داء هنتنغتون

داء هنتنغتون هو مرض عقلي وراثي يشابه تدهور مرحلي للحالة العقلية، بسبب موت خلايا في المخ. هو مرض تنكسي مترقٍ يُسبب تلف خلايا عصبية معينة في الدماغ، ونتيجة لذلك تظهر حركات لا إرادية واضطرابات عاطفية وتدهور في الحالة العقلية.

يصاب مريض داء هنتنغتون بالخرف، وفقد الذاكرة. تختلف نسبة حدوث المرض من منطقة لأخرى ،لكن الإحصائيات في الغرب وحده، تقدر إصابة 5-8 أشخاص لكل 100 ألف شخص. الأعراض الظاهرة للمرض، تتسم بخلل حركي لا إرادي. وصف المرض لأول مرة 1872 على يد الطبيب النيويوركي جورج هانتينغتون ومن هنا اكتسب المرض هذا الاسم، فيدعى أحيانا بمرض هنتنغتون أو تناذر هنتنغتون. يبدأ الداء بشكل تدريجي بحركات تشنجية يصحبها تغيرات عقلية كفقدان الذاكرة واضطراب الشخصية وأيضا حركات سريعة مفاجأة في الجسم لذا وصف بالرقاص. وهو داء وراثي إلى حد كبير لم يتم اكتشاف أي علاج له، ويستطيع الأطباء إعطاء عمر متوقع للمريض عند تشخيصه بالحالة.
أسباب وراثية
ينتقل المرض بمؤثرات وراثية، مع ولوج كامل ،إذ أنه يكفي الاليل (Allele) واحد للجين المصاب، لظهور المرض بالحامل، بمعنى آخر المرض، دائما ما يظهر لدى الشخص الحامل للجين المصاب. داء هنتنغتون يعتبر المرض الوحيد المعروف المحدد، الذي لا يوجد فرق بين حامل نسخة واحدة مصابة وبين حامل نسختين مصابتين، فالجين المصاب موجود على الكروموزوم 4، ويقوم بترميز بروتين باسم هنتنغتون.وهو بروتين بوزن 350 وحدة كتل ذرية وهو فعال في سيتو بلأزمات الخلية العصبية في المخ. وهو عيب يسبب تجمع بروتين زائد في المخ وهي زيادة تؤدي لموت الخلية في المخ. وهو عيب يظهر خلاله رجوع ثلاثة نيوكليوتايد (CAG) مرة تلو الأخرى فتكون النتيجة إنتاج حامض أمينو جلوتامين. وفي حال وجود أكثر من 40 رجوع يظهر المرض وتكون أعراضه صعبة، وظهوره يكون في سن مبكرة أكثر بمرور الأجيال.

الأعراض
تظهر أعراض المرض بصورة عامة في أواسط العقد الرابع أي 35 فما فوق ولكنها يمكن أن تبدأ في أي سن من الطفولة حتى الشيخوخة. في المراحل المبكرة هناك بعض التغييرات الطفيفة في الشخصية، الإدراك والمهارات الجسدية. الأعراض الجسدية تلاحظ أولاً بما أن الأعراض النفسية والإدراكية هي غير قوية لدرجة أن تلاحظ في المراحل الابتدائية. الأعراض الجسدية الأولية الأكثر وضوحاً هي الحركات التشنجية العشوائية التي تعرف بمرض عصبي الرقاص فتطرأ تغييرات عقلية مثل عصبية زائدة، تشنج، اكتئاب أو حركات لا ارادية للوجه والجسم ومن الجدير ذكره هنا أن حوالي 10% من المرضى التي لديهم رجعات كافية تظهر لديهم علامات المرض في سن مبكرة أكثر قبل سن 20 إذ تظهر الأعراض على هيئة تصلب عضلات. يتقدم المرض ببطء ولكن دون توقف ويقدر معدل الحياة بعد ظهور الأعراض الأولية 15 -20 عام ومع الوقت يستفحل المرض وتشتد أعراضه سيما الحركات اللا إرادية التي تنتشر من الوجه والأصابع إلى بقية الجسم ويتجلى الخرف ومشاكل الذاكرة وصعوبة الكلام تشتد وتتفاقم حالة الأكتئاب في النهاية يصل المريض لمرحلة يكون فيها ملتزم الفراش وغير قادر على الحركة وفي هذه المرحلة يحدث الموت ليس لنتيجة العيب الجيني الذي سبب ظهور المرض بل نتيجة الاختناق أو قصور القلب أو الالتهاب الرئوي الحاد الخ.

يمكن أن تتنوع أعراض وعلامات داء هنتينغتون بشكل واضح من شخص لآخر، ويتطور داء هنتينغتون ببطء عادةً، وتتعلق شدة الأعراض والعلامات بدرجة تلف الخلية العصبية. تحدث الوفاة بهذا المرض عادةً بعد حوالي 10 – 30 سنة من ظهور الأعراض، وقد يكون تطوّر المرض أسرع لدى الناس الأصغر سناً.

الوصف
داء هنتينغتون مرض وراثي، تظهر أعراضه وعلاماته عادةً في منتصف العمر، وعادةً ما تكون وطأة المرض أشد وتطوّر الأعراض أسرع إذا أصاب الأعمار الأصغر سناً، ونادراً ما يُصاب الأطفال بداء هنتينغتون.

تتوفر الأدوية للمساعدة في تدبير أعراض وعلامات داء هنتينغتون، ولكن العلاج لا يمنع التدهور العقلي والجسدي المرافق لهذه الحالة.

تتضمن الأعراض والعلامات الباكرة لداء هنتينغتون
تغيرات شخصية، مثل الاهتياج أو الغضب أو الاكتئاب أو فقدان الاهتمام.
نقص القدرات المعرفية، مثل صعوبة اتخاذ القرارات أو تعلم معارف جديدة أو الإجابة على الأسئلة وتذكر المعلومات الهامة.
مشاكل بسيطة في التوازن.
حركات خرقاء.
حركات وجهية لا إرادية، مثل التكشير.
تتضمن الأعراض والعلامات اللاحقة لداء هنتينغتون
حركات لا إرادية تشنجية مفاجئة في الجسم (رقص).
مشاكل خطيرة في التوازن والتنسيق.
حركات عين سريعة ومتشنجة.
كلام بطيء أو متردد أو متلعثم.
مشاكل البلع.
عته.
أعراض وعلامات داء هنتينغتون لدى الشباب التي تشابه أعراض وعلامات داء باركنسون
صلابة عضلية.
رعاش.
حركات بطيئة.
يمكن أن تحدث النوبات أيضاً لدى المرضى الذين ظهرت عندهم أعراض باكرة لداء هنتينغتون.

يجب استشارة الطبيب عند ملاحظة تغيّرات في الحركة أو السيطرة العاطفية أو القدرة العقلية، ويمكن أن تكون هذه الأعراض والعلامات نتيجة حالات عديدة لذا ليس من الضرورة أن تشير لداء هنتينغتون.

يمكن مناقشة الطبيب في حال وجود قصة عائلية لداء هنتينغتون حول اختبار المورثات الذي يستطيع تحديد وجود أو عدم وجود المورثة المُصابة. إنَّ قرار إجراء الفحص الجيني هو قرار شخصي، إذ أنَّ عدم معرفة البعض بوجود المورثة المعيبة لديهم يوترهم ويشتتهم، وبالمقابل فإنَّ البعض الآخر يرهق من معرفة أن الحالة سوف تتطوّر لديهم، في حال الشك فيما إذا كان الاختبار مناسباً للشخص يمكن عندها استشارة الخبير الوراثي الذي قد يساعد في تفسير نتائج الاختبار السلبية أو الإيجابية ويساعد خلال إجراء الاختبار وفيما بعد لمعرفة منافع ومضار الاختبار.

الأسباب
إن داء هنتينغتون هو مرض وراثي ينتج عن مورثة شاذة واحدة. يشير الأطباء للمرض كاضطراب صبغي جسمي سائد لأن نسخة واحدة من المورثة المعيبة تورث من أحد الأبوين، تكفي لتُسبب المرض، إذا كان لدى أحد الأبوين مورثة معيبة مفردة فإن نسبة النسل المصاب هي 50 %، بما أن الأعراض والعلامات عادةً لا تظهر حتى منتصف العمر، فإنَّ بعض الآباء قد لا يعرفون بأنهم يحملون المورثة حتى بعد إنجابهم الأولاد مع احتمال توريثهم لهذه المورثة.
إذا لم يرث الطفل المورثة المعيبة، فإنه لن يُصاب بداء هنتينغتون ولن يورثه للجيل التالي. في النهاية، سوف يظهر المرض لدى كل شخص يحمل المورثة إذا عاش لفترة طويلة بما فيه الكفاية.
اكتشفت الباحثون وجود بروتين يُعبر عنه بمورثة داء هنتينغتون المعيبة ويتفاعل مع بروتين آخر ليغير من طريقة تراكم الكولسترول في الدماغ، يعتبر الكولسترول ضرورياً لخلايا الدماغ السليمة وللشبكة الموجودة بين هذه الخلايا، لكن يجب أن يتواجد الكولسترول بكميات مناسبة وفي المكان المناسب، وعندما تتشوش شبكة الخلايا الدماغية، قد تتأثر المهارات الحركية والمعرفية والكلامية. إذا تمكن العلماء من معرفة طريقة لمنع هذا التفاعل بين البروتينات، قد يتمكنون من تطوير معالجة دوائية موجهة.
المضاعفات
تستمر الأعراض والعلامات بعد ظهور المرض حتى الموت، ورغم أن الأعراض والعلامات تتنوع من شخص لآخر، فإن الوظائف الحيوية كالبلع والأكل والكلام والمشي تتدهور عادةً بمرور الوقت، كما يعتبر الاكتئاب عرضاً شائعاً لدى مرضى داء هنتينغتون، إضافة لخطر الانتحار لدى بعض هؤلاء المرضى، لكن ينجم الموت عموماً عن اختلاطات المرض، كالسقوط أو إنتان مثل ذات الرئة.

وبائيات المرض
يعني ظهور المرض في وقت متأخر أنه لا يؤثر عادة على التكاثر. الانتشار العالمي من لداء هنتنغتون هو 5-10 حالات لكل 100.000 شخص، ولكنه يختلف جغرافيًا بشكل كبير نتيجة العرق والهجرة المحلية وأنماط الهجرة السابقة. تتماثل نسبة انتشار بالنسبة للرجل والمرأة، وترتفع نسبة حدوثه بين الشعوب ذوات الأصل الأوروبي الغربي، بمعدل حوالي 7 لكل 100.000 شخص، وهي الأقل في بقية العالم؛ فعلى سبيل المثال، تصل النسبة واحد لكل مليون شخص من أصل آسيوي وإفريقي. وجدت دراسة وبائية عام 2013 عن انتشار داء هنتنغتون في المملكة المتحدة بين عامي 1990 و2010 أن معدل الانتشار في المملكة المتحدة كان 12.3 لكل 100،000. بالإضافة إلى ذلك، فإن بعض المناطق المحلية لديها انتشار أعلى بكثير من متوسطها الإقليمي. واحدة من أعلى حالات الإصابة هي في المناطق المعزولة من منطقة بحيرة ماراكايبو ي فنزويلا، حيث أصاب داء هنتنغتون 700 شخص لكل 100،000 شخص. تم العثور على مناطق أخرى عالية الانتشار بالنسبة للمرض في تاسمانيا ومناطق محددة من اسكتلندا، وويلز والسويد. ترتفع نسبة الانتشار في بعض الحالات نتيجةً لتأثير المؤسس، وهي هجرة تاريخية لشركات الطيران إلى منطقة العزل الجغرافي. وقد تم تتبع بعض هذه الناقلات مئات السنين باستخدام دراسات علم الأنساب. يمكن أن يُعطي النمط الفرداني الوراثي أيضًا دلائل على الاختلافات الجغرافية للانتشار. فعل سبيل المثال، تتمتع أيسلندا على العكس من ذلك بنسبة انتشار منخفضة إلى حد ما من 1 لكل 100000، على الرغم من حقيقة أن الأيسلنديين كشعب ينحدرون من القبائل الجرمانية المبكرة من الدول الاسكندنافية مثل السويديين؛ حيث تعود جميع الحالات باستثناء واحدة إلى ما يقرب من قرنين إلى نسل زوجين عاشا في أوائل القرن التاسع عشر. تتمتع فنلندا أيضًا بنسبة منخفضة تصل إلى 2.2 فقط لكل 100،000 شخص.

وحتى تم اكتشاف الاختبار الجيني، شملت الإحصائيات التشخيص السريري المستند على أساس الأعراض الجسدية وتاريخ العائلة لداء هنتنغتون، باستثناء أولئك الذين ماتوا لأسباب أخرى قبل التشخيص. يمكن الآن إدراج هذه الحالات في الإحصاءات؛ وعندما يصبح الاختبار متاحًا على نطاق أوسع، فمن المرجح أن تزداد تقديرات انتشار المرض وحدوثه.

العلاج
لا يوجد علاج مرضٍ لإيقاف أو عكس داء هنتينغتون، لكن يمكن لبعض المقاربات السيطرة على الأعراض والعلامات، لكن داء هنتينغتون يُسبب في النهاية العجز العقلي والفيزيائي، وقد يصبح المريض بحاجةٍ لعناية تمريضية منزلية مع تطوّر المرض.

الأدوية
تيترابينازين هو الدواء الأول المُصدق من قبل هيئة الدواء والغذاء لعلاج الأعراض والعلامات الخاصة بداء هنتينغتون.
يُساعد هذا الدواء في إنقاص الحركات التشنجية اللاإرادية لداء هنتينغتون بزيادة كمية الدوبامين في الدماغ، وتتضمن التأثيرات الجانبية المُحتملة الأرق والنعاس والغثيان وعدم الارتياح، ولا ينصح باستعمال هذا الدواء لدى المكتئبين وخاصة ذوي الأفكار الانتحارية.

قد تُساعد المهدئات مثل كلونازيبام ومضادات الذهان مثل هالوبيريدول وكلوزابين في السيطرة على الحركات والثورات العنيفة والهلوسات. إن هذه الأدوية مساعدة لكنها تسبب التخدير، وفي بعض الحالات، يمكنها أن تسبب تيبساً إضافياً وصلابة.
قد تُساعد أدوية متنوعة تشمل فلوكسيتين وسيرترالين ونورتريبتيلين في السيطرة على الاكتئاب وحالات الوسواس القهري التي تحدث عند بعض مرضى داء هنتينغتون، كما قد تُساعد أدوية مثل ليثيوم في السيطرة على تقلب المزاج والانفعالات الشديدة.
يمكن أن تتضمن التأثيرات الجانبية للعديد من الأدوية التي تعالج أعراض داء هنتينغتون فرط الاستثارة والتعب وعدم الارتياح.

المعالجة الكلامية
قد يؤثر داء هنتينغتون على الكلام والقدرة على التعبير عن الأفكار المعقدة، وقد تُساعد المعالجة الكلامية، ويجب تذكير أصدقاء المريض وعائلته ومن يعتني به بأن عدم قدرة المريض على الكلام لا تعني بالضرورة أنه لا يفهم ما الذي يحدث حوله، إذ يجب عليهم الاستمرار بالتحدث للمريض وإبقاء المحيط طبيعياً قدر الإمكان.

العلاج الفيزيائي والوظيفي
يُساعد العلاج الفيزيائي في الحفاظ على العضلات أقوى وأكثر مرونة، مما يساعد في الحفاظ على التوازن ويقلل من خطورة السقوط، كما قد يُساعد العلاج الوظيفي المريض بجعل منزله أكثر أمناً وإعطائه الإستراتيجيات المناسبة لمواجهة مشاكل الذاكرة والتركيز، ولاحقاً في سياق تطوّر المرض يمكن أن يُساعد العلاج الوظيفي المريض في تحديات الأكل وارتداء الملابس والنظافة.

العلاجات التجريبية والأبحاث الحديثة
يحاول العلماء العمل على اكتشاف علاجات جديدة لإبطاء مسار داء هنتينغتون.
إن أحد مجالات الأبحاث هو مشاركة أنواع معينة من أدوية السرطان والإيدز، حيث أضعفت هذه المشاركة تطور داء هنتينغتون لدى ذبابة الفاكهة.
إن العلاج بالخلايا الجذعية هو سبيل آخر للبحث، حيث يمكن للخلايا الجذعية المزروعة في الدماغ أن تُساعد في إنقاص بعض الضرر الحاصل للعصبونات في الدماغ.
أظهرت الدراسات الحيوانية نتائج واعدة، لكن يجب إجراء أبحاث بشكل أكثر.
في تشرين 1 عام 2008، بدأت الأبحاث بإجراء التجارب السريرية العشوائية على دواء جديد يدعى ACR16، حيث يقوم هذا الدواء بتثبيت مستويات الدوبامين، التي يمكن أن تحسن الوظائف الحركية والمعرفية والنفسية لدى المرضى.

Huntington disease

Huntington's disease (HD), also known as Huntington's chorea, is an inherited disorder that results in the death of brain cells.[5] The earliest symptoms are often subtle problems with mood or mental abilities.[2] A general lack of coordination and an unsteady gait often follow.[3] As the disease advances, uncoordinated, jerky body movements become more apparent.[2] Physical abilities gradually worsen until coordinated movement becomes difficult and the person is unable to talk.[2][3] Mental abilities generally decline into dementia.[4] The specific symptoms vary somewhat between people.[2] Symptoms usually begin between 30 and 50 years of age, but can start at any age.[5][4] The disease may develop earlier in life in each successive generation.[2] About eight percent of cases start before the age of 20 years and typically present with symptoms more similar to Parkinson's disease.[4] People with HD often underestimate the degree of their problems.[2]

HD is typically inherited, although up to 10% of cases are due to a new mutation.[2] The disease is caused by an autosomal dominant mutation in either of an individual's two copies of a gene called Huntingtin.[5] This means a child of an affected person typically has a 50% chance of inheriting the disease.[5] The Huntingtin gene provides the genetic information for a protein that is also called "huntingtin".[2] Expansion of CAG (cytosine-adenine-guanine) triplet repeats in the gene coding for the Huntingtin protein results in an abnormal protein, which gradually damages cells in the brain, through mechanisms that are not fully understood.[5] Diagnosis is by genetic testing, which can be carried out at any time, regardless of whether or not symptoms are present.[6] This fact raises several ethical debates: the age at which an individual is considered mature enough to choose testing; whether parents have the right to have their children tested; and managing confidentiality and disclosure of test results.[3]

There is no cure for HD.[5] Full-time care is required in the later stages of the disease.[3] Treatments can relieve some symptoms and in some improve quality of life.[4] The best evidence for treatment of the movement problems is with tetrabenazine.[4] HD affects about 4 to 15 in 100,000 people of European descent.[2][4] It is rare among Japanese, while the occurrence rate in Africa is unknown.[4] The disease affects men and women equally.[4] Complications such as pneumonia, heart disease, and physical injury from falls reduce life expectancy.[4] Suicide is the cause of death in about 9% of cases.[4] Death typically occurs fifteen to twenty years from when the disease was first detected.[5]

The first likely description of the disease was in 1841 by Charles Oscar Waters.[8] The condition was described in further detail in 1872 by the physician George Huntington.[8] The genetic basis was discovered in 1993 by an international collaborative effort led by the Hereditary Disease Foundation.[9][10] Research and support organizations began forming in the late 1960s to increase public awareness, to provide support for individuals and their families, and to promote research.[10][11] Current research directions include determining the exact mechanism of the disease, improving animal models to aid with research, testing of medications to treat symptoms or slow the progression of the disease, and studying procedures such as stem cell therapy with the goal of repairing damage caused by the disease.
Genetics
All humans have two copies of the Huntingtin gene (HTT), which codes for the protein Huntingtin (HTT). The gene is also called HD and IT15, which stands for 'interesting transcript 15'. Part of this gene is a repeated section called a trinucleotide repeat, which varies in length between individuals and may change length between generations. If the repeat is present in a healthy gene, a dynamic mutation may increase the repeat count and result in a defective gene. When the length of this repeated section reaches a certain threshold, it produces an altered form of the protein, called mutant Huntingtin protein (mHTT). The differing functions of these proteins are the cause of pathological changes which in turn cause the disease symptoms. The Huntington's disease mutation is genetically dominant and almost fully penetrant: mutation of either of a person's HTT alleles causes the disease. It is not inherited according to sex, but the length of the repeated section of the gene and hence its severity can be influenced by the sex of the affected parent.[13]

Genetic mutation
HD is one of several trinucleotide repeat disorders which are caused by the length of a repeated section of a gene exceeding a normal range.[13] The HTT gene is located on the short arm of chromosome 4[13] at 4p16.3. HTT contains a sequence of three DNA bases—cytosine-adenine-guanine (CAG)—repeated multiple times (i.e. ... CAGCAGCAG ...), known as a trinucleotide repeat.[13] CAG is the 3-letter genetic code (codon) for the amino acid glutamine, so a series of them results in the production of a chain of glutamine known as a polyglutamine tract (or polyQ tract), and the repeated part of the gene, the PolyQ region
Generally, people have fewer than 36 repeated glutamines in the polyQ region which results in production of the cytoplasmic protein Huntingtin.[13] However, a sequence of 36 or more glutamines results in the production of a protein which has different characteristics.[13] This altered form, called mutant huntingtin (mHTT), increases the decay rate of certain types of neurons. Regions of the brain have differing amounts and reliance on these types of neurons, and are affected accordingly.[13] Generally, the number of CAG repeats is related to how much this process is affected, and accounts for about 60% of the variation of the age of the onset of symptoms. The remaining variation is attributed to environment and other genes that modify the mechanism of HD.[13] 36–39 repeats result in a reduced-penetrance form of the disease, with a much later onset and slower progression of symptoms. In some cases the onset may be so late that symptoms are never noticed.[13] With very large repeat counts, HD has full penetrance and can occur under the age of 20, when it is then referred to as juvenile HD, akinetic-rigid, or Westphal variant HD. This accounts for about 7% of HD carriers.[25]

Inheritance
Huntington's disease has autosomal dominant inheritance, meaning that an affected individual typically inherits one copy of the gene with an expanded trinucleotide repeat (the mutant allele) from an affected parent.[13] Since penetrance of the mutation is very high, those who have a mutated copy of the gene will have the disease. In this type of inheritance pattern, each offspring of an affected individual has a 50% risk of inheriting the mutant allele and therefore being affected with the disorder (see figure). This probability is sex-independent.[26]

Trinucleotide CAG repeats over 28 are unstable during replication, and this instability increases with the number of repeats present.[13] This usually leads to new expansions as generations pass (dynamic mutations) instead of reproducing an exact copy of the trinucleotide repeat.[13] This causes the number of repeats to change in successive generations, such that an unaffected parent with an "intermediate" number of repeats (28–35), or "reduced penetrance" (36–40), may pass on a copy of the gene with an increase in the number of repeats that produces fully penetrant HD.[13] Such increases in the number of repeats (and hence earlier age of onset and severity of disease) in successive generations is known as genetic anticipation.[13] Instability is greater in spermatogenesis than oogenesis;[13] maternally inherited alleles are usually of a similar repeat length, whereas paternally inherited ones have a higher chance of increasing in length.[13][27] It is rare for Huntington's disease to be caused by a new mutation, where neither parent has over 36 CAG repeats.[28]

In the rare situations where both parents have an expanded HD gene, the risk increases to 75%, and when either parent has two expanded copies, the risk is 100% (all children will be affected). Individuals with both genes affected are rare. For some time HD was thought to be the only disease for which possession of a second mutated gene did not affect symptoms and progression,[29] but it has since been found that it can affect the phenotype and the rate of progression.
Mechanism
The huntingtin protein interacts with over 100 other proteins, and appears to have multiple biological functions.[31] The behavior of this mutated protein is not completely understood, but it is toxic to certain cell types, particularly in the brain. Early damage is most evident in the striatum, but as the disease progresses, other areas of the brain are also more conspicuously affected. Early symptoms are attributable to functions of the striatum and its cortical connections—namely control over movement, mood and higher cognitive function.[13] DNA methylation also appears to be changed in HD

كيلي ماكدونالد

كيلي ماكدونالد (بالإنجليزية: Kelly Macdonald) ممثلة اسكتلندية من مواليد 23 فبراير 1976، حاصلة على جائزة الإيمي 2006 كأفضل ممثلة عن فيلم فتاة في المقهى، كما حصلت على جائزة نقابة ممثلي الشاشة 2007 كأفضل فريق ممثلين عن دورهم في فيلم لا وطن للمسنين. في 2012 قامت بعمل أداء صوتي لشخصية مريدا من فيلم ديزني بيكسار أسطورة مريدا.

Kelly Macdonald

Kelly Macdonald (born 23 February 1976) is a Scottish actress best known for her roles in Trainspotting (1996), Gosford Park (2001), Intermission (2003), Nanny McPhee (2005), Boardwalk Empire (2010–2014), Harry Potter and the Deathly Hallows – Part 2 (2011), Brave (2012), and the Black Mirror episode "Hated in the Nation" (2016). For the 2005 TV film The Girl in the Cafe, she was nominated for a Golden Globe and won the Emmy Award for Outstanding Supporting Actress in a Miniseries or Movie. In 2003, she appeared in the acclaimed BBC miniseries State of Play as journalist Della Smith.

For her role in the 2007 film No Country for Old Men, she was nominated for the BAFTA Award for Best Actress in a Supporting Role. She went on to star for five seasons in the HBO series Boardwalk Empire (2010–2014) as Margaret Thompson, for which she received a 2011 Emmy Award nomination.
Early life
Macdonald was born in Glasgow, Scotland and moved to Aberdeen when she was five years old. Her mother was a garment industry sales executive. Macdonald attended Eastwood High School from 1987 to 1993.[1]

Career
Macdonald's career began while she was working as a barmaid in Glasgow.[2] She saw a leaflet advertising an open casting session for Trainspotting and decided to audition, winning the part of Diane,[3] the underage seductress to Ewan McGregor's Renton. Other roles include Mary O'Neary in Two Family House,[4] and an actress playing Peter Pan in Finding Neverland.[5] She had major roles, in Robert Altman's British period piece Gosford Park,[6] where she played an aristocrat's maid, and in Intermission (2003), as Deirdre.[7]

On radio, she portrayed Mary in the 1999 BBC radio drama Lifehouse, based on Pete Townshend's abandoned rock opera, some of the songs for which were released on The Who's album Who's Next.[8] On television, her highest profile roles have been in two BBC dramas, the Paul Abbott serial State of Play (2003),[9] and the one-off Richard Curtis piece The Girl in the Café (2005).[10] Both of these were directed by David Yates, and both also starred Bill Nighy. For her performance in The Girl in the Café, she was nominated for the Golden Globe Award for Best Actress – Miniseries or Television Film in 2006,[11] and won the Primetime Emmy Award for Outstanding Supporting Actress in a Miniseries or a Movie.[12]

Macdonald starred in the 2005 film Nanny McPhee,[13] as the scullery maid Evangeline, and has since had supporting roles in A Cock and Bull Story (2006),[14] and the Coen brothers' Academy Award-winning No Country for Old Men (2007),[15] for which she was nominated for a BAFTA Award for Best Actress in a Supporting Role.[16] It was reported that she had to fight her agent to be considered for the role, but in 2017 Macdonald denied the story.[17]

Other films where she had supporting roles include Choke (2008), the film adapted by Clark Gregg from the 2001 Chuck Palahniuk novel, as Paige Marshall; In the Electric Mist (2009) (based on James Lee Burke's In the Electric Mist with Confederate Dead (1993), as Kelly Drummond, alongside Tommy Lee Jones and John Goodman; and Skellig (2009), as Louise.[18] She played the lead in The Merry Gentleman (2008), as Kate Frazier.[19]

In 2010, she played her first comedy role, in the British independent romantic comedy film The Decoy Bride.[20] (released in 2012). In 2011, she played the "Grey Lady" (revealed to be Helena Ravenclaw) in Harry Potter and the Deathly Hallows – Part 2, the final instalment of the Harry Potter film franchise. She replaced Nina Young, who originally played the role. In 2012, she provided the voice of Merida, the heroine of the Disney/Pixar film Brave, and starred as Dolly in Anna Karenina.

From 2010 until its ending in 2014, she starred in the HBO crime drama Boardwalk Empire as Margaret Thompson, the wife of Prohibition-era Atlantic City crime boss Nucky Thompson (Steve Buscemi). She appeared in all five seasons of the series. In 2011, she and the rest of the show's cast were awarded the Screen Actors Guild Award for Outstanding Performance by an Ensemble in a Drama Series. In 2016, she starred in Ricky Gervais' Special Correspondents as Claire Maddox,[21] and Swallows and Amazons as Mrs. Walker.[22] In 2016, she played the lead role in "Hated in the Nation", an episode of the anthology series Black Mirror for which she received critical acclaim.[23] On 3 November 2016 Macdonald was featured in the trailer for Danny Boyle's T2 Trainspotting confirming she would reprise her role as Diane from the original film. In 2017, she co-starred opposite Benedict Cumberbatch in the BBC film The Child in Time.

Personal life
In August 2003, Macdonald married musician Dougie Payne, bassist of soft rock band Travis. They have two children and moved back to their home town of Glasgow in 2014, after living in London and New York City.[24][25] They separated in 2017

Cold Call

Cold calling is the solicitation of business from potential customers who have had no prior contact with the salesperson conducting the call.[1][2] It is an attempt to convince potential customers to purchase either the salesperson's product or service. Generally, it is referred as an over-the-phone process, making it a source of telemarketing,[3] but can also be done in-person by door-to-door salespeople. Though cold calling can be used as a legitimate business tool, scammers can use cold calling as well.
Evolution
Cold calling, as a means to conduct business, has seen changes as technology has increased. Salespeople who use cold calling once followed specific guidelines in order to produce more profit. These guidelines, now believed to be misconceptions by Wendy Weiss,[4] were as follows:

The more calls a salesperson makes, the more sales will be made.
Any number in the phone book is a potential customer.
Practice makes perfect.
Manipulation is key.
Always be closing.[4]
Cold calling has developed from a form of giving sales pitch using a script[4] into a targeted communication tool. Salespeople call from a list of potential customers that fit certain parameters built to help increase the likelihood of a sale. This modern cold calling, sometimes called "warm calling", tries to "dig deeply to understand"[4][5] the potential customer. To avoid being seen as scammers, legitimate businesses use cold calling as an introduction rather than as a means to close the sale.

Warm calling
Warm calling is when the sales people contact prospective customers that may have a relationship with the business, rather than complete strangers. These previous relationships may be mutual friends, trigger events, or signs of interest in the product or service which are often a result of Inbound marketing (e.g. visiting a website, social media interactions, webinars). Warm calling considers the buyer's perspective and acknowledges that not everyone can be a prospective client.

Criticisms
With the development of newer technology and the internet, cold calling has gained some criticism. Jeffrey Gitomer wrote in a 2010 article for The Augusta Chronicle that "the return on investment on cold calling is under zero."[6] Gitomer believes that cold calling will only annoy customers and will not attract business. Gitomer also believes that referral marketing is a better form of selling and marketing.[6] According to Gitomer, there are "2.5 basic understandings of a cold call":[7]

Cold calling is the lowest percentage sale call.
Cold calling has a very high rejection rate.
Multiple rejections can change the salesperson's mentality and make it more difficult to act friendly and complete calls.[7]
Since the rise of Internet, social media and instant text messaging, a significant portion of people tends to prefer texting over calling and ignores incoming phone calls from unfamiliar numbers.[8][9]
Cold calling has also been used by scammers. One such example was when groups of impostors pose as members of the Microsoft support team. The impostors call several homes from a database of Microsoft owners. The Microsoft customers were then told that there was a virus on their computers, and in order to fix it, they had to download a specific program. The program gave access to the computer files for the impostors.[10] In July 2006, a survey conducted by Lactofree determined that cold callers were the most annoying thing in the UK.[11] Cold calling has been a hallmark in the proliferation of boiler room scams selling fraudulent investment and sports betting schemes from Australia's Gold Coast.[12]

Rules and regulations
Many countries have rules and regulations that limit and control how, when and whom companies can cold call. These rules and regulations are often implemented by government bodies that deal with telecommunication laws in their specific country.

United States
The United States telecommunication laws are developed and enacted by the Federal Trade Commission (FTC). The FTC aims to "puts consumers in charge of the number of telemarketing calls they get at home".[13] The United States, along with many individual states, have enacted various "Do Not Call" lists. These lists are based on the national US Do Not Call List which was enacted in 2003.[13] Every month, since January 2005,[14] companies are required by law to check the "Do Not Call List" database. They are required to remove the registered numbers from their leads lists. However the "Do Not Call List" has certain limitations. Even if a person is registered for the "Do Not Call List", certain organizations can still call. These organizations include:

Telephone surveyors, charities, and political organizations
Organizations that one has had a business relationship with over the previous 18 months
Any company one has given written permission[13]
The FTC has also set certain regulations on when one can be called. Cold calling can only be done in between 8 a.m and 9 p.m. The caller is also required by law to tell the customer who they are and what organization they represents. This includes clarifying if the organization is a for-profit organization or charity. The salesperson also must reveal all information about the product they are selling. This means that they are legally required not to lie.[13]

Many other government organizations monitor cold calling within their jurisdiction including the U.S. Securities and Exchange Commission(SEC).[15] The SEC specializes in monitoring cold calling that deals with stocks, specifically stockbrokers. When investing over the phone, the SEC states that written banking information must be given. This means that an investment cannot be made over the phone.[15]

Canada
The National Do Not Call List (DNCL) is administered by the Canadian Radio-television and Telecommunications Commission (CRTC). As with the U.S. version, the rules exclude surveyors, charities, political organizations/candidates, organizations that one has had a business relationship with over the previous 18 months or has otherwise granted permission, as well as newspapers seeking subscribers.[16][17]

United Kingdom
The United Kingdom has its own version of the "Do Not Call List" known as the Telephone Preference Service (TPS). Any citizen of the United Kingdom can register for the list that aims to eliminate its participants from receiving unsolicited calls from organizations including charities and political parties unlike the United States and Canada.[18] TPS was first enacted in 1999 and eventually saw changes in 2003 that ultimately created the Privacy and Electronic Communications (EC Directive) Regulations 2003.[18] While the TPS prevents unsolicited sales and marketing calls, it does not prevent "recorded/automated messages, silent calls, market research, overseas companies, debt collection, scam calls"[19] according to the TPS website.

In 2012, Richard Herman from Middlesex sent an invoice to a company for the time they had kept cold-calling him.[20] He eventually took the company to the small claims court, leading to the company settling out of court.[20] He had been phoned several times by the company despite being listed with the Telephone Preference Service.[20]

Australia
Australia has its own version of the "Do Not Call List" known as the Do Not Call Register.[21] The "Do Not Call Register" is under the jurisdiction of the Australian Communications and Media Authority (ACMA) which acts as the supreme telecommunications authority in Australia. Registering for the "Do Not Call Register" prevents telemarketers and fax marketers from contacting registered members. Registration for the program is free and will last for eight years.[21] Similar to other countries, there are exceptions to the "Do Not Call Register". These exceptions include: political parties, charities and educational institutions. The "Do Not Call Register" takes effect 30 days after registration.[21]

Republic of Ireland
In the Republic of Ireland, the "National Directory Database" is an index of numbers that cannot be called for the purposes of 'cold calls' and/or sales and advertising.[22] An unsolicited marketing call to a number on the National Directory Database is a criminal offence.[22]

Japan
Some financial products are totally not permitted to cold-call, but the practice is generally permitted within a guideline which requires stating the name of the business, full name of the caller, name of the product and intention of solicitation. There is no do-not-call list. The Japanese government's Financial Services Agency maintains a list of known fraudulent entities involved in financial cold-calling scams.[23]

European Union
Within the European Union, the Data Privacy Directive 2002/58/EC requires the governments of its member states to issue laws until June 2007 that prohibit general cold calling. However, the directive allows both an opt-in or an opt-out model, i.e. requiring a national register for phone numbers which either do (opt-in) or do not (opt-out) welcome cold calls

Arron Banks

Arron Fraser Andrew Banks (born 1966) is a British businessman and political donor.[2] He is the co-founder (with Richard Tice) of the Leave.EU campaign.[1][3] Banks was previously one of the largest donors to the UK Independence Party (UKIP) and helped Nigel Farage’s campaign for Britain to leave the EU.

In November 2018 the National Crime Agency opened an investigation into Banks following concerns raised over the source of his funding.[4] In September 2019, the National Crime Agency dropped its investigations into Banks and Leave.EU. The NCA found "no evidence that any criminal offences have been committed."[5]

He has been reported to have had multiple meetings with Russian embassy officials[6] as well as being offered business opportunities in Russia in the run-up to the Brexit referendum.[7][8][9] He has denied any wrongdoing and said: "There was no Russian money and no interference of any type."[10]

His 2016 book The Bad Boys of Brexit: Tales of Mischief, Mayhem & Guerrilla Warfare in the EU Referendum Campaign was ghostwritten by Isabel Oakeshott.[11][12]

Banks claimed he was suspended from UKIP in March 2017, but UKIP said his membership had "lapsed earlier in the year"
Early life
Banks was raised by his mother in Basingstoke, Hampshire; his father worked as a sugar plantation manager in various African countries. From the age of 13 he attended a boarding school in Berkshire called Crookham Court, before being expelled for "an accumulation of offences", including the sale of lead stolen from the roofs of school buildings, and "high-spirited bad behaviour". He then attended St Bartholomew's School in Newbury but was expelled again. He returned to Basingstoke, where he sold paintings, vacuum cleaners, and then houses.[14]

Business career
Banks was offered a junior job at the insurance market of Lloyd's of London. According to The Guardian, by the age of 27, he was running a division of Norwich Union. The article also claims that he spent a year working for Berkshire Hathaway, the investment company of Warren Buffett.[15] However, in another investigation, both Norwich Union and Warren Buffett reject Banks' claim that he worked for them.[16] Then, from an office above a bakery in Thornbury, Gloucestershire, Banks started a motorcycle insurance broker, Motorcycle Direct, and within a few years the company was big enough to sell for "a few million". He used the money to found Commercial Vehicle Direct about which he says "within a very short period we were the largest van insurance company in the country".[17][14] Over seven years, along with Australian business partner John Gannon, Banks expanded this business to become Group Direct Limited, and in 2008 the company floated by means of a reverse takeover as Brightside Group. He was its CEO from June 2011 to June 2012, at which time the company was listed on the Alternative Investment Market (AIM).[18][19][17] After Banks was dismissed from the company in 2012, he sold £6m worth of the shares in 2013, and received significantly more when the investment firm AnaCap Financial Partners bought Brightside the following year.[16]

Banks was the chief executive (CEO) of Southern Rock Insurance Company in 2014, which underwrites insurance policies for the website GoSkippy.com, founded by Banks.[20][21] After Banks' departure, Brightside took legal action against him, alleging he used confidential information in setting up GoSkippy.com six months later. There were also legal actions between Southern Rock and Brightside.[22][23]

He was previously CEO of AIM-listed Manx Financial Group from April 2008 to February 2009.[18] According to Companies House records, Banks has set up 37 different companies using slight variations of his name. The names used by Banks are Aron Fraser Andrew Banks, Arron Andrew Fraser Banks, Arron Fraser Andrew Banks and Arron Banks. The profiles for the first three names all use the same date of birth but register different lists of companies. When asked by The Guardian about this, he declined to answer questions on the topic.[24]

Banks also owns Eldon Insurance, whose CEO Elizabeth Bilney was also in charge at Leave.EU. The company's profits leapt to £16.7m for the first half of 2017 after recording a £284,000 profit in 2015 and a loss of £22,500 in 2016 despite a 40% increase in revenue. Banks said that Eldon's business had been transformed by the same AI technology used in the Brexit campaign.[25] The offshore holding company that controls Eldon Insurance is ICS Risk Solutions, which funds many of Banks' activities and has paid over £77m between 2015 and 2018 to prop up Southern Rock after Gibraltarian regulators found the business to be trading while technically insolvent.[26] As part of an agreement with the regulators Banks resigned his directorships at Eldon in 2013 and Southern Rock in 2014, also accepting a "period of ban or self-exclusion from other insurance directorships".[27]

He also bought Old Down Manor from musician Mike Oldfield, converting it into a wedding venue.[14]

In 2016, the leaked Panama Papers indicated Banks along with Elizabeth Bilney were shareholders of British Virgin Islands company PRI Holdings Limited, which was the sole shareholder of African Strategic Resources Limited.[28] However, a spokesperson for Banks has denied any links to the lawyer named and denies that Banks was involved with the Papers.[29] Banks has stated that he has a controlling interest in a diamond mine in Kimberley, South Africa, and a licence to mine in Lesotho.[22][30] In July 2018, Banks denied that money paid to a government minister in Lesotho was a bribe; it had been paid to guarantee diamond mining rights in Lesotho. In 2013, Banks paid £65,000 into the private bank account of Thesele Maseribane, then the Basotho minister for women's equality. Banks subsequently covered the £350,000 costs of a political campaign for Maseribane, in 2014, following a military coup in Lesotho.[31]

In January 2015, one estimate of his wealth was £100 million.[22] In November 2017, an estimate of £250 million was mentioned.[32]

Other business associations
Following intensified media scrutiny after his initial donation to UKIP, it emerged that Banks was involved in mining in southern Africa and had connections to Belize. Banks also has connections to companies based in Gibraltar and the Isle of Man, and close connections with family members of the Belizean Prime Minister.[33] However, following remarks made by The Thick of It creator Armando Iannucci on BBC One's Question Time programme, Banks denied owning a company in Belize or seeking to avoid UK tax "via any device". Describing the comments as "clearly defamatory", he threatened legal action towards Iannucci if he did not get an apology within a week.[34][needs update]

Asked if his companies paid full corporation tax, Banks said: "I paid over £2.5m of income tax last year ... My insurance business, like a lot of them, is based in Gibraltar but I've got UK businesses as well that deal with customers and pay tax like everyone else."[33] One of the UK businesses of which Banks is director, Rock Services Ltd, had a turnover of £19.7m in 2013 and paid corporation tax of £12,000. The company deducted £19.6m in "administrative expenses", and the main activity appears to be "recharge of goods and services" with Southern Rock Insurance Company.[33] Southern Rock Insurance states on its website that it underwrites policies for the customers of GoSkippy.com, which is run by Banks.

Rock Services and Southern Rock Insurance's ultimate holding company is Rock Holdings Ltd, a company based on the Isle of Man.[33] Banks has also been a "substantial" shareholder in STM Fidecs, of which Leave.EU is a subsidiary; the company claims to be specialising in "international wealth protection", maximising tax efficiencies for entrepreneurs and expatriates and of "structuring international groups, particularly separating and relocating intellectual property and treasury functions to low- or no-tax jurisdictions".[35]

Political career and donations
Banks was previously a Conservative Party donor but in October 2014 decided to donate £100,000 to UKIP. In response to comments from William Hague that Banks had not been a major Conservative donor in past years, and that Hague had never heard of Banks. Banks increased his donation to £1 million to the UK Independence Party (UKIP), directly referencing Hague's interview as his reasoning. Banks said that he had changed party allegiance because he agrees with UKIP's policies and its view that the European Union "is holding the UK back" because it's a "closed shop for bankrupt countries".[36][20][37] Banks has been described as the "leading figure" behind the anti-EU Grassroots Out and Leave.EU,[38] as well as the official Vote Leave campaign.[39][40]

He signalled his intention to stand for UKIP in the constituency of Thornbury and Yate at the 2015 general election,[41] but the candidate chosen by the party was Russ Martin, who came third. He indicated he would stand in Clacton at the 2017 general election against Douglas Carswell,[42] but later decided otherwise.[43]

Banks claimed in March 2017 that he had been suspended from UKIP; he believed the reason was that he had criticised the leadership. UKIP said, however, that his membership had lapsed before this time.[13]

In May 2018, Banks attended a fundraising event for the Democratic Unionist Party, alongside Nigel Farage, and stated that he would support a bid by Farage to seek office as a DUP candidate after the end of his tenure as Member of the European Parliament in 2019.[44]

In August 2018, it was reported that Banks had applied to join the Conservative Party and had suggested that Leave.EU supporters do the same, with the aim of voting in the party's next leadership election. According to the report, Andy Wigmore, Leave.EU's communications director, who had also applied to join the party, announced that they had received an email welcoming them, but the Conservative Party stated that their applications had not been approved.[45] A Conservative Party spokesman said that welcoming emails were automatic but applications were subsequently reviewed, and theirs had been rejected.[46]

Conservative Party donations
A spokesman for Nigel Farage said that Banks had funded the Chipping Sodbury office for the South Gloucestershire Conservatives "to the tune of £250,000". However, a Conservative spokesperson said the support was "nothing like the order of magnitude" of sums claimed, and estimated that the donations were "probably around the £22,000 mark".[20]

A UKIP source told The Guardian that Banks had also loaned £75,417 to Thornbury and Yate Conservative Party through Panacea Finance (his former company) in September 2007, registered on the Electoral Commission and to be paid back by 2022. However, Companies House records show that Banks resigned from the company in September 2005; therefore it was considered questionable as to whether Banks was controlling the company at the time, or whether he was "using the firm as a 'proxy donor'", according to The Guardian.[24]

Relationship with Eurosceptic organisations
In October 2014, Banks donated £1 million to the UK Independence Party[2][47] and has since raised the equivalent of $11 million for the party. Conservative MP William Hague had called Banks "somebody we haven't heard of" following his defection to UKIP.[2] In response, Banks increased his donation from £100,000 to £1 million, saying: "I woke up this morning intending to give £100,000 to UKIP - then I heard Mr Hague's comment about me being a Mr Nobody. So in light of that I have decided to give £1 million."[37] This donation was one of the largest sums of money ever received by UKIP.[33] Banks also funded Leave.EU by the equivalent of $5 million, and has been seen as the financial backer of the Brexit campaign.[48]

Banks threatened Douglas Carswell with deselection in September 2015 when it emerged that Carswell supported Vote Leave, as opposed to the Leave.EU campaign funded by Banks,[49] describing Carswell as "borderline autistic with mental illness wrapped in", according to The Huffington Post.[50]

In April 2016, Private Eye reported that Leave.EU "is registered at Companies House as Better for the Country Ltd. and controlled by major UKIP donor Arron Banks, after Gibraltar company STM Fidecs Nominees Ltd. transferred its interest to him in August."[40] Banks, along with property investor Richard Tice and media guru Andrew Wigmore, donated £4.3m to the group.[40]

Banks credits the success of Leave.EU to their hiring of Goddard Gunster and their subsequent adoption of "an American-style media approach."[51]

Possibility of "rightwing Momentum"
In September 2016, following Banks' statement that UKIP would be "dead in the water" if Diane James did not become leader,[52] he said that he would leave UKIP if Steven Woolfe was prevented from running for leader and two other senior members remained in the party: "If Neil Hamilton and Douglas Carswell [UKIP's only MP] remain in the party, and the NEC decide that Steven Woolfe cannot run for leader, I will be leaving Ukip".[53]

Banks was sceptical of UKIP under the leadership of Paul Nuttall.[54] Banks said that Leave.EU would continue campaigning as a "rightwing Momentum", ensuring that politicians do not renege on their commitment to leave the EU.[51] Banks has also considered starting and funding a pro-Brexit, nonpartisan citizens' movement called Patriotic Alliance, based on the Five Star Movement,[48] which would target "the 200 worst, most corrupt MPs" for deselection.[55]

Brexit
Main article: Brexit
Banks said that Britain's EU membership "is like having a first class ticket on the Titanic". He also said that "Economically, remaining in the EU is unsustainable."[56][57]

Banks said in May 2016 that his strategy during the Brexit campaign was to "bore the electorate into submission", in the hope that a low turnout would favour Brexit.[58][59]

See also: Vote Leave § Relationship with other groups
Banks threatened to sue the official Vote Leave campaign's candidacy as the official spokesperson for the "Leave" vote in the 2016 EU referendum, which may have possibly delayed the vote by two months. However, Banks has since rejected this and stated that he would not pursue a judicial review any further.[60] Banks has also claimed that Vote Leave were lying when they claimed that the UK sends £350 million to the European Union, claiming this does not take into consideration the rebate which the UK receives from the EU, and that the £350 million is not actually sent to the EU headquarters in Brussels.[61]

Following the murder of Jo Cox, Arron Banks commissioned a poll on whether her murder had affected public opinion on voting. Asked whether the wording of the poll was "tasteless", Banks said "I don't think so",[62] adding that: "We were hoping to see what the effect of the event was. That is an interesting point of view, whether it would shift public opinion."[63]

There were questions as to the source of funds he used to support Brexit and in November 2018 this was referred for criminal investigation.[64] Banks has denied any wrongdoing and stated that he welcomes the police investigation to put an end to the allegations.[65]

A Channel 4 investigation also appeared to point to the conclusion that Banks' Leave.EU organisation faked migrant footage and photographs of "migrants" assaulting women. The photos were never used, but the fake video went viral. Banks accused Channel 4 News journalists of creating "fake news" themselves.[66]

Post-referendum events
Following Britain's vote to leave the EU, Banks emailed a note to journalists attacking his critics. Banks described the United Kingdom's parliamentary Electoral Commission as "the legal division of the In campaign" and disagreed with their decision to enlist Vote Leave as the official campaign. Banks' response to the information commissioner, who in 2016 fined the campaign £50,000 for sending more than half a million unsolicited text messages, was a succinct "Whatever".[67][68] Banks views the Brexit vote as "a kind of halfhearted revolution" due to the fact that Theresa May, who supported Britain remaining in the EU, would end up betraying those who voted to leave.[48]

In November 2017, the Electoral Commission announced that it was investigating whether election rules were broken during the EU referendum, in donations worth a total of £8.4 million to Leave.EU campaigners made by Banks and by Better for the Country Ltd, a company of which Banks is a registered director.[69][70]

The National Crime Agency investigated Leave.EU, Banks and Bilney, as well as other individuals and entities, following the referral of material from a second Electoral Commission investigation. The referral was announced amid concerns that Banks was not the true source of £8m donated to Leave.EU.[71] In September 2019 the National Crime Agency said it had found no evidence of criminality after investigating a series of claims against the Brexit campaign group Leave.EU and Arron Banks. The NCA said: "It will therefore take no further action against Mr Banks, Ms Bilney, Better for the Country Ltd or Leave.EU in respect of this specific matter."[72]

Links to Russian officials and Donald Trump campaign
See also: Russian interference in the 2016 United States elections and Russian interference in the 2016 Brexit referendum
From September 2015, Banks, along with Andy Wigmore, had multiple meetings with Russian officials posted at the Russian embassy in London.[8] In November 2015, Alexander Yakovenko, the Russian ambassador, introduced Banks to a Russian businessman, which was followed by other business proposals on the part of Russians.[7][73] Banks was offered a chance to invest in Russian-owned gold or diamond mines; the deal involved funding from a Russian state-owned bank, and was announced 12 days after the Brexit referendum.[8][7] It is not clear if Banks invested.

For two years, Banks said his only contacts with the Russian government consisted of one "boozy lunch" with the ambassador. After The Observer reported that he had had multiple meetings at which he had been offered lucrative business deals, Banks told a parliamentary inquiry into fake news he had had "two or three" meetings. In July 2018 when pressed by The New York Times, he said there had been a fourth meeting. The Observer has seen evidence that suggests his Leave.EU campaign team met with Russian embassy officials as many as 11 times in the run-up to the EU referendum and in the two months beyond.[6]

It has been reported that on 12 November 2016, Arron Banks had a meeting with president-elect Donald Trump in Trump Tower and that upon return to London, Banks had lunch with the Russian ambassador where they discussed the Trump visit.[8]

Greta Thunberg
In August 2019, Banks was criticised by Labour MPs Tonia Antoniazzi and David Lammy after a tweet about the climate change activist Greta Thunberg as she set off on a two week voyage in a zero carbon yacht. Banks tweeted: "Freak yachting accidents do happen in August". Banks later stated: "Obviously I don't hope she encounters a freak yachting accident. I just enjoy watching the ludicrous tweeter mob following the next outrage."[74][75]

Websites
LibLabCon.com
Banks gave significant funds to LibLabCon.com, a satirical website dedicated to attacking the three major parties. The website included jokes about the treatment of religious people by the Conservatives, claimed Chuka Umunna is "Labour's chief spokesman for tokenism" and described Amnesty International as an organisation which supports "loudmouth idiots chained to a radiator".[76]

Westmonster
On 19 January 2017 (one day before Donald Trump's inauguration as the 45th President of the United States) Banks launched Westmonster alongside Michael Heaver, former press adviser to Nigel Farage.[77][78] It is modelled on the right-wing US websites Breitbart News and the Drudge Report and claims to be "pro-Brexit, pro-Farage, pro-Trump, anti-establishment, anti-open borders, anti-corporatism".[79][80] In the morning of 19 January, Westmonster's Twitter account had gained more than 2,500 followers.[81]

Owners
The official registered owners of the site are Heaver, who owns 50% of the website and is a daily editor,[77] and Better for the Country Ltd. Better for the Country Ltd is the company that ran Leave.EU (one of two main pro-Brexit campaigns, affiliated to Farage) and is directed by Leave.EU's chief executive, Elizabeth Bilney. Better for the Country Ltd is also registered to the same address as Westmonster.

Early contributors and style
Westmonster's welcome message stated that the political establishment had "taken one hell of a beating" in 2016, adding that "2017 might just be even bigger".[81] The timing of this launch was seen as sign that the anti-establishment media which helped Trump to gain power was arriving in the UK, according to BBC News' media editor Amol Rajan.[77]

Although primarily a news aggregator website akin to the Drudge Report, Westmonster does plan to publish original content[77][78] and enlist the support of celebrities and backbench MPs.[81] Early contributions to the website include a piece written by Nigel Farage stating that the "political establishment" of the United Kingdom had not woken up to European populist movements, as well as articles showing majority British support for a burka ban and criticising "remoaners" for "trying to subvert the will of the people"

Football Manager 2020

Football Manager 2020 (officially abbreviated as FM20) is a 2019 football-management simulation video game developed by Sports Interactive and published by Sega as a successor to Football Manager 2019. It was released worldwide on 18 November 2019.[1] In June 2019, it was announced that FM20 will be a launch title for the Google Stadia streaming platform.
Playable leagues
As with FM 2019, the game offers 51 playable leagues[3] in five of the world's continents: Africa, Asia (including Australia), Europe, North America and South America.[4] Coverage is heavily slanted towards European teams, with 34 of its 51 countries having playable leagues, while South Africa is the only country of Africa's total 54 that is covered.

There are 116 leagues in this version.[3] The Romanian Liga III has been dropped from the leagues previously available in FM 2019, while the new Welsh second-tier divisions, Cymru North and Cymru South, have been added.