document_title: England at the FIFA World Cup

question_text: when was the last time england played semi finals world cup

question_tokens: when was the last time england played semi finals world cup

text: England at the FIFA World Cup

text: History[edit]

text: Brazil 1950[edit]

text: England's first qualifying campaign for the FIFA World Cup doubled as the 1950 British Home Championship. The series kicked off for England on 15 October 1949 at Ninian Park, Cardiff, against Wales. Stan Mortensen gave England the lead after twenty two minutes, and just seven minutes later, Jackie Milburn doubled the lead. This was the first goal of Milburn's hat trick, which left England 4–0 up with 20 minutes to play. Mal Griffiths scored a consolation goal for Wales ten minutes from time, but England held on for a comfortable victory.

text: A month later, England welcomed Ireland to Maine Road, and it began well for the home side as Jack Rowley scored inside six minutes. England were already 6–0 up, thanks to Jack Froggatt, two for Stan Pearson, Stan Mortensen and a second from Rowley, by the time Ireland struck back through Samuel Smyth after 55 minutes. Rowley added a third and a fourth to his tally in the three minutes following Smyth's goal, however, leaving the score at 8–1 at the hour mark. The frantic scoring rate calmed down after that, with only one apeice before the final whistle, with Stan Pearson completing his brace for England's ninth, and Bobby Brennan scoring for Ireland.

text: It was not until May 1950 that England travelled to Hampden Park to face Scotland, who were also undefeated after their games against Ireland and Wales. With the top two from the group qualifying, both teams were guaranteed progression to the finals, and the game was solely for the honour of winning the British Home Championship, and the seeding advantage to be enjoyed upon reaching Brazil. A solitary goal from Roy Bentley gave England the victory, the title and the top spot in the group.

text: 1950 Finals[edit]

text: England were seeded in pot one for the finals, which meant they were the favourites to progress from Group 2, which also contained Spain, Chile and the United States. England's campaign kicked off against Chile in Rio de Janeiro, and, as was expected, England cruised to a 2–0 victory, courtesy of goals from Stan Mortensen and Wilf Mannion.[citation needed]

text: Their troubles began four days later when they faced the Americans in Belo Horizonte in what has become one of the most famous matches of all time. Joe Gaetjens scored the only goal of the match to give the United States an unlikely victory, which has gone down as one of the World Cup's greatest upsets. A myth arose that the English newspapers were so confident of an English victory that when the result was telegrammed back, they assumed a misprint and printed the score as 10–1 in England's favour. However, this has proven to be untrue.[5]

text: This left England in a sticky situation prior to their final match, against Spain in Rio. They needed to win, and for Chile to beat the United States to stand any chance of going through, and even then they would need the goal averages to fall in their favour. As it turned out, no such calculations were necessary, despite Chile's victory, as Spain's Zarra scored the only goal of the game, eliminating England from the competition.

text: Switzerland 1954[edit]

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text: As with their first World Cup, England's qualifying for the 1954 edition also constituted the 1953–54 British Home Championship. They played Wales at Ninian Park as their first match once again, and the 4–1 result was the same. However, unlike four years earlier, it was the home side that went into the lead, after twenty two minutes through Ivor Allchurch. Despite being 1–0 down at half time, England scored four within eight minutes of the restart; two each for Dennis Wilshaw and Nat Lofthouse.

text: Goodison Park was the venue for England's home clash against Ireland, who were newly renamed Northern Ireland. Harold Hassall got England off to a good start with a goal after just ten minutes. Eddie McMorran put the Irish back on terms just before the hour mark, but Hassall completed his brace six minutes later. Lofthouse completed a comfortable 3–1 win for England.

text: With the top two in the group qualifying for the finals, the final game between England and Scotland, at Hampden Park, settled nothing except the placings within the group, despite Scotland having dropped a point with a 3–3 draw at home to Wales. Allan Brown put the home side ahead after just seven minutes, but it was all square again thanks to Ivor Broadis just four minutes later. Johnny Nicholls gave England the lead for the first time just after half time, and they began to extend a lead after Ronnie Allen's 68th-minute goal. Jimmy Mullen made the game all but certain seven minutes from time, and although Willie Ormond scored a consolation for Scotland with just 1 minute to play, England topped the competition for the second time in a row.

text: 1954 Finals[edit]

text: England were drawn in Group 4 for the finals, with hosts Switzerland, Italy and Belgium. In an odd twist, unique to the 1954 tournament, England and Italy, as the two seeded teams in the group, did not have to play each other.

text: Equally, Switzerland and Belgium did not have to play each other. England's first game in Switzerland was against Belgium in Basel, and they suffered a shock as Léopold Anoul put the Belgians into the lead after just five minutes. Ivor Broadis put the favourites back on terms just over twenty minutes later, and although Nat Lofthouse gave England the lead 10 minutes later, it was proving to be tougher than they had expected against the Belgians.

text: Broadis scored his second just after the hour, but Henri Coppens hit back four minutes later to keep Belgium in the game at only 3–2 down. Anoul completed his brace another four minutes after that to level the scores again. In another oddity peculiar to this World Cup, drawn matches in the group stage would go to extra time, and as such the teams played on with the score at 3–3.

text: Just one minute into the added period, Lofthouse added a fourth for England and they seemed to have won it, but Jimmy Dickinson scored an own goal three minutes later to put the score back at 4–4. It stayed this way until the extra period was up, and as penalty shoot-outs were yet to be invented and replays were not used in the group, the match was recorded as a draw.

text: England's second and final group game was against the hosts in Bern. This proved to be an easier game for the Three Lions, and they scored one goal in each half (from Jimmy Mullen and Dennis Wilshaw respectively) to give them a comfortable win of 2–0. As Switzerland (against England), Italy (against Switzerland) and Belgium (against Italy) had all lost one game, England progressed as group winners, along with Switzerland, who won a play-off against Italy.

text: England faced the winners of group three and defending champions Uruguay in the quarter-finals. Carlos Borges gave the South Americans the lead inside 5 minutes, but Lofthouse put England back on terms ten minutes later. England were clearly struggling, but held on until just before half time, when Obdulio Varela gave the lead back Uruguay.

text: Juan Alberto Schiaffino doubled the lead just after the break, but Tom Finney kept England's foot in the door with his sixty seventh-minute goal. However, it was all over after Javier Ambrois restored the two-goal lead with twelve minutes to play. The score remained at 4–2, and England were eliminated from the cup.

text: Sweden 1958[edit]

text: For the first time, England had to play against countries other than the Home Nations to reach the Finals in Sweden. They were drawn against the Republic of Ireland and Denmark. In the qualifying round, England won three out of the four games and drew the other. Four months before the World Cup, Roger Byrne, Duncan Edwards, David Pegg and Tommy Taylor all lost their lives in the Munich air disaster while playing for Manchester United. At the finals, which is the only tournament to have seen all Home Nations take part, the Home Nations were all drawn in different groups.

text: England were drawn against the Soviet Union (2–2), Brazil (0–0) and Austria (2–2), who finished third in the 1954 World Cup. At the end of the group stage, Soviet Union and England each had three points, and had scored four goals and conceded four goals. This meant there was a play-off to decide the second-placed team in the group, the winner to qualify. England lost the play-off 1–0 and were thus knocked out. The only consolation for England was that they were the only team to play the eventual winners Brazil and not lose.

text: Chile 1962[edit]

text: The third World Cup which took place in South America, saw England qualify by successfully qualifying from the group, which contained Portugal and Luxembourg, defeating Luxembourg on both occasions, and defeating Portugal at home, and drawing in Lisbon.

text: At the finals, England were drawn in a group with Hungary, Argentina and Bulgaria. England defeated Argentina 3-1, thanks to goals from Ron Flowers, Bobby Charlton and Jimmy Greaves,[6] before playing out a goalless draw with Bulgaria, and a 2–1 defeat to Hungary.[7]

text: England finished in second place behind Hungary and played the winners of group 3, defending champions Brazil, in the quarter-finals. Brazil scored first through Garrincha, before an equaliser for Gerry Hitchens before half time. However, second-half goals from Garrincha and Vavá meant Brazil won the game 3–1, and eliminated England from the competition.[8] This defeat was manager Walter Winterbottom's last game in charge. Winterbottom had led England to four World Cup Finals. From May 1963, Alf Ramsey became the manager of England.

text: England 1966[edit]

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text: In the 1966 World Cup Finals, England used their home advantage and, under Ramsey, won their first, and only, World Cup title. England played all their games at Wembley Stadium in London, which became the last time that the hosts were granted this privilege. After drawing 0–0 in the opening game against former champions Uruguay, which started a run of four games all ending goalless. England then beat both France and Mexico 2–0 and qualified for the quarter-finals.[citation needed]

text: The quarter-finals saw England play Argentina, which ended in a 1–0 win to England. This match saw the start of the rivalry between England and Argentina, when Argentinian Antonio Rattín was dismissed by German referee Rudolf Kreitlein in a very fierce game. A 2–1 win against Portugal in the semi-final then followed. Portugal were the first team to score against England in the tournament. The final saw England play West Germany, with the result finishing in a 4–2 win for England, after extra time.[citation needed]

text: Mexico 1970[edit]

text: 1970 saw the first World Cup finals take place in North America and England qualified automatically for the tournament by winning the 1966 FIFA World Cup. England were drawn in a group with Romania, former world champions Brazil and Czechoslovakia. Each of the matches only saw one goal, with England defeating Romania and Czechoslovakia, and losing to Brazil. The quarter-final saw a repeat of the 1966 final, with England playing West Germany. England were hampered by the fact that first-choice goalkeeper Gordon Banks was ill, and Peter Bonetti played instead. England led 2–0 with goals by Alan Mullery and Martin Peters, but in the 70th minute, Franz Beckenbauer pulled one goal back for West Germany.

text: After Beckenbauer's goal, Ramsey substituted Bobby Charlton, who overtook Billy Wright as England's most capped player ever, with caps totalling 106. Uwe Seeler equalised for the Germans in the eighty first minute, thereby taking the game into extra time. During extra time, Gerd Müller scored the winning goal for West Germany which saw the German side win 3–2. This turned out to be Charlton's last game for England.[9]

text: West Germany 1974[edit]

text: For the first time, England did not qualify for a World Cup. In a group with Olympic champions Poland and Wales, England could not overtake Poland. After only drawing at home to Wales 1–1 and losing the first leg 2–0 to Poland, meant that England had to beat Poland at home, whilst Poland only needed to draw. Poland managed to withstand England's attacks in the first half, who had Martin Peters playing for them. Poland took the lead in the 57th minute with a goal from Jan Domarski.

text: England equalised six minutes later, with a penalty converted by Allan Clarke. England were unable to score any more goals with goalkeeper Jan Tomaszewski keeping England at bay. Brian Clough had previously called Tomaszewski a "clown".[10] The commentator of the game then said "it's all over".[11] Poland took this good form to the finals and ended in third place. After failing to qualify, Alf Ramsey resigned from his post and after a time, where Ramsey and his predecessor had lasted a total of 29 years, no manager was able to last in the job for longer than eight years. This ended when Bobby Robson became England manager.[12]

text: Argentina 1978[edit]

text: England also did not qualify for the fourth World Cup which took place in South America. This time, England were denied by Italy, who had scored three more goals than England after both teams finished on the same points. Goals scored dictated who qualified after the head-to-head record between the two sides finished the same, following a 2–0 home win for each team. The lower-ranked teams in the group were Finland and Luxembourg, but the size of the wins against them proved to be decisive. Nevertheless, Ron Greenwood was given a second chance in charge of England, after taking the role in 1977.

text: Spain 1982[edit]

text: 1982 saw the first time where the European Qualifying Rounds were divided into groups of five teams, where the top two teams qualify for the World Cup. Greenwood used his second chance and took England to Spain by finishing second behind Hungary and above Romania, Switzerland and Norway.

text: At the finals, England won all three group games, defeating France 3-1, with a brace from Bryan Robson,[13] before beating Czechoslovakia 2–0, with a Jozef Barmos own goal, [14] and World Cup newcomers Kuwait 1–0, thanks to a Trevor Francis goal.[15]

text: The next round saw a second group stage consisting of three teams, a first time event at the World Cup. England drew with West Germany 0–0 and after the Germans beat Spain 2–1, England then had to beat Spain with a two-goal difference to progress to the next round. England, however, only managed a 0–0 draw against the Spanish. England remained unbeaten at the end of the tournament. After the World Cup, Ron Greenwood's time as England manager ended, and he was replaced by Bobby Robson.

text: Mexico 1986[edit]

text: 1986 saw the second World Cup to take place in Mexico. England qualified for Mexico 1986 by winning four games and drawing four times against Northern Ireland, who qualified in second place, Romania, Finland and Turkey. In Mexico, England lost their opening game to Portugal 1–0 and could only manage a goalless draw against Morocco. The final group game, however, saw England beat Poland 3–0, which is one of the three highest scores for England at the World Cup, with Gary Lineker scoring a hat-trick.

text: This result took England to second place and finished behind Morocco. England then also beat Paraguay 3–0 in the Round of 16. In the quarter-finals, England renewed their rivalry with Argentina in a game that has become notorious for the Argentina goals, both scored by Diego Maradona. Maradona's first goal, known as the Hand of God, was illegal and should not have counted, as he used his hand to punch the ball into the net. However, the referee missed this infringement, and ruled that the goal should stand. Maradona then made the score 2-0, famously dribbling from inside Argentina's half and around several English players before scoring. Gary Lineker pulled back the score to 2-1, but England ran out of time to equalise, and were eliminated. Nevertheless, Lineker finished with the Golden Boot by scoring six goals and thereby becoming England's first Golden Boot winner.

text: Italy 1990[edit]

text: By winning three and drawing three, England qualified for Italia '90, the second World Cup to be held in Italy, scoring ten goals and conceding none. England were unbeaten through qualification, winning three games, and drawing the remaining three, but still finished second to Sweden, whom they drew with twice.[16] England profited from Romania's 3–1 win over Denmark, who, had they won, would have qualified as the third-best second-placed team. West Germany and England were able to qualify for Italia '90 as the best second-placed teams in the groups with four teams.

text: Because a few years previously saw English hooligans at European competition matches, England were forced to play their group games on Sardinia and Sicily. In group F, was the European champions Netherlands, the Republic of Ireland and Egypt and England. After opening the tournament with a 1–1 draw against Ireland and a 0–0 draw against the Dutch, England then beat Egypt 1–0. This was Egypt's first appearance since the 1934 World Cup.[citation needed] England won the group with four points.[17]

text: In the next round, England had to play Belgium. The game went to extra time, and in the hundredth and nineteenth minute, David Platt scored the winning goal.[18] England also had to play extra time against Cameroon in the quarter-finals. Cameroon were the first African team to have reached the quarter-finals. England opened the scoring through David Platt, but Cameroon quickly turned around the game to lead 2-1. Lineker subsequently won and scored a penalty in the 83rd minute to ensure the game went to extra time. He then scored a second penalty, to see England reach the semi-finals.[19]

text: In the semi-finals, England met West Germany. There was no separating the two teams after 90 minutes, which made England the first team to have played extra time in three successive World Cup games. There was also no separating the two teams after extra time, thereby taking the game to penalties.[20]

text: Although English goalkeeper Peter Shilton dived the right way for every penalty, he was unable to save any. German goalkeeper Bodo Illgner, having failed to save any of England's first three penalties, saved England's fourth penalty, taken by Stuart Pearce. Olaf Thon then scored for Germany, meaning that England's Chris Waddle would have to score his fifth penalty and hope that Shilton saved the Germans' fifth penalty. However, Waddle's penalty missed completely, going high over the crossbar, thereby resulting in England's being knocked out of the competition.[20] The third-place playoff between England and Italy saw England lose their only game of the tournament in normal time. Even though this was England's best finish since the 1966 World Cup, Bobby Robson's time as England manager had come to an end.[21][22]

text: United States 1994[edit]

text: For the 1994 World Cup in the United States, under the leadership of new manager Graham Taylor, England surprisingly did not qualify for the tournament. In a group with six teams, England lost to Norway and the Netherlands, finishing third above Poland, Turkey and San Marino.

text: England went into their final game with San Marino knowing they would need a seven-goal victory and for Poland to beat the Netherlands in the other match in order to qualify.[23] In the game against San Marino, Davide Gualtieri scored against England after nine seconds, taking the lead for the outsiders. England went on to win 7–1, which was too small a goal margin. Additionally, despite the half-time score between the Poles and the Dutch being 1-1, the Dutch went on to win 3-1, meaning that however many goals England scored, they could still not qualify. Taylor's tenure in charge ended and he was replaced by Terry Venables, who was dismissed after England lost the semi-final of Euro 1996, hosted in England.[12]

text: France 1998[edit]

text: After missing out on the World Cup in 1994, England, managed by Glenn Hoddle, qualified for the World Cup in France. England were drawn in Group 2 of UEFA qualifying with Italy, Poland, Georgia and Moldova. England beat Poland, Georgia and Moldova both home and away, but a home defeat to Italy in their fourth match meant they went into the final qualifier at the Stadio Olimpico in Rome just a point ahead of the Azzurri and only needing a draw to qualify automatically; defeat would see them have to navigate a play-off to secure qualification. The match finished as a goalless draw and England finished top of the group.

text: At the finals in France, England played in Group G. England defeated Tunisia 2–0 in the first game, with goals from Alan Shearer and Paul Scholes.[24] Their second match saw England lose 2–1 to Romania; despite an 81st-minute equaliser from Michael Owen, Dan Petrescu scored a winner shortly before injury time. In their final group game, England defeated Colombia 2–0 in the decisive match, thanks to goals from midfielders Darren Anderton and David Beckham.[25] England finished second in Group G, which saw them qualify for the last 16 phase, and play the winner of 1998 FIFA World Cup Group H, Argentina.

text: In a fiery game containing six yellow cards and two penalties, David Beckham was controversially sent off in the 47th minute for what many felt was at most a yellow card offence, knocking over Diego Simeone.[26][27] Gabriel Batistuta opened the scoring from the penalty spot in the fifth minute of the game, before an equaliser also from the spot by Alan Shearer four minutes later. England took the lead through Owen, in the 16th minute. Argentina drew level through Javier Zanetti in injury time of the first half.

text: The game finished 2-2, and, as neither team were able to find a winner in extra time, penalties were needed to decide the team that qualified to the next round. While David Seaman did save one penalty, Argentine goalkeeper Carlos Roa managed to save two, including the vital one from David Batty, thereby knocking England out of the World Cup. Beckham subsequently received death threats and was sent bullets in the post.[28][29]

text: South Korea/Japan 2002[edit]

text: In 2002 the World Cup took place in Asia for the first time. England, under its first ever foreign manager in Swedish Sven-Göran Eriksson, were able to qualify for the tournament. England were drawn in Group 9, alongside Germany, Finland, Greece and Albania. In the last ever game in the original Wembley Stadium, (which closed after the match) England played Germany, losing 1–0, the only goal scored by Dietmar Hamann. The match was the last under the management of Kevin Keegan, who resigned at the end of the match, and was replaced by Eriksson.[30] By beating Germany 5–1 in Munich, England's qualifying campaign was revitalised, and they qualified automatically, by drawing 2–2 with Greece. Germany, who could only draw 0–0 with Finland, had to play a play-off game against Ukraine, with England qualifying ahead winning the group.

text: In Japan, England had to play against Eriksson's homeland, Sweden, and both settled out for a 1–1 draw. England and Beckham gained a measure of revenge for their previous 1998 defeat in defeating Argentina 1-0, thanks to a Beckham penalty. However, England could only manage a disappointing 0-0 draw against Nigeria, meaning that although they were able to qualify for the second round, where they played Denmark, they qualified as runners up, which meant that they would meet favourites Brazil in the quarter-finals if they qualified.

text: England played Denmark in the round of 16 defeating Denmark 3–0, thanks to goals from Micheal Owen, Rio Ferdinand, and Emile Heskey.[31] England played four-time World Cup winners and 1998 runners-up Brazil in the quarter-finals. Despite leading through a Michael Owen goal, a mistake by David Seaman saw England lose 2–1, and Brazil won their fourth World Cup match against England, and went on to win the tournament.[32]

text: Germany 2006[edit]

text: England were drawn into Group 6 of European qualifying for the 2006 World Cup. The group featured other home nations in Wales, and Northern Ireland, as well as Poland (who had eliminated England the last time the World Cup took place in Germany), Azerbaijan and Austria. England won eight of the 10 games, and qualified as group winners, in front of Portugal, despite drawing to Austria in Vienna, and losing to Northern Ireland in Belfast.[33]

text: In Germany, however, England were less convincing. England played in Group B, alongside Paraguay, Trinidad and Tobago, and Sweden. England started with a 1–0 win against Paraguay; which was won due to a 3rd minute own goal.[34] The second game against first time qualifiers Trinidad and Tobago saw England have to wait until the 83rd minute for England to take the lead, with Peter Crouch opened the scoring with a goal many felt was illegal, and the second goal of the game coming in added time from Steven Gerrard.[35][36] The last group game saw England play against Sweden, where an eventual 2-2 draw saw them qualify for the next round as group winners, thereby avoiding playing hosts Germany.[37]

text: In the last 16 stage, a free kick from David Beckham saw England win 1–0 against Ecuador and reach the quarter-finals, where they faced Portugal.[38] The game finished goalless, and England once again were knocked out on penalties and Portuguese goalkeeper Ricardo became the first goalkeeper to save three penalties in a penalty shoot-out. Ricardo saved from Frank Lampard, Steven Gerrard, and Jamie Carragher; the only England player who successfully converted his penalty was Owen Hargreaves. Portugal won the shoot-out 3-1, despite misses from Petit and Hugo Viana. This game was also Erickson's final match as England manager.[39]

text: South Africa 2010[edit]

text: Qualification for the first African World Cup went successfully for new England manager Fabio Capello, after previous manager Steve McClaren was unable to secure qualification to the Euro 2008. By winning nine times and only losing to Ukraine, England qualified ahead of Croatia, Belarus, Kazakhstan and Andorra. England's group was seen as a favourable one, containing comparatively much weaker teams.[40] However, England opened up their campaign with a 1–1 draw against the United States, thanks to a major error by goalkeeper Robert Green. They then drew 0–0 against Algeria and were booed off the field by their own fans, drawing the ire of striker Wayne Rooney.[41] England eventually qualified for the next round by beating Slovenia 1–0, but only qualified as runners up to the United States, thereby meaning they would draw favourites Germany.

text: In the second round match, Germany took the lead after 20 minutes after goalkeeper Manuel Neuer played the ball down the pitch to Miroslav Klose, who opened the scoring. The score became 2–0 to Germany after 32 minutes. Shortly after, England defender Matthew Upson scored a header. Later, Frank Lampard had a shot at goal which was disallowed despite crossing the line; which was confirmed on replays. German goalkeeper Manuel Neuer admitted subsequently he knew the ball had crossed the line, but decided to deceive the referee.[42] The German media reported it was "revenge for Wembley", while the English media criticised FIFA's refusal to implement goal-line technology. Ironically, despite his earlier opposition to goal-line technology, Sepp Blatter said that it should be introduced after a Ukrainian goal against England at Euro 2012 was ruled out.[43][42][44] As England tried to equalise, Germany used this to their advantage and scored two more goals. This became Germany's biggest win against England in a World Cup, winning 4-1.[45]

text: Brazil 2014[edit]

text: Under Roy Hodgson, who replaced Fabio Capello after Euro 2012 after a disagreement between Capello and The FA, England qualified for the second World Cup to be held in Brazil. Ukraine were again one of the opponents in the qualifying rounds. The other opponents included Montenegro, Poland, Moldova and San Marino. After winning six games and drawing four, England qualified unbeaten.[46]

text: The draw for the finals saw England have to play against Italy and Uruguay, both former world champions, which meant that it was the first ever time three World Champions were drawn in the same group, along with Costa Rica. England lost to Italy and Uruguay, and were thus knocked out after two games. The final match against Costa Rica finished as a goalless draw. This performance was statistically their worst ever performance at a World Cup, ending up with just one point after two losses to Uruguay and Italy and a goalless draw with Costa Rica in the dead rubber match, their lowest points total in the group stage of a world cup.[46]

text: Russia 2018[edit]

text: England played in UEFA Group F in qualification for the 2018 World Cup, in a group of six with Slovakia, Slovenia, Scotland, Lithuania and Malta, with only the winner of the group guaranteed qualification. England went into the qualification process under manager Sam Allardyce, only for Allardyce to leave the post after just one game due to controversy regarding discussing breaking FIFA rules.[47]

text: Under Allardyce's replacement, Gareth Southgate, England went undefeated throughout qualification, winning eight matches out of 10, drawing with Slovenia 0–0 in Ljubljana, and drawing 2–2 with Scotland in Glasgow thanks to a 90th-minute equaliser from Harry Kane.[48] This was the third successive major tournament that England were undefeated, having been undefeated in 2014 World Cup, and Euro 2016 qualifying.

text: 2018 Finals[edit]

text: Under Gareth Southgate, the England team began their tournament in group G against Tunisia. The game started well for England with a goal from Harry Kane in the 11th minute. Tunisia equalised through Sassi from the penalty spot in the 35th minute following a foul by Kyle Walker. Some controversy followed as various potential offences against Harry Kane, inside the penalty area were ignored by the referee and no VAR checks were carried out. England persevered and scored a second goal in the 91st minute, again by Harry Kane, resulting in a 2–1 victory.[49]

text: In the second group stage match, England surpassed their record for goals scored in a World Cup match by beating Panama 6-1. Jesse Lingard scored the third goal, John Stones scored the first and fourth goals and Harry Kane scored a hat-trick with the second, fifth and sixth goals.[50]

text: In the final group stage match, England narrowly lost 1-0 to Belgium. Adnan Januzaj scored the sole goal of the game; but with both teams having fielded reserve teams, with England specificially making 9 changes. Before the game media outlets stating that a loss could potentially become beneficial, as the winner would be in the half of the draw with four of the top seven sides in the world.[c][51][52] The result that meant Belgium topped the group and England finished second, setting up a last 16 clash with Group H winners Colombia.[53][54]

text: England played their last 16 match in the Otkritie Arena, Moscow, with the same team as against Tunisia. Harry Kane scored his sixth goal of the tournament, and the third penalty, after once again being fouled in the box from a corner, similarly as against Panama.[55] The score remained at 1-0, until stoppage time, where a header from Yerry Mina beat Jordan Pickford in goal to bring the game to extra time. Neither team managed to score in extra time, and the match went to penalties, which England won 4-3.[56] As well as being the first knockout match England had won at a major tournament since 2006 (last defeating Ecuador in the last 16); it was the first time England won a World Cup penalty shoot-out.[d][58] The match was notable for the heated atmosphere the game was played in, with a total of eight yellow cards being shown in the match.[e][59]

text: England played against Sweden in their quarter-final at Cosmos Arena, Samara on 7 July 2018.[57] They won the match 2–0, with defender Harry Maguire scoring his first England goal, a header scored from a corner, and Dele Alli a second header from close range. This would send them through to their third World Cup semi-final and their first since 1990, and third overall.[60] The team played Croatia in the semi-final, resulting in a 2–1 loss after extra time.[61] England would later finish fourth in the competition, the best result since 1990. England would lose again to Belgium in the 3rd place playoff, thanks to goals from Thomas Meunier and Eden Hazard, despite a Eric Dier shot being cleared off the line by Toby Alderweireld.[62]

text: The tournament would see England score nine goals from set-pieces – the most by a team in a single World Cup tournament since 1966.[63]

text: Records[edit]

text:
Year
Round
Position
GP
W
D*
L
GF
GA
GD

 1930
Not a FIFA member

 1934

 1938

 1950
Group stage
8th
3
1
0
2
2
2
0

 1954
Quarter-finals
7th
3
1
1
1
8
8
0

 1958
Group stage
11th
4
0
3
1
4
5
-1

 1962
Quarter-finals
8th
4
1
1
2
5
6
-1

 1966
Champions
1st
6
5
1
0
11
3
8

 1970
Quarter-finals
8th
4
2
0
2
4
4
0

 1974
Did not qualify

 1978

 1982
Second group stage
6th
5
3
2
0
6
1
5

 1986
Quarter-finals
8th
5
2
1
2
7
3
4

 1990
Semi finals
4th
7
3
3
1
8
6
2

 1994
Did not qualify

 1998
Round of 16
9th
4
2
1
1
7
4
3

  2002
Quarter-finals
6th
5
2
2
1
6
3
3

 2006
Quarter-finals
7th
5
3
2
0
6
2
4

 2010
Round of 16
13th
4
1
2
1
3
5
-2

 2014
Group stage
26th
3
0
1
2
2
4
-2

 2018
Semi-finals
4th
7
3
1
3
12
8
4

 2022
TBD

   2026

Total
15/21
1 title
69
29
21
19
91
64
27

text:
showEngland's World Cup record

First Match

 England 2–0 Chile  (25 June 1950; Rio de Janeiro, Brazil)

Biggest Win

 England 6–1 Panama  (24 June 2018; Nizhny Novgorod, Russia)

Biggest Defeat

 England 1-4 Germany  (27 June 2010; Bloemfontein, South Africa)

Best Result

Champions at the 1966 World Cup

Worst Result

Group stage in 1950, 1958 and 2014

text: By match[edit]

text:
Year

Round

Against

Score

Scorers

 1950
Group 2
 Chile
2–0
Mortensen, Mannion

 United States
0–1

 Spain
0–1

 1954
Group 4
 Belgium
4–4 (aet)
Broadis (2), Lofthouse (2)

  Switzerland
2–0
Mullen, Wilshaw

Quarter-final
 Uruguay
2–4
Lofthouse, Finney

 1958
Group 4
 Soviet Union
2–2
Kevan, Finney

 Brazil
0–0

 Austria
2–2
Haynes, Kevan

Play-off
 Soviet Union
0–1

 1962
Group 4
 Hungary
1–2
Flowers

 Argentina
3–1
Flowers, Charlton, Greaves

 Bulgaria
0–0

Quarter-final
 Brazil
1–3
Hitchens

 1966
Group 1
 Uruguay
0–0

 Mexico
2–0
Charlton, Hunt

 France
2–0
Hunt (2)

Quarter-final
 Argentina
1–0
Hurst

Semi-final
 Portugal
2–1
Charlton (2)

Final
 West Germany
4–2 (aet)
Hurst (3), Peters

 1970
Group 3
 Romania
1–0
Hurst

 Brazil
0–1

 Czechoslovakia
1–0
Clarke

Quarter-final
 West Germany
2–3 (aet)
Mullery, Peters

 1982
Group 4
 France
3–1
Robson (2), Mariner

 Czechoslovakia
2–0
Francis, Barmoš (o.g.)

 Kuwait
1–0
Francis

Group B Round 2
 West Germany
0–0

 Spain
0–0

 1986
Group F
 Portugal
0–1

 Morocco
0–0

 Poland
3–0
Lineker (3)

Round of 16
 Paraguay
3–0
Lineker (2), Beardsley

Quarter-final
 Argentina
1–2
Lineker

 1990
Group F
 Republic of Ireland
1–1
Lineker

 Netherlands
0–0

 Egypt
1–0
Wright

Round of 16
 Belgium
1–0 (aet)
Platt

Quarter-final
 Cameroon
3–2 (aet)
Platt, Lineker (2)

Semi-final
 West Germany
1–1 (aet)[f]
Lineker

Third-place play-off
 Italy
1–2
Platt

 1998
Group G
 Tunisia
2–0
Shearer, Scholes

 Romania
1–2
Owen

 Colombia
2–0
Anderton, Beckham

Round of 16
 Argentina
2–2 (aet)[f]
Shearer, Owen

  2002
Group F
 Sweden
1–1
Campbell

 Argentina
1–0
Beckham

 Nigeria
0–0

Round of 16
 Denmark
3–0
Ferdinand, Owen, Heskey

Quarter-final
 Brazil
1–2
Owen

 2006
Group B
 Paraguay
1–0
Gamarra (o.g.)

 Trinidad and Tobago
2–0
Crouch, Gerrard

 Sweden
2–2
Cole, Gerrard

Round of 16
 Ecuador
1–0
Beckham

Quarter-final
 Portugal
0–0 (aet)[f]

 2010
Group C
 United States
1–1
Gerrard

 Algeria
0–0

 Slovenia
1–0
Defoe

Round of 16
 Germany
1–4
Upson

 2014
Group D
 Italy
1–2
Sturridge

 Uruguay
1–2
Rooney

 Costa Rica
0–0

 2018
Group G
 Tunisia
2–1
Kane (2)

 Panama
6–1
Stones (2), Kane (3), Lingard

 Belgium
0–1

Round of 16
 Colombia
1–1 (aet)[g]
Kane

Quarter-final
 Sweden
2–0
Maguire, Alli

Semi-final
 Croatia
1–2 (aet)
Trippier

Third-place play-off
 Belgium
0–2

text: By opponent[edit]

text:
Country

Played

Won

Drawn

Lost

GF

GA

GD

 Argentina
5
3
1
1
8
5
+3

 Germany
5
1
2
2
8
10
-2

 Brazil
4
0
1
3
2
6
-4

 Sweden
3
1
2
0
5
3
+2

 Belgium
4
1
1
2
5
7
-2

 Portugal
3
1
1
1
2
2
0

 Uruguay
3
0
1
2
3
6
-3

 France
2
2
0
0
5
1
+4

 Paraguay
2
2
0
0
4
0
+4

 Tunisia
2
2
0
0
4
1
+3

 Czechoslovakia
2
2
0
0
3
0
+3

 Colombia
2
1
1
0
3
1
+2

 Romania
2
1
0
1
2
2
0

 Soviet Union
2
0
1
1
2
3
-1

 United States
2
0
1
1
1
2
-1

 Spain
2
0
1
1
0
1
-1

 Italy
2
0
0
2
2
4
-2

 Panama
1
1
0
0
6
1
+5

 Poland
1
1
0
0
3
0
+3

 Denmark
1
1
0
0
3
0
+3

 Chile
1
1
0
0
2
0
+2

  Switzerland
1
1
0
0
2
0
+2

 Mexico
1
1
0
0
2
0
+2

 Trinidad and Tobago
1
1
0
0
2
0
+2

 Cameroon
1
1
0
0
3
2
+1

 Kuwait
1
1
0
0
1
0
+1

 Egypt
1
1
0
0
1
0
+1

 Ecuador
1
1
0
0
1
0
+1

 Slovenia
1
1
0
0
0
0
+1

 Austria
1
0
1
0
2
2
0

 Republic of Ireland
1
0
1
0
1
1
0

 Bulgaria
1
0
1
0
0
0
0

 Morocco
1
0
1
0
0
0
0

 Netherlands
1
0
1
0
0
0
0

 Nigeria
1
0
1
0
0
0
0

 Algeria
1
0
1
0
0
0
0

 Costa Rica
1
0
1
0
0
0
0

 Hungary
1
0
0
1
1
2
-1

 Croatia
1
0
0
1
1
2
-1

text: Last update: 14 July 2018

text: Players' honours[edit]

text: List of England players with the most appearances at World Cups[edit]

text:
No.

Name

Matches

World Cups

1
Peter Shilton
17
1982, 1986, 1990

2

Terry Butcher
14
1982, 1986, 1990

Bobby Charlton
14
1958, 1962, 1966, 1970

Ashley Cole
14
2002, 2006, 2010

Bobby Moore
14
1962, 1966, 1970

6
David Beckham
13
1998, 2002, 2006

7

Steven Gerrard
12
2006, 2010, 2014

Gary Lineker
12
1986, 1990

Michael Owen
12
1998, 2002, 2006

10
Chris Waddle
11
1986, 1990

text: List of England top goalscorers at World Cups[edit]

text:
No.

Name

Goals

World Cups

1
Gary Lineker
10
1986 (6) and 1990 (4)

2
Harry Kane
6
2018

3
Geoff Hurst
5
1966 (4) and 1970 (1)

4

Bobby Charlton
4
1962 (1) and 1966 (3)

Michael Owen
4
1998 (2) and 2002 (2)

6

Nat Lofthouse
3
1954

Roger Hunt
3
1966

David Platt
3
1990

David Beckham
3
1998, 2002 and 2006

Steven Gerrard
3
2006 (2) and 2010

11
Nine players
2

text: Current as of 3 July 2018 after the match against Colombia

text: List of England goals by tournament[edit]

text:
World Cup

Goalscorer(s)

1950

Wilf Mannion, Stan Mortensen

1954

Nat Lofthouse (3), Ivor Broadis (2), Tom Finney, Jimmy Mullen, Dennis Wilshaw

1958

Derek Kevan (2), Tom Finney, Johnny Haynes

1962

Ron Flowers (2), Bobby Charlton, Jimmy Greaves, Gerry Hitchens

1966

Geoff Hurst (4), Roger Hunt (3), Bobby Charlton (3), Martin Peters

1970

Allan Clarke, Geoff Hurst, Alan Mullery, Martin Peters

1982

Trevor Francis (2), Bryan Robson (2), Paul Mariner, Own Goal

1986

Gary Lineker (6), Peter Beardsley

1990

Gary Lineker (4), David Platt (3), Mark Wright

1998

Michael Owen (2), Alan Shearer (2), Darren Anderton, David Beckham, Paul Scholes

2002

Michael Owen (2), David Beckham, Sol Campbell, Rio Ferdinand, Emile Heskey

2006

Steven Gerrard (2), David Beckham, Joe Cole, Peter Crouch, Own Goal

2010

Jermain Defoe, Steven Gerrard, Matthew Upson

2014

Wayne Rooney, Daniel Sturridge

2018

Harry Kane (6), John Stones (2), Jesse Lingard, Harry Maguire, Dele Alli, Kieran Trippier

text: List of England players playing abroad at a World Cup[edit]

text: Historically, very few English World Cup squad members were playing for a club in a foreign league at the time of their selection to the national squad.[64]

text:
Year

Number (Country)

Players (Apps)

1950–1958

0

1962

1 (Italy)

Gerry Hitchens (2)

1966–1970

0

1982

1 (West Germany)

Tony Woodcock (2)

1986

2 (Italy)

Mark Hateley (3), Ray Wilkins

1990

5 (4 in Scotland, 1 in France)

Terry Butcher (5), Trevor Steven (3), Gary Stevens (2), Chris Woods (0); Chris Waddle (7)

1998

0

2002

1 (Germany)

Owen Hargreaves (2)

2006

2 (1 Spain, 1 Germany)

David Beckham (5); Owen Hargreaves (4)

2010

0

2014

1 (Scotland)

Fraser Forster (0)

2018

0

text: Team awards and records[edit]

text: Team Awards

text:
World Champions 1966
FIFA Fair Play Trophy 1990
FIFA Fair Play Trophy 1998 (shared)

text: Individual Awards

text:
Golden Ball 1966: Bobby Charlton
Silver Ball 1966: Bobby Moore
Golden Boot 1986: Gary Lineker
Golden Boot 2018: Harry Kane
Bronze Boot 1966: Geoff Hurst
Golden Glove 1966: Gordon Banks
Best Young Player Award 1998: Michael Owen

text: Team Records

text:
Most penalty shoot-outs lost: 3 (shared with Italy and Spain)
Biggest World Cup victory: 6-1 vs Panama, 2018 Group Stage [65]

text: Individual Records

text:
Only hattrick in a final: Geoff Hurst (1966 v West Germany)
Oldest captain: Peter Shilton (age 40 years and 292 days, 1990 v Italy)
Most clean sheets: Peter Shilton (10, 1982-1990) (shared with Fabien Barthez)

text: Refereeing[edit]

text: Four FIFA World Cup finals were officiated by English referees, more than by any other football association. The first Englishman to officiate a final, George Reader, is also the oldest World Cup referee to date, as he was 53 years and 236 days old at the 1950 decisive match between Uruguay and Brazil. The other final referees are William Ling (1954), Jack Taylor (1974) and Howard Webb (2010).

text: Arthur Ellis, who was a linesman at the 1950 final, is part of an elite group of referees who has been called up for three consecutive World Cups (1950–1958).


document_title: Facioscapulohumeral muscular dystrophy

question_text: what disease does thomas have in the man in the high castle

root

text: Facioscapulohumeral muscular dystrophy

text: Symptoms[edit]

text: Because of the extreme variability of the disease, an authoritative and scientifically confirmed set of symptoms does not yet exist. The prevalence is widely placed at 1/20,000, but the exact prevalence is not known. A November 2008 report from Orpha.net, an organization backed by the Institut National de la Santé et de la Recherche Médicale (INSERM), listed a prevalence of 7/100,000, but the May 2014 version of this report places the prevalence at 4/100,000.[4] A 2014 population-based study in the Netherlands reported a significantly higher prevalence of 12 in 100,000.[4]

tag: p

text: Symptoms:

text:
Facial muscle weakness (eyelid drooping, inability to whistle, decreased facial expression, depressed or angry facial expression, difficulty pronouncing the letters M, B, and P)
Shoulder weakness (difficulty working with the arms raised, sloping shoulder)
Hearing loss
Abnormal heart rhythm
Unequal weakening of the biceps, triceps, deltoids, and lower arm muscles
Loss of strength in abdominal muscles (causing a protuberant abdomen and lumbar lordosis) and eventual progression to the legs
Foot drop

tag: ul

text: Genetics[edit]

text: FSHD Type 1 (also called FSHMD1A) (4q35 deletion)[edit]

text: More than 95% of cases of FSHD are associated with the deletion of integral copies of a tandemly repeated 3.2kb unit (D4Z4 repeat) at the subtelomeric region 4q35 on Chromosome 4 of the human genome, of which a normal chromosome includes between 11-150 repetitions of D4Z4.[10] There are both heterochromatin and euchromatin structures within D4Z4 and one putative gene called DUX4.[10][11] Inheritance is autosomal dominant, though up to one-third of the cases appear to be from de novo (new) mutations. The heterochromatin is specifically lost in the deletions of FSHD while the euchromatin structures remain.[10] If the entire region is removed, there are birth defects, but no specific defects on skeletal muscle. Individuals appear to require the existence of 11 or fewer repeat units to be at risk for FSHD.

text: In addition, a few cases of FSHD are the result of rearrangements between subtelomeric chromosome 4q and a subtelomeric region of 10q. This location contains a tandem repeat structure highly homologous to 4q35.[12] Disease occurs when the translocation results in a critical loss of tandem repeats to the 4q site.

text: FSHD Type 2[edit]

text: A large family was reported with a phenotype indistinguishable from FSHD in which no pathological changes at the 4q site or translocation of 4q-10q are found.[13][14] It had been suggested that this may be due to limitations in the available tests.[15]

text: In 2012, a majority of FSHD2 cases were reported linked to mutations in the SMCHD1 gene on chromosome 18. This leads to substantially reduced levels of SMCHD1 protein, and subsequently, hypomethylation of the 4q D4Z4 region. The FSHD2 phenotype arises in individuals who inherited both the SMCHD1 mutations plus a normal sized D4Z4 region on a permissive 4qA allele. This establishes a genetic/mechanistic intersection of FSHD1 and FSHD2.[16]

text: A Unifying Theory[edit]

text: On 19 August 2010, a paper entitled A Unifying Genetic Model for Facioscapulohumeral Muscular Dystrophy was published in Science showing that the candidate gene DUX4 undergoes a "toxic gain of function" as a result of single nucleotide polymorphisms in the region distal to the last D4Z4 repeat. According to the research, this leads to a "canonical polyadenylation signal for transcripts derived from DUX4".[8] This is the first time in the history of genetics in which "junk" DNA has been shown to reanimate and cause disease. The documentation of the conditions under which the DUX4 gene became reanimated answered the question of why no one whose dead gene was repeated more than 10 times ever got FSHD but only some people with fewer than 10 copies did get the disease[7] Several organizations including the New York Times highlighted this research (See MDA, FSH Society, University of Rochester, NYT).

text: Dr. Francis Collins, who oversaw the first sequencing of the Human Genome with the National Institutes of Health stated:[7]

text: “If we were thinking of a collection of the genome’s greatest hits, this would go on the list,”

text: Daniel Perez, co-founder, President and CEO of the FSH Society hailed the new findings saying:

text: "This is a long-sought explanation of the exact biological workings of a disease that affects an estimated one in 14,000 or 22,100 Americans and 490,000 worldwide,” he said, adding that this discovery “creates an enormous opportunity for research to develop ways to prevent or treat FSHD.”

text: The MDA stated that:

text: "The new findings will make it easier to diagnose FSHD in someone with symptoms and predict who will develop the disease in someone without symptoms. Now, the hunt is on for which proteins or genetic instructions (RNA) cause the problem for muscle tissue in FSHD."

text: Quoted in the University of Rochester press release, one of the report's co-authors, Silvère van der Maarel of the University of Leiden, stated that

text: “It is amazing to realize that a long and frustrating journey of almost two decades now culminates in the identification of a single small DNA variant that differs between patients and people without the disease. We finally have a target that we can go after.”

text: The original identification of the D4Z4 deletions was found in 1992. This research now shows that a second mechanism is needed for FSHD to be present and that the remaining versions of the DUX4 become more active (open for transcription) because the DNA at the tip of chromosome 4 is less tightly coiled as a result of the deletions.

text: Chronology of Important FSHD-related Genetic Research[edit]

text: Landouzy and Dejerine describe a form of childhood progressive muscle atrophy with a characteristic involvement of facial muscles and distinct from pseudohypertrophic (Duchenne’s MD) and spinal muscle atrophy in adults.[17]

text: Landouzy and Dejerine describe progressive muscular atrophy of the scapulo-humeral type.[18]

text: Tyler and Stephens study 1249 individuals from a single kindred with FSHD traced to a single ancestor and describe a typical Mendelian inheritance pattern with complete penetrance and highly variable expression. The term facioscapulohumeral dystrophy is introduced.[19]

text: Padberg provides the first linkage studies to determine the genetic locus for FSHD in his seminal thesis "Facioscapulohumeral disease."[20]

text: The complete sequence of the Dystrophin gene (Duchenne’s MD) is determined.[21]

text: The genetic defect in FSHD is linked to a region (4q35) near the tip of the long arm of chromosome 4.[22]

text: FSHD, in both familial and de novo cases, is found to be linked to a recombination event that reduces the size of 4q EcoR1 fragment to < 28 kb (50–300 kb normally).[23]

text:
4q EcoR1 fragments are found to contain tandem arrangement of multiple 3.3-kb units (D4Z4), and FSHD is associated with the presence of < 11 D4Z4 units.[24]
A study of seven families with FSHD reveals evidence of genetic heterogeneity in FSHD.[25]

text:
The heterochromatic structure of 4q35 is recognized as a factor that may affect the expression of FSHD, possibly via position-effect variegation.[26]
DNA sequencing within D4Z4 units shows they contain an open reading frame corresponding to two homeobox domains, but investigators conclude that D4Z4 is unlikely to code for a functional transcript.[26][27]

text: The terms FSHD1A and FSHD1B are introduced to describe 4q-linked and non-4q-linked forms of the disease.[28]

text: FSHD Region Gene1 (FRG1) is discovered 100 kb proximal to D4Z4.[29]

text: Monozygotic twins are identified with identical 23 kb EcoR1 fragments but vastly different clinical expression of FSHD.[30]

text: Complete sequencing of 4q35 D4Z4 units reveals a promoter region located 149 bp 5' from the open reading frame for the two homeobox domains, indicating a gene that encodes a protein of 391 amino acid protein (later corrected to 424 aa[31]), given the name DUX4.[32]

text: Investigators assessed the methylation state (heterochromatin is more highly methylated than euchromatin) of DNA in 4q35 D4Z4. An examination of SmaI, MluI, SacII, and EagI restriction fragments from multiple cell types, including skeletal muscle, revealed no evidence for hypomethylation in cells from FSHD1 patients relative to D4Z4 from unaffected control cells or relative to homologous D4Z4 sites on chromosome 10. However, in all instances, D4Z4 from sperm was hypomethylated relative to D4Z4 from somatic tissues.[33]

text:
A polymorphic segment of 10 kb directly distal to D4Z4 is found to exist in two allelic forms, designated 4qA and 4qB. FSHD1 is associated solely with the 4qA allele.[34]
Three genes (FRG1, FRG2, ANT1) located in the region just centromeric to D4Z4 on chromosome 4 are found in isolated muscle cells from individuals with FSHD at levels 10 to 60 times greater than normal, showing a linkage between D4Z4 contractions and altered expression of 4q35 genes.[35]

text: A further examination of DNA methylation in different 4q35 D4Z4 restriction fragments (BsaAI and FseI) showed significant hypomethylation at both sites for individuals with FSHD1, non-FSHD-expressing gene carriers, and individuals with phenotypic FSHD relative to unaffected controls.[36]

text: Contraction of the D4Z4 region on the 4qB allele to < 38 kb does not cause FSHD.[37]

text: Transgenic mice overexpressing FRG1 are shown to develop severe myopathy.[38]

text:
The DUX4 open reading frame is found to have been conserved in the genome of primates for over 100 million years, supporting the likelihood that it encodes a required protein.[39]
Researchers identify DUX4 mRNA in primary FSHD myoblasts and identify in D4Z4-transfected cells a DUX4 protein, the overexpression of which induces cell death.[31]
DUX4 mRNA and protein expression are reported to increase in myoblasts from FSHD patients, compared to unaffected controls. Stable DUX4 mRNA is transcribed only from the most distal D4Z4 unit, which uses an intron and a polyadenylation signal provided by the flanking pLAM region. DUX4 protein is identified as a transcription factor, and evidence suggests overexpression of DUX4 is linked to an increase in the target paired-like homeodomain transcription factor 1 (PITX1).[40]

text:
The terms FSHD1 and FSHD2 are introduced to describe D4Z4-deletion-linked and non-D4Z4-deletion-linked genetic forms, respectively. In FSHD1, hypomethylation is restricted to the short 4q allele, whereas FSHD2 is characterized by hypomethylation of both 4q and both 10q alleles.[41]
Splicing and cleavage of the terminal (most telomeric) 4q D4Z4 DUX4 transcript in primary myoblasts and fibroblasts from FSHD patients is found to result in the generation of multiple RNAs, including small noncoding RNAs, antisense RNAs and capped mRNAs as new candidates for the pathophysiology of FSHD.[42]

text:
A unifying genetic model of FSHD describes the requirement for FSHD-type deletions of D4Z4 to occur on a permissive allele containing a poly-A adenylation signal (PAS) in the pLAM1 region adjacent to the final D4Z4 unit. The non-permissive 4qB allele lacks a PAS, does not generate a stable DUX4 transcript, and is not linked to FSHD. The corresponding D4Z4 region on chromosome 10 (10q26) lacks a PAS altogether, and deletions in this region are not involved in FSHD.[43]
DUX4 is found actively transcribed in skeletal muscle biopsies and primary myoblasts. FSHD-affected cells produce a full length transcript, DUX4-fl, whereas alternative splicing in unaffected individuals results in the production of a shorter, 3'-truncated transcript (DUX4-s). The low overall expression of both transcripts in muscle is attributed to relatively high expression in a small number of nuclei (~ 1 in 1000). Higher levels of DUX4 expression in human testis (~100 fold higher than skeletal muscle) suggest a developmental role for DUX4 in human development. Higher levels of DUX4-s (vs DUX4-fl) are shown to correlate with a greater degree of DUX-4 H3K9me3-methylation.[44]

text:
Some, but not all, instances of FSHD2 are linked to mutations in the SMCHD1 gene on chromosome 18. This leads to substantially reduced levels of SMCHD1 protein, and subsequently, hypomethylation of the 4q D4Z4 region. The FSHD2 phenotype arises in individuals who inherited both the SMCHD1 mutations plus a normal sized D4Z4 region on a permissive 4qA allele. This establishes a genetic/mechanistic intersection of FSHD1 and FSHD2.[16]
The prevalence of FSHD-like D4Z4 deletions on permissive alleles is significantly higher than the prevalence of FSHD in the general population, challenging the criteria for molecular diagnosis of FSHD.[45]
When expressed in primary myoblasts, DUX4-fl acted as a transcriptional activator, producing a > 3-fold change in the expression of 710 genes.[46] A subsequent study using a larger number of samples identified DUX4-fl expression in myogenic cells and muscle tissue from unaffected relatives of FSHD patients, per se, is not sufficient to cause pathology, and that additional modifiers are determinants of disease progression.[47]
A mechanism is proposed in which DBE-T (D4Z4 Regulatory Element transcript), a long non-coding RNA transcribed from a 4q35 region proximal to D4Z4, is expressed in FSHD, leading to the recruitment of the Trithorax group protein Ash1L, an increase in H3k36me2-methylation, and ultimately de-repression of 4q35 genes.[48]

text:
Mutations in SMCHD1 are shown to act as a disease modifier, increasing the severity of FSHD1 in individuals who also exhibit a contraction of D4Z4.[49]
Transgenic mice carrying D4Z4 arrays from an FSHD1 allele (with 2.5 D4Z4 units), although lacking an obvious FSHD-like skeletal muscle phenotype, are found to recapitulate important genetic expression patterns and epigenetic features of FSHD.[50]

text:
DUX4-fl and downstream target genes are expressed in skeletal muscle biopsies and biopsy-derived cells of fetuses with FSHD-like D4Z4 arrays, indicating that molecular markers of FSHD are already expressed during fetal development.[51]
In an open access article in Skeletal Muscle, researchers "review how the contributions from many labs over many years led to an understanding of a fundamentally new mechanism of human disease" and articulate how the unifying genetic model and subsequent research represent a "pivot-point in FSHD research, transitioning the field from discovery-oriented studies to translational studies aimed at developing therapies based on a sound model of disease pathophysiology." They describe the consensus mechanism of pathophysiology for FSHD as a "inefficient repeat-mediated epigenetic repression of the D4Z4 macrosatellite repeat array on chromosome 4, resulting in the variegated expression of the DUX4 retrogene, encoding a double-homeobox transcription factor, in skeletal muscle." [9]

text: FSH Society[edit]

text: In 1991 the FSH Society was founded by two individuals with FSHD, Daniel Perez and Stephen Jacobsen. The FSH Society raised funding to provide seed grants for FSHD research, advocated for the field to standardize the name of the disease as "facioscapulohumeral muscular dystrophy" and "FSHD", and co-wrote the MD-CARE Act, passed into law in 2001, which for the first time mandated federal resources, including National Institutes of Health funding, for all muscular dystrophies. The FSH Society has grown into the world's largest grassroots organization advocating for patient education and scientific and medical research.[52]

text: FSHD Foundation[edit]

text: In 2007 the FSHD Global Research Foundation was established to increase the amount of funding available to research bodies. The Foundation has identified 13 priority areas of interest for FSHD research.[53]

text: FSHD-EUROPE[edit]

text: In 2009 the FSHD-EUROPE was founded by European associations.[54]

text: Pathophysiology[edit]

text: In 2014, researchers undertook a "review [of] how the contributions from many labs over many years led to an understanding of a fundamentally new mechanism of human disease" and articulated how the unifying genetic model and subsequent research represent a "pivot-point in FSHD research, transitioning the field from discovery-oriented studies to translational studies aimed at developing therapies based on a sound model of disease pathophysiology." They proposed a consensus mechanism of pathophysiology for FSHD as a "inefficient repeat-mediated epigenetic repression of the D4Z4 macrosatellite repeat array on chromosome 4, resulting in the variegated expression of the DUX4 retrogene, encoding a double-homeobox transcription factor, in skeletal muscle." [9]

text: In more lay terms, the D4Z4 repeats (most people have about 200 or so) normally keep DUX4 repressed (the repeat-mediated repression). When there are drastically fewer repeats (approximately 10 or less) in addition to the small genetic change on Chromosome 4 called a haplotype polymorphism, DUX4 expresses itself (the inefficient repression component) via a complex set of mechanisms that make the genetic neighborhood around the DUX4 gene more conducive to gene expression (the epigenetic component). The figure on the right describes this process in detail.

text: Testing[edit]

text: Since the early 2000s, genetic testing that measures the size of the D4Z4 deletions on 4q35 has become the preferred mechanism for confirming the presence of FSHD. As of 2007, this test is considered highly accurate but is still performed by a limited set of labs in the US, such as Athena diagnostics under test code 405. However, because the test is expensive, patients and doctors may still rely on one or more of the following tests, all of which are far less accurate and specific than the genetic test:[55]

text:
Creatine kinase (CK) level: This test measures the Creatine kinase enzyme in the blood. Elevated levels of CK are related to muscle atrophy.
electromyogram (EMG): This test measures the electrical activity in the muscle
nerve conduction velocity (NCV): This test measures the how fast signals travel from one part of a nerve to another. The nerve signals are measured with surface electrodes (similar to those used for an electrocardiogram), and the test is only slightly uncomfortable.
muscle biopsy: Through outpatient surgery a small piece of muscle is removed (usually from the arm or leg) and evaluated with a variety of biochemical tests. Researchers are attempting to match results of muscle biopsies with DNA tests to better understand how variations in the genome present themselves in tissue anomalies.

text: Therapies[edit]

text:
No approved therapies exist specifically for FSHD.
Based on the consensus model of pathophysiology, researchers propose four approaches for therapeutic intervention:[9]

enhance the epigenetic repression of the D4Z4
target the DUX4 mRNA, including altering splicing or polyadenylation;
block the activity of the DUX4 protein
inhibit the DUX4-induced process, or processes, that leads to pathology.

Physiotherapy could improve patients' functional status by providing therapeutic exercises.
Occupational therapy can sometimes be used for ADL training and to help cope with new devices to make things easier.
Several compounds aimed at increasing muscle mass have been pursued including:

ACVR2B is a compound identified in 2005/2006 by researchers at Johns Hopkins[1]. It increased muscle mass in a non-muscular dystrophy mouse by up to 60% in two weeks.
MYO-029 (Stamulumab) is an experimental myostatin-inhibiting drug developed by Wyeth Pharmaceuticals for the treatment of muscular dystrophy. Myostatin is a protein that inhibits the growth of muscle tissue. MYO-029 is a recombinant human antibody designed to bind and inhibit the activity of myostatin.[56] A 2005/2006 study was completed by Wyeth in Collegeville, PA, and included participants afflicted with FSHD. The study could not prove its efficacy and did not proceed further.

aTyr Pharma First FSHD Patient Study of ResolarisTM -- First Physiocrine-Based Therapeutic Administered to Patients.[57]

"The Phase 1b/2 study is a double-blind, placebo-controlled, multiple ascending dose trial in up to 44 FSHD patients at multiple sites in the European Union. The exploratory trial is designed to evaluate safety, tolerability, pharmacokinetics and the biological activity of ResolarisTM in adult patients with FSHD. Our ResolarisTM FSHD trial represents the first patient administration of a naturally occurring protein derived from a new class of physiological modulators, Physiocrines. We believe this trial will be an important step in our plan to develop new medicines that will have a meaningful impact for patients by activating physiological pathways important to skeletal muscle health.[58]

text: Procedures used to improve quality of life[edit]

text:
Quality of life can be measured with specific questionnaires.[59]
Scapular fusion: surgical fusion of the scapula to the thorax.
Scapular bracing: a scapular brace helps stabilize the shoulder and correct glenohumeral positioning.

text: History[edit]

text: FSHD was first described in 1884 by French physicians Louis Landouzy and Joseph Dejerine. In their paper of 1886, Landouzy and Dejerine drew attention to the familial nature of the disorder and mentioned that four generations were affected in the kindred that they had investigated.[60] Formal definition of FSHD's clinical features didn't occur until 1952 when a large Utah family with FSHD was studied. Beginning about 1980 an increasing interest in FSHD led to increased understanding of the great variability in the disease and a growing understanding of the genetic and pathophysiological complexities. By the late 1990s, researchers were finally beginning to understand the regions of Chromosome 4 associated with FSHD.[10]

text: Since the publication of the unifying theory in 2010, researchers continued to refine their understanding of DUX4. With increasing confidence in this work, researchers proposed the first a consensus view in 2014 of the pathophysiology of the disease and potential approaches to therapeutic intervention based on that model.[9]

text: A chronology of important milestones in the history of genetic research related to FSHD is included below in the Genetics section.

text: Over the years, FSHD has, at various times, been referred to as:

text:
Landouzy-Dejerine[2]
Landouzy-Dejerine syndrome[60]
Erb-Landouzy-Dejerine syndrome[60]
Landouzy-Dejerine dystrophy or atrophy[60]
faciohumeroscapular

text: In Fiction[edit]

text: In the Amazon Video series The Man in the High Castle, Obergruppenführer John Smith's son, Thomas, is diagnosed with Landouzy-Dejerine syndrome. In the alternate history of the series in which the Axis powers won World War II, Nazi policy is to euthanize those affected by this and other serious conditions to preclude their "drag on society."