappointment that the extradition will proceed given the “political nature” of the U.S. charges.

“The President of the United States has repeatedly stated that he would interfere in Ms. Meng’s case if he thought it would assist the U.S. negotiations with China over a trade deal,” lawyer David Martin stated.

Martin also expressed concerns that the charges wouldn’t qualify as offenses in Canada.

Ottawa’s move was expected, but the legal process could take years, University of Alberta law professor Joanna Harrington said. The final extradition decision will be made by the Minister of Justice, who will likely be asked to consider any political circumstances, she said. It would be “very rare” for a minister to disagree with the extradition judge.

As Meng’s legal case unfolds, Huawei continues to fight for access to Western markets as telecoms around the world prepare to build 5G networks. Canadian allies including the U.S., Australia and New Zealand have moved to block Huawei from their 5G networks over fears its gear contains backdoors that could enable the Chinese government to conduct espionage. Huawei insists it operates separately from the government and vows it would never use its equipment for spying.

A Huawei booth is seen at a China Mobile 5G experience centre in Shanghai. Huawei is important to China as a “poster child” of a company that can succeed in developed and underdeveloped nations alike, one expert says.

On Thursday, Huawei published a full-page ad in the Wall Street Journal inviting American media to visit corporate headquarters in China to clear up “misunderstandings” the U.S. government has about the telecom.

Last week, its logo was everywhere at the Mobile World Congress in Spain, an event that attracted 100,000 people with name tags hanging from Huawei-branded lanyards.

Huawei is extremely important to the Chinese government as a “poster child” of a company that can succeed in both developed and underdeveloped nations, said University of Alberta Professor Gordon Houlden, director of the China Institute and former foreign service officer in Beijing.

“China really wants a few of their companies to be global brand names in the way Samsung and Honda are, and they don’t really have many candidates,” Houlden said in an interview.

In Canada, Telus Corp. and BCE Inc. have partnered with Huawei to test 5G technology, but neither has officially chosen Huawei as their 5G supplier of choice. Canadian contracts are just a piece of Huawei’s global affairs, but the business is important to China from the perspective of winning in Western nations, Houlden said.

Gordon Houlden, director of the China Institute

Public Safety Canada is in the midst of a security review of 5G technology, but has yet to make a decision on the use of Huawei equipment. On Friday, a spokesman would not reveal a time frame for a decision.

But Meng’s extradition hearing casts a pall over Canada’s business relationship with China, Houlden said. While he has yet to see evidence that the increased tensions have caused a sharp downturn in trade, he anticipates pressure will increase the longer Meng’s case takes.

“It’s a mess with no clear exit,” he said. “There’s some concern that new initiatives, big projects will be more difficult. I’d like to think the majority of the trade will go forward if only because it’s also very much in China’ s interest.”

Even if the U.S. comes to a trade deal with China, it’s unlikely that Canada’s problems will be resolved as long as Meng is kept here, said Alan Alexandroff, director of the Global Summitry Project at the University of Toronto’s Munk School of Global Affairs.

The U.S. administration’s attempt to decouple itself from China when it comes to trade doesn’t make sense in a global economy, Alexandroff said. The strategy moving forward should be to treat China as “neither friend nor foe,” he added.

“That’s what we need to somehow craft. That’s not going to be easy.”

from Financial Post https://ift.tt/2T7HfyY via IFTTT Blogger Mortgage Tumblr Mortgage Evernote Mortgage Wordpress Mortgage href="https://www.diigo.com/user/gelsi11">Diigo Mortgage

[kathrynkfuller9]

IJL and the wedding market: A happy union

International Jewellery London launches Bridal Showcase

  International Jewellery London (IJL) will be celebrating bridal jewellery and the strength of the bridal jewellery market at a special Bridal Showcase at this year’s show, 2-4 September.

This show floor bridal feature, sponsored by award-winning British fine jewellery manufacturer Hockley Mint, will give an insight into key bridal trends, allowing buyers to meet with leaders in the sector.

  Exhibitors taking part in the Bridal Showcase include Hockley Mint, Domino, Gecko, Charles Green, Martin Guthmann, Divine Jewels and Kohinoor Jewellers. The showcase will provide an overview of the latest developments in the bridal jewellery market, alongside fresh takes on timeless classics.

      Exhibitors in the bridal showcase from left to right: Domino Jewellery, Gecko, Kohinoor Jewellers, Hockley Mint, Charles Green, Divine Jewells Inc, Martin Guthmann

  The Bridal Showcase will also feature a seminar on ‘Bridal Trends & Global Weddings: Reaping the Rewards’ (Catwalk Theatre, 10:30, 2 September); a vast range of bridal jewellery on display across the show floor; and numerous events at exhibitor stands.

Fotoulla Michael, Head of Sales at IJL said: “The bridal sector is a key focus for the jewellery industry in the UK, and is certainly booming with over 250,000 marriages taking place in the UK last year. 94% of our visitors from the UK and internationally look to gain an insight into the bridal sector by attending IJL, so the show is an excellent opportunity to discover the latest trends.”

For the first time, the show will also see Hockley Mint (D90) offer pieces from its Mari Me bridal collection available to order as mounts. These award-winning designs in Hockley Mint’s first fully branded bridal collection have previously only been available to retailers as fully finished pieces.

Gary Wroe, MD of Hockley Mint, said: “We are delighted to be sponsoring the new Bridal Showcase area at September’s IJL show. As a Birmingham-based manufacturer of high-quality fine jewellery, bridal jewellery is our speciality; as we start to see more and more retail jewellers moving back towards selling more traditional jewellery and focusing on their specialist knowledge, we hope that this area will attract and inspire visitors at the show.”

In addition to Hockley Mint, the Bridal Showcase will showcase IJL exhibitors Domino, Gecko, Charles Green, Martin Guthmann, Divine Jewels and Kohinoor Jewellers.

IJL is sponsored by the NAJ and supported by the GIA, Gem-A, The Houlden Group, The Company of Master Jewellers, The Responsible Jewellery Council, Euromonitor and CIBJO.

IJL offers buyers the largest variety of gemstones under one roof in Britain today — an unparalleled array of exquisite gems, including white and colour diamonds, rubies, emeralds and sapphires, as well as fabulous colour stones, such as tourmalines, citrines, and garnets, both in loose form and set in contemporary and vintage jewellery. International Jewellery London delivers year-round inspiration, innovation, education and new business opportunities.

IJL is #withyou offering insights into the world of luxury jewellery and accessories. Find out more about our 2018 exhibitors or contact a member of our team for more information.

  from Trending Jewellery https://blog.jewellerylondon.com/ijl-and-the-wedding-market-a-happy-union/

[neurogenpapers]

Polymyositis without Beneficial Response to Steroid Therapy: Should Miyoshi Myopathy be a Differential Diagnosis?

PubMed: Related Articles Polymyositis without Beneficial Response to Steroid Therapy: Should Miyoshi Myopathy be a Differential Diagnosis? Am J Case Rep. 2017 Jan 05;18:17-21 Authors: Scalco RS, Lorenzoni PJ, Lynch DS, Martins WA, Jungbluth H, Quinlivan R, Becker J, Houlden H Abstract BACKGROUND Miyoshi myopathy (MM) is an autosomal-recessive muscle disorder caused by mutations in the DYSF gene. Clinical features and histopathological changes in dysferlinopathies may mimic inflammatory myopathies and a high degree of clinical suspicion is required to guide the genetic investigation. CASE REPORT We report the case of a 16-year-old male who presented with severe bilateral calf pain and elevated CK levels (15 000 IU/l) who was on prolonged steroid therapy prompted by the clinical suspicion of inflammatory myopathy. Three years into his illness, he was referred for neuromuscular evaluation presenting with untreatable muscle pain and progressive weakness. The diagnosis of "refractory polymyositis" was revisited. Targeted exome sequencing revealed homozygous pathogenic mutations in the DYSF gene, confirming a diagnosis of Miyoshi myopathy. CONCLUSIONS Our case illustrates that severe muscle pain may be the initial feature of Miyoshi myopathy and should be considered in the differential diagnosis of inflammatory myopathies. Although the described patient reported partial clinical improvement in muscle pain, steroid treatment is not an effective therapy for dysferlinopathy patients and it did not prevent disease progression. In addition, we confirm the utility of next-generation sequencing approaches to myopathies, particularly in complex or unusual cases when muscle biopsy is not available. PMID: 28053302 [PubMed - in process] http://dlvr.it/N39sfP

[icarussol]

ALIEN vs. HALO by Martin Houlden

[neurogenpapers]

Polymyositis without Beneficial Response to Steroid Therapy: Should Miyoshi Myopathy be a Differential Diagnosis?

IoN UCL PubMed: Related Articles Polymyositis without Beneficial Response to Steroid Therapy: Should Miyoshi Myopathy be a Differential Diagnosis? Am J Case Rep. 2017 Jan 05;18:17-21 Authors: Scalco RS, Lorenzoni PJ, Lynch DS, Martins WA, Jungbluth H, Quinlivan R, Becker J, Houlden H Abstract BACKGROUND Miyoshi myopathy (MM) is an autosomal-recessive muscle disorder caused by mutations in the DYSF gene. Clinical features and histopathological changes in dysferlinopathies may mimic inflammatory myopathies and a high degree of clinical suspicion is required to guide the genetic investigation. CASE REPORT We report the case of a 16-year-old male who presented with severe bilateral calf pain and elevated CK levels (15 000 IU/l) who was on prolonged steroid therapy prompted by the clinical suspicion of inflammatory myopathy. Three years into his illness, he was referred for neuromuscular evaluation presenting with untreatable muscle pain and progressive weakness. The diagnosis of "refractory polymyositis" was revisited. Targeted exome sequencing revealed homozygous pathogenic mutations in the DYSF gene, confirming a diagnosis of Miyoshi myopathy. CONCLUSIONS Our case illustrates that severe muscle pain may be the initial feature of Miyoshi myopathy and should be considered in the differential diagnosis of inflammatory myopathies. Although the described patient reported partial clinical improvement in muscle pain, steroid treatment is not an effective therapy for dysferlinopathy patients and it did not prevent disease progression. In addition, we confirm the utility of next-generation sequencing approaches to myopathies, particularly in complex or unusual cases when muscle biopsy is not available. PMID: 28053302 [PubMed - in process] http://dlvr.it/N31DgR

[artmonia]

Fisherman: by Martin Houlden.