Genetic mutations associated with RE/ARE spectrum.
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More than half of the publishers listed alongside IntechOpen (18 out of 30) are Social Science and Humanities publishers. IntechOpen is an exception to this as a leader in not only Open Access content but Open Access content across all scientific disciplines, including Physical Sciences, Engineering and Technology, Health Sciences, Life Science, and Social Sciences and Humanities.
\\n\\nOur breakdown of titles published demonstrates this with 47% PET, 31% HS, 18% LS, and 4% SSH books published.
\\n\\n“Even though ItechOpen has shown the potential of sci-tech books using an OA approach,” other publishers “have shown little interest in OA books.”
\\n\\nAdditionally, each book published by IntechOpen contains original content and research findings.
\\n\\nWe are honored to be among such prestigious publishers and we hope to continue to spearhead that growth in our quest to promote Open Access as a true pioneer in OA book publishing.
\\n\\n\\n\\n
\\n"}]',published:!0,mainMedia:null},components:[{type:"htmlEditorComponent",content:'
Simba Information has released its Open Access Book Publishing 2020 - 2024 report and has again identified IntechOpen as the world’s largest Open Access book publisher by title count.
\n\nSimba Information is a leading provider for market intelligence and forecasts in the media and publishing industry. The report, published every year, provides an overview and financial outlook for the global professional e-book publishing market.
\n\nIntechOpen, De Gruyter, and Frontiers are the largest OA book publishers by title count, with IntechOpen coming in at first place with 5,101 OA books published, a good 1,782 titles ahead of the nearest competitor.
\n\nSince the first Open Access Book Publishing report published in 2016, IntechOpen has held the top stop each year.
\n\n\n\nMore than half of the publishers listed alongside IntechOpen (18 out of 30) are Social Science and Humanities publishers. IntechOpen is an exception to this as a leader in not only Open Access content but Open Access content across all scientific disciplines, including Physical Sciences, Engineering and Technology, Health Sciences, Life Science, and Social Sciences and Humanities.
\n\nOur breakdown of titles published demonstrates this with 47% PET, 31% HS, 18% LS, and 4% SSH books published.
\n\n“Even though ItechOpen has shown the potential of sci-tech books using an OA approach,” other publishers “have shown little interest in OA books.”
\n\nAdditionally, each book published by IntechOpen contains original content and research findings.
\n\nWe are honored to be among such prestigious publishers and we hope to continue to spearhead that growth in our quest to promote Open Access as a true pioneer in OA book publishing.
\n\n\n\n
\n'}],latestNews:[{slug:"stanford-university-identifies-top-2-scientists-over-1-000-are-intechopen-authors-and-editors-20210122",title:"Stanford University Identifies Top 2% Scientists, Over 1,000 are IntechOpen Authors and Editors"},{slug:"intechopen-authors-included-in-the-highly-cited-researchers-list-for-2020-20210121",title:"IntechOpen Authors Included in the Highly Cited Researchers List for 2020"},{slug:"intechopen-maintains-position-as-the-world-s-largest-oa-book-publisher-20201218",title:"IntechOpen Maintains Position as the World’s Largest OA Book Publisher"},{slug:"all-intechopen-books-available-on-perlego-20201215",title:"All IntechOpen Books Available on Perlego"},{slug:"oiv-awards-recognizes-intechopen-s-editors-20201127",title:"OIV Awards Recognizes IntechOpen's Editors"},{slug:"intechopen-joins-crossref-s-initiative-for-open-abstracts-i4oa-to-boost-the-discovery-of-research-20201005",title:"IntechOpen joins Crossref's Initiative for Open Abstracts (I4OA) to Boost the Discovery of Research"},{slug:"intechopen-hits-milestone-5-000-open-access-books-published-20200908",title:"IntechOpen hits milestone: 5,000 Open Access books published!"},{slug:"intechopen-books-hosted-on-the-mathworks-book-program-20200819",title:"IntechOpen Books Hosted on the MathWorks Book Program"}]},book:{item:{type:"book",id:"3302",leadTitle:null,fullTitle:"New Research Directions in DNA Repair",title:"New Research Directions in DNA Repair",subtitle:null,reviewType:"peer-reviewed",abstract:"This book is intended for students and scientists working in the field of DNA repair. Select topics are presented here to illustrate novel concepts in DNA repair, the cross-talks between DNA repair and other fundamental cellular processes, and clinical translational efforts based on paradigms established in DNA repair. The book should serve as a supplementary text in courses and seminars as well as a general reference for biologists with an interest in DNA repair.",isbn:null,printIsbn:"978-953-51-1114-6",pdfIsbn:"978-953-51-5375-7",doi:"10.5772/46014",price:159,priceEur:175,priceUsd:205,slug:"new-research-directions-in-dna-repair",numberOfPages:674,isOpenForSubmission:!1,isInWos:1,hash:"1370d613ce38c993753a3dd25f102a3c",bookSignature:"Clark Chen",publishedDate:"May 22nd 2013",coverURL:"https://cdn.intechopen.com/books/images_new/3302.jpg",numberOfDownloads:47217,numberOfWosCitations:63,numberOfCrossrefCitations:44,numberOfDimensionsCitations:98,hasAltmetrics:1,numberOfTotalCitations:205,isAvailableForWebshopOrdering:!0,dateEndFirstStepPublish:"April 17th 2012",dateEndSecondStepPublish:"May 8th 2012",dateEndThirdStepPublish:"August 12th 2012",dateEndFourthStepPublish:"November 10th 2012",dateEndFifthStepPublish:"December 10th 2012",currentStepOfPublishingProcess:5,indexedIn:"1,2,3,4,5,6",editedByType:"Edited by",kuFlag:!1,editors:[{id:"62462",title:"Prof.",name:"Clark",middleName:null,surname:"Chen",slug:"clark-chen",fullName:"Clark Chen",profilePictureURL:"https://mts.intechopen.com/storage/users/62462/images/1731_n.jpg",biography:"Dr. Clark C. Chen received his B.S. from Stanford University in 1992, M.S. from Columbia University in 1993, and his M.D.-Ph.D. from Harvard Medical School in 2001. He completed his neurosurgery training at the Massachusetts General Hospital and subsequently completed independent fellowships in stereotactic neurosurgery and radiosurgery. Dr. Chen previously served as the director of Clinical Neuro-Oncology at the Beth Israel Deaconess Medical Center before his current role as the Director of Stereotactic and Radiosurgery and Co-Director of Surgical Neuro-Oncology at the University of California, San Diego. Dr. Chen’s research is directed at identifying alterations in DNA repair pathways as they relate to brain cancer therapy. Dr. Chen is the recipient of the Damon Runyon Fellowship Award, the James Kerr Award, American Brain Tumor Association Investigator Award, Paul Calabresi Scholar Award, Burroughs Wellcome Career Award, William Guy Forbeck Scholar Award, the Doris Duke Clinical Scientist Award and the Kimmel Scholar Award.",institutionString:null,position:null,outsideEditionCount:0,totalCites:0,totalAuthoredChapters:"4",totalChapterViews:"0",totalEditedBooks:"4",institution:{name:"University of California, San Diego",institutionURL:null,country:{name:"United States of America"}}}],equalEditorOne:null,equalEditorTwo:null,equalEditorThree:null,coeditorOne:null,coeditorTwo:null,coeditorThree:null,coeditorFour:null,coeditorFive:null,topics:[{id:"421",title:"Molecular Genetics",slug:"biochemistry-genetics-and-molecular-biology-microbiology-molecular-genetics"}],chapters:[{id:"44542",title:"Recombination Hot-Spots and Defense Players – Maintenance of Genomic Integrity",doi:"10.5772/54016",slug:"recombination-hot-spots-and-defense-players-maintenance-of-genomic-integrity",totalDownloads:1586,totalCrossrefCites:0,totalDimensionsCites:0,signatures:"Radhika Pankaj Kamdar and Basuthkar J. 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It is anticipated that this book will make a scientific contribution to energy and environmental regulations, quality and efficiency of energy services, energy supply security, energy market-based approaches, government interventions, and the spread of technological innovation for a broad spectrum of researchers, academics, graduate and doctoral students, and other scientists both today and in the future.",isbn:"978-1-78923-874-7",printIsbn:"978-1-78923-873-0",pdfIsbn:"978-1-83962-954-9",doi:"10.5772/intechopen.77437",price:119,priceEur:129,priceUsd:155,slug:"energy-policy",numberOfPages:156,isOpenForSubmission:!1,hash:"7b3214f2f9bbd4ca03ca927267b13cbf",bookSignature:"Tolga Taner",publishedDate:"July 22nd 2020",coverURL:"https://cdn.intechopen.com/books/images_new/7633.jpg",keywords:null,numberOfDownloads:3405,numberOfWosCitations:0,numberOfCrossrefCitations:5,numberOfDimensionsCitations:12,numberOfTotalCitations:17,isAvailableForWebshopOrdering:!0,dateEndFirstStepPublish:"September 4th 2018",dateEndSecondStepPublish:"September 25th 2018",dateEndThirdStepPublish:"November 24th 2018",dateEndFourthStepPublish:"February 12th 2019",dateEndFifthStepPublish:"April 13th 2019",remainingDaysToSecondStep:"2 years",secondStepPassed:!0,currentStepOfPublishingProcess:5,editedByType:"Edited by",kuFlag:!1,biosketch:null,coeditorOneBiosketch:null,coeditorTwoBiosketch:null,coeditorThreeBiosketch:null,coeditorFourBiosketch:null,coeditorFiveBiosketch:null,editors:[{id:"197240",title:"Associate Prof.",name:"Tolga",middleName:null,surname:"Taner",slug:"tolga-taner",fullName:"Tolga Taner",profilePictureURL:"https://mts.intechopen.com/storage/users/197240/images/system/197240.jpg",biography:"Dr. Tolga Taner is the head of the Department of Motor Vehicles and Transportation Technology at Aksaray University, Turkey. 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A brief socioeconomic literature review, approaching economic, political and social analyses, reveals that Brazilian society has distinctive characteristics, in which the emergence of new economic agents and institutions are responsible for disseminating financial activities and entrepreneur practices in the country. The Brazilian financial market has developed several institutions to create a stable environment encouraging savings and investment from the majority of the population who are unable, unfamiliar with or were marginalized by the financial system.
It is possible to observe a serious of actions that were important for the development of the Brazilian economic scenario; and, it is also significant highlighted that this events were particularly emphasized in Brazilian media as being essential for economic growth. These included some procedures about providing new laws for corporations; offering protection for minority shareholders[1] -; strengthening of Securities and Exchange Commission of Brazil, whose activities were restructured to support financial market development [1] -; establishing benefits for companies that adopt the corporate governance practices launched by the Brazilian Development Bank[1] -; and the creation of a capital market plan[1] - to guarantee the sustainability of the stock market. These recent events have brought security and ensured the creditability of the Brazilian financial market[1] -.
Therefore, in April 2008, Brazil was classified as investment grade. It suggests that the country was regarded as having low risk of default and the nation is safe to receive investment[1] -. Consequently, the news affected the stock market and its index registered a new record score[1] -. It is also important to mention that Brazil also received substantial investment by foreign investors, as a consequence of the world attention focused on the BRIC’s (Brazil, Russia, India and China)[1] -.
At least, Brazil symbolically won a great position in the international scenario and it is seen as a virtuous economic, political and legal environment. In this scenario, new agents emerged; they are considered financial intermediaries that entice ordinary people to became entrepreneur and encourage them to the finance field through different activities such as production of books, lectures and coaches. In this sense, it is attractive to present theses intermediates and their activities, because this makes it possible to understand how some actors are able to bring individuals into new economic practices.
The Brazilian economy context allowed the emergence of new actors. They are moral entrepreneurs of finance, educating on ways to invest honestly to achieve economic success, even during times of crisis. By capitalizing on volatility and uncertainty, they educate how instability can be the best time for the development of economic skills.
The expansion of this culture of investment is popularized and connected with models of self-help. This phenomenon has been consolidated into a new model of society that supports the growth of the entrepreneur and financial practices in Brazil and it legitimatizes personal economic activities.
This paper presents some recent literature on aspiring female entrepreneurs in order to argue about the social (re)signification that relates the role of women in family and society, approaching the transformation of evaluative concepts that surround the socio-economic life of women through books and websites that encourages entrepreneurship and economic decision practices to reach financial independence.
The debate about the role of the State and the economy in Brazil led to a series of studies, mainly from the 1980s, and it continues at present, especially about the state’s role such as regulation and deregulation issues (Evans, 1995). Overall, the 1980s were characterized by a neo-conservative wave and its proposal of a minimal state. In 1990s, when the neoliberal proposal became less acceptable, the movement toward reform, or more specifically, the state reconstruction began to be a dominant program (Bresser-Pereira, 1997). According to Bresser-Pereira and Grau (1999), the globalization process from the 1990s also required new methods of more efficient public administration; consequently, it increased the importance of new actors to implement social services that was guaranteed by the State.
At this same time, the State was seen as inefficient and corrupt, in which political and economic reforms were being discussed to change this scenario. However, this situation created a new dynamic, which provided the rise of new agents and organizations, for example, the growth of non-governmental organization (NGOs) and self-help groups that were concerned about bringing social and economic development to Brazilian nation (Bresser-Pereira; Grau, 1999).
These developments help us understand not only the reform of the State but also the development of the Brazilian financial market and the emergence of corporate governance issues in Brazil (Grün, 2003). Corporate governance, which is shaped by the financial sphere, appeared as a requirement for transparency via society, emerging as a criticism of the Brazilian bureaucratic model. The corporate governance movement was supported during the Fernando Henrique Cardoso government (1995-2002) and was tied to the privatization issues, because talking about transparency was essential to warm up the capital market and to attract investors. The government needed to be transparent to increase investors’ confidence in the privatization process, which means that the government was required to adopt corporate governance to be perceived as transparent institution (Grün, 2003).
Carvalho (2002) argues that it is possible to observe modifications of institutional investor’s attitudes during the Fernando Henrique Cardoso government. As a result, important facts emerge, such as, the internationalization of the Brazilian capital market, the increasing number of companies accessing international market through American Depositary Receipt (ADR)[1] -; the opening of the financial system for multinational institutions, and the increasing importance of foreign investors in the domestic stock market.
In 2002, the principal candidate for the Presidency, Luiz Inácio Lula da Silva, who represents the left and unionism, went to Bovespa (the principal stock exchange in Brazil). At that time, he wanted to demonstrate adherence to economic and corporate governance practices established in the financial market. As a symbolic approach, it is possible to argue that corporate governance has gained huge attention in the nation (Grün, 2003).
It is important to highlight, according to Sá (2003), that the corporate governance discourse is associated with economic progress, and that the financial actors claim that it is through the economic growth that Brazilian society will achieve social development. In general, social and economic development became to be seen as part of the same political approach.
Therefore, corporate governance becomes a great solution for Brazilian society. It is recognized not only as a neoliberal prescription to solve the problem of companies’ capitalization, but also as a means to enlarge both financial markets, and the Brazilian economy and social field (Grün, 2004).
In this sense, Brazil, like other emerging nations, is experiencing rapid financialization. This process is shaped by financial changes in the global financial market and involves restructuring of the country’s financial organization through privatization, fiscal and monetary reform and the creation of new programs and institutions that encourage economic growth and popular investment (Grün, 2009). Financialization includes not only financial intermediaries but also self-help deals (Leite, 2012).
The financialization processes is formed by an interesting dynamic in Brazil, in which corporate governance practices gain significance in the context of the critique of the inefficient state. Here, it is possible to observe the rise of new economic actors that appeared as a solution to change the “uncertainty” of the economic and political scenarios.
Thus, the pathway of corporate governance, which goes through the economic and political issues to the capital market in Brazil, opens a new way to understand how actors try to change the traditional perception of the market through their symbolic strategies (Grün, 2007). Moreover, regarding the popularization of financial market issues, the Brazilian state is watching its expansion, but to some extent, without control over the situation.
At the same time, that the financial market is being reshaped, it is possible to notice the rise of personal finance and entrepreneurial discourses that it is linked to self-help schemes in Brazil. An interesting evidenece of this scenario comes from the bookstores’ shelves that are filled with books and releases that deal with money and financial advices.
McGee (2005) explains that in a period of declining security, there is no surprise if one finds a marked increase in the number of self-help titles. In this way, McGee emphasizes how the promise of self-help can lead individuals into a new sort of enslavement, into a cycle where the self is not improved but endlessly belabored.
Simultaneously, it allocates to the individual sphere the need to rely on private financing, rather than relying on the government. It formed the basis for the development of a new way of thinking in Brazil, the stimulus to economic growth by individuals through the ideals of self-help, that means – “if success is solely the result of one’s own efforts, then the responsibility for any failure must necessarily be individual shortcomings or weaknesses” (McGee, 2005:13). This movement seeks to reach the general population, it is not restricted to a portion of society able to innovate, save and invest in the markets.
In Brazil, Paula and Wood Jr. (2003) highlight that the first signs of demand for personal finance issues were noted after the release of the books: Your Financial Future[1] - and Rich Dad, Poor Dad.
Among the list of publications related to personal finance, there are several books directed to women, for example, “Rich Woman – the investment guide for Women”. The formula she uses is her “husband’s idea” – he is the author of Rich Dad, Poor Dad. She employs her own history of life to teach how to be financially independent. Other illustration is the book: Normal Girls Go Shopping; Expert Girls Go to the Stock Market written by Brazilian authors –; they advertise – “You do not know, but you were made to invest”. The authors focus on how women are educated to hate money [1] -. In general, the advices for women emphasize that women do not enrich because they focus on needs of others rather than on themselves. Consequently, these facts prevent women from becoming independent.
These books follow a model that structures a kind of “cultural guide”, in which enhances questions about conspicuous consumption, biological and social women’s periods like – career, marriage, children, divorce, boyfriend, pregnancy, etc. By the way, it is important to observe that they emphasize the quest for financial independence, but highlight issues that lead to the idea of housekeeping and family.
The book titled “Smart woman: (…) makes investments” [1] - is a typical example. The narrative focus on issues related to career and indicated some steps to achieve financial independence, but it also highlighted that women could not forget how to be a good mother and a respectable housewife. The author emphasizes the needed for women to think of theirselves, but also points to their traditional role in family.
In general, these publications also deal with traditional housekeeping like shopping, caring children and family. These subjects appeared associated with issues like financial planning, credit cards, and investments as guidelines to motivate them to set goals and to manage time, money and other resources as central entrepreneurship skills to achieve financial independence.
In this sense, several reports about the increasing number of women participation in markets and their relationship with money were found in a range of economic Brazilian newspaper[1] -. Some reports mention it as a new phenomenon that were being occurred, mostly in the upper middle classes – it shows that some women left their professional life to manage the family’s assets. As presented in the quote below:
(…) she is graduated in marketing management. Main Occupation “home broker.” That’s how she defines herself. Three years ago, she started managing the family financial assets. She was encouraged by her husband, a mechanical engineer who works in the area of power generation. She attended a seminar of the “Women in Action,” realized by Bovespa. Since then, she has managed the couple’s investments. She is happy, because she is watching the multiplication of their moneyData taken from: “Público feminino é arma da bolsa parareterpessoafísica”. Valor Econômico, 26/03/2009.Free translation made by the author..
In this scenario, the São Paulo Stock Exchange leads a program directed to female entrepreneurs – it is called “Women in action” (2003). The project was designed to give women the step-by-step guidance to learn about business plan. It also elucidates demands about budget skills, and presents advices about financial options and requirements.
This fact is clear more evident, when it focus on Sebrae (Brazilian government agency that provides support to micro and small enterprises) that encourages women of all social classes to became entrepreneur. For example, the “Yearbook of Women Entrepreneurs and Workers in Micro and Small Businesses” endorses that investigators analyzed gender profiles in small businesses from 2001 to 2011 and they reveal that female entrepreneurs in the country grew by 21.4% in this period (Stewart, 2013)[1] -. In the yearbook, a technical Director emphasizes that:
“As business women, they can better reconcile their work with their obligations to take care of their homes and their children. Nearly half of these women are solely responsible for the education of their children, and that doesn’t stop them from starting a business with dedication.”Ibid.\n\t\t\t\t
At present, it is notable the spread of entrepreneurship education as a subject in the curriculum of all educational levels, from preschool to high school. At the same time, it is important to mention the expansion of MBA´s that focus on business management professionals in the country. However, Sebrae, since the early 1990s stands out as the main institution related to the promotion of entrepreneurship culture in Brazil.
It is worth to emphasize that, in the beginning of 2014´s, was inaugurated the Sebrae Business School (São Paulo) the first public educational institution dedicated to offer courses about entrepreneurship at technical and graduation levels[1] -.
Thus, entrepreneurship themes in schools and universities is a recent topic. There are no records of specific courses directed to women in these institutions. Besides the acting of Sebrae, regarding to entrepreneurship education of women, there are programs sponsored by the federal government and nonprofit institutions. In this case, it is noteworthy to highlight some projects in Brazil.
On the one hand, the “Mulheres Mil” is a program inserted into the set of public policy priorities of the federal government, in which aims to stimulate equity, access to education, and gender equality, especially, in the Northeast and North regions. Under three axes – education, citizenship and sustainable development – the program seeks to provide social inclusion focused on autonomy and creation of alternatives workplace to improve the quality of their lives and their communities[1] -.
Another program is the “10,000 Women” promoted by Fundação Getulio Vargas and IE Business School, launched in 2008, is a philanthropic initiative that seeks to bring business education for women and to improve the quality of their learning. [1] -.
On the other hand, there are also initiatives from nonprofits associations, like the Association of Women Entrepreneurs (Brasilia) that seek to ensure the empowerment of women, as well as, to promote opportunities for the social and cultural equality between men and women. It is important to note that the courses offered range from computer, hardware, and telemarketing to courses like home services and caregiver for the elderly sector[1] -.
In general, the stimulus to be an entrepreneur, proclaimed by the “gurus” and government institutions, emphasizes the importance of planning a career path that involves being a businessperson and financially independent. That means, women could be an autonomous worker, and they also could work in their own home; therefore, it is possible being a respectable mother and a virtuous housewife – “Generally, women are better able to achieve a balance between their personal and professional lives than men. In fact, many women go the entrepreneurial path in order to have more flexibility with their time” – elucidates a technical Director of Dieese in the reporter (Stewart, 2013).
However, these discourses obscure the underworld of informal and precarious labor, affecting in particular the lower classes.This phenomenon reinforces the traditional role of women and it supports the fact that they are working longer and harder than ever before. Far from the idea that women work for a salary or for their independence, most women work because they needed it.
These financial consultants have been infiltrated through different sectors of society, forming a particular dynamic. This movement has resonance in Brazilian society, because it resets the social roles in their place, that is, the women are ‘backing home’ to take care of families’ money (Leite, 2012).
The arguments employed by such consultants massively reinforce the needed for entrepreneurial motivation to achieve financial independence. However, such arguments appear strictly related to issues such as marriage, home, family, children, quality of life, etc. The advisors also report some financial family problems, and they consider a lack of financial understanding and poor money management skills that were not coordinated by the women.
These events indicate an interesting sociological phenomenon, in which the woman comes out of the labor market and back into the domestic sphere, that means, the women are coming back to their traditionally safe environment – the oikos.
Whereas, the “housekeepingt” is the role that historically was part of the women’s activities, but, in this case, the female figure is seen not as a house manager, but as a kind of a home “investor”. Apparently, this new phenomenon places the woman in her place of origin: the home.
We are watching a phenomenon that involves issues relating to the family; where the “cultural guide” preaches that women have to take care of their home and children, not only financially but also in a traditional way. The relationship between financial world and the role of wife/mother can also be easily found in books that encourages entrepreneurship attitudes in Brazil.
By comparing books considered bestsellers in the United States with which are translated in Brazil, it is possible to note that the advices directed to women given by Brazilian intermediaries are not so aggressive related to some suggestions given by one of the most popular financial counselors in the United States.
The American financial guru uses aggressive and hostile language to give financial advices and to talk about money to the viewers´ of her show. Overall, her aggressive style and the visibility she gives to the fact that she is lesbian apparently would not work in Brazilian society. After more than 10 years of success in the United States, there are no translations of her books to Portuguese. However, there is an adaptation of her financial guide “Women and Money,” in 2009, realized by the principal financial advisor in Brazil.
In the preface of “Women and Money,” the Brazilian author addresses that he agrees with the simple way she presents complex subjects such as finance for the public. However, he emphasizes that some opinions and suggestions made by Suze are clearly different from his arguments.
On the other hand, “Rich Dad, Pood Dad´s”, who also is one of the best-known American financial book – the author has a different attitude. His principles follows the form of a story, which tells the Rich Dad’s path to get rich.
Thus, it is necessary to point out that the “adaptation”of ideas is a great evidence that outlines the “cultural guide,” which conduct and attract individuals to the financial universe in which issues such as family, money, and wealth express a peculiarity of Brazilian society.
From the exposure of these facts, it is possible to argue that the construction of this new kind of economic figure is tied to the ideas that lead to family issues in Brazil, which becomes an apparatus for stability and security. This “new figure” annihilates the negative aspects of the entrepreneur, if he is greedy and individualistic, the new example should worry in accumulating wealth to enjoy and ensure your family’s future. In other words, the expansion of the financial and entrepreneur practices is supported by Christian ideals about the strengthening of the family ties, which focus on the need for savings.
The appeals for the individuals to take control of their own financial independence symbolically legitimize foreign economic practices that was seen before as a negative skills. Thus, the financial advisors help to demystify the capital market, which gradually become a not “hazard” form of investment in Brazilian society.
There is a mechanism that operates both in the objective and in subjective spheres of society, in which sets up the legitimacy of the financial world in the country. The “new” economic culture that have been created is surrounded by family ethics and operates as an apparatus capable of changing social behaviors and sensitivities, ie, the habitus (Bourdieu, 1997), which, in turn, reflects social rearrangements and redirects the roles of individuals and family in society.
The advices instill in the public receptor a new perception about the financial world, leading individuals to believe that acting “economically” is a moral action. There is a moral influence being created to perform individual’s actions that transforms attitudes and practices. Personal finance promoters have created a new social space, supported by performances, miraculous calculations, magic and science. Therefore, the idea about happiness and satisfaction is associated with the possession of money (Zelizer, 1994; Simmel, 1990).
The tremendous importance of money for understanding the basic motives of life lies in the fact that money embodies and sublimates the practical relation of man to the objects of his will, his power and his impotence; one might say, paradoxically, that man is an indirect being. I am here concerned with the relation of money to the totality of human life only in so far as it illuminates our immediate problem, which is to comprehend the nature of money through the internal and external relationships that find their expression, their means or their effects in money (Simmel, 1990: 211).
Historically, Donzelot (1986) demonstrates the importance of savings for the advent of working class families as a practice of discipline individual unruly behavior. The rebirth of the questions that refer to a temperance movement reinforces the meaning of the familialism concept (Lenoir, 2003), which suggests thinking “family” as a category that is integrated to financial sphere as a natural institution.
For this author, the meaning of “family” is dynamic, because, historically, it has been changing with the emergence of economic models and new forms of political and social organization. According to Lenoir, the family cannot be regarded as a principle of evolution, but as a product of economic, political and social context, in which it is possible to argue that the advance of financial and entrepreneurial practices also operates as a tool that “normalize” the routine of a family life – leading women to their traditional place.
It is noteworthy that other agents and institutions also contribute to strengthening the current ethics of the family as disciplinary rules of behavior (Foucault, 1994). For example, in Brazil, there is the “State”, which formulates policies and programs of economic and social inclusion that values the family unit, and the Catholic Church, which carries out a campaign about the indissolubility of marriage, promoting ideas against the abortion, the homosexuality and the contraception, etc.
Similarly, it is possible to observe the expansion of neopentecostal churches, which strengthen the family entity as a synonym for professional and economic success; they try to establish a new meaning for “money”, introducing economic topics such as entrepreneurship, business, personal finance, among others. At the Universal Church of the Kingdom of God, according to Mariano, the individual with financial problems are induced to participate in the ritual of prosperity, and, if he pay the tithe, he will receive pastoral councils, prayers, exorcisms, and promises of incentives for self-employed (Mariano, 1999: 60).
In the same way, the individuals, are called by the personal financial promoters, to reflect about the budget and to start carrying out a new financial plan, and also they are incentivized to initiate an innovative business. These prosperity churches are symbolic associated with financial and entrepreneur intermediaries, because culturally they are promoting new attitudes regarding the financial and entrepreneurial life. In fact, there is a formation of a “cultural guide” that admit the versions of different agents and institutions that figure the social space; however, we are watching a cultural progress of the finance and entrepreneur ideas proposed by unthinkable characters surrounded by an optimism culture and familialism ethics.
This brief scenario shows a specific reality in Brazil, the apparent link between this sector and family issues illustrates a peculiarity, which supports, respectively, the rise of the financial intermediaries and the legitimacy of the investment and entrepreneurs practice as a “natural” phenomenon.
The logic employed as an argumentative “sermon” operates as a symbolic violence (Bourdieu, 1997), which induces the individual to strictly control their daily economic practices. People are influenced by biases existing in the financial field, so become an entrepreneur and accumulate wealth; it is part of a natural human condition. The magic that makes up the formulas presented by the consultants has been linked to family metaphors of the universe that are used to justify the financial world (Lakoff, 1996), while concepts such as investment, uncertainty, instability and risk are been part of the individuals and families daily lives.
This dynamic opens way to reflect about transfer the analysis focused on socioeconomic aspects to the cultural field, but this finding may indicate that we are facing the rise of a conservative society, in which a new “order of justice” has been established in Brazil (Douglas, 1996; Grün, 1998). It is possible to observe the existence of an ideology that values the ethics of moral norms reinforced by economic inclusion of families disseminated by a variety of agents, which make up a sort of second nature, that is, a national habitus (Elias, 1986).
Gurus’ performances help to instill in the public receptor a new perception about the financial world. According to Becker (1977), social norms are created by groups or individuals that are called “moral entrepreneurs.” Therefore, these gurus are significant to spread new social values. They are “moral entrepreneurs” of financial and entrepreneurial education.
The books and contents analyzed try to educate and to bring items to be copied – this could transformer the social meanings given to people and social activities. The mechanism used by gurus transforms individual in their collective experience and thus it contributes to the (re) production of habitus (Bourdieu, 1997).
There is a moral influence being created to perform individuals that transforms attitudes and modes of action. Financial education promoters have created a new social space, supported by performances, miraculous calculations, magic and science. Therefore, the idea about happiness and satisfaction are connected to the possession of money (Zelizer, 1994; Simmel, 1990) and linked with the notion of heteronormative family.
“When personal finance becomes the way in which ordinary people are invited to participate in that large abstraction called the economy, a new set of signals are introduced as to how life is to be lived and what it is for” (Martin, 2002). The argument supported by Martin reinforces what happened in Brazil. The personal finance advice creates an “obsession” for making money that dominates lifestyle, personal ambitions and decisions.
Consequently, the risk becomes part of everyday life (Martin, 2002). The moral entrepreneurs also use the argument that, currently, people have no more security in daily life. In a period, where instability and unemployment worries the population, personal finance books show cases of business or professional success based on self-help schemes and family triumph.
In recent years, there has been a proliferation of articles and books heralding this supposed phenomenon. It illustrates the emergence of financial guides services directed to individuals and families in Brazil. In general, these specialists, considered “gurus”, search for guiding individuals toward changes, related to entrepreneurial motivations and practices of risky investments. Currently, these gurus teach about new careers, how to succeed a new enterprise, how to make money; in this sense, they also provide some recommendations about savings and investments.
The most recent studies related to entrepreneurship and women take into consideration researches that focus on the problems they face, their managerial practices, and their skills for success and gender differences. Specifically, the interest in understanding women’s entrepreneurial activities is a result of the importance they are gaining in the entrepreneurial sector as well as evidence that women efforts are been associated with being an entrepreneur, securing their financial independence without leaving their traditional role as mother and as housewife.
In this regard, the results show, in what extent, publications on entrepreneurship and personal finance focused on women “magically” standardizes feminists ideals linked to the notion of material wealth (money) and financial independence, at the same time, it is strictly associated with moral values that defines the “traditional” role of women.
These financial consultants have been infiltrated through different sectors of society, forming a particular dynamic, for example, they are called to give lectures in neopentecostal churches, in this sense, the priests also have transmitted financial thoughts ‘religiously’ to their believers. This movement has resonance in Brazilian society, because it resets the social roles in their place, that is, an important evidence a is that the women are ‘backing home’.
By these evidences, this paper presented that entrepreneur issues are been embedded in Brazilian society. It constitutes a kind of “cultural guide” that addresses a moral topic, supporting the idea of personal and financial success. These speeches are embeddedness by an optimism culture that encourages individuals to take economic choices that were not considered as a moral practice before.
Self-limited epilepsy with centrotemporal spikes (SECTS), well-known as Rolandic epilepsy is the most frequent among the childhood focal epilepsies and may account for about 15–25% of all epileptic syndromes diagnosed between the ages of 5 to 15 years [1]. It is termed ‘rolandic’ epilepsy because the focal seizures are originated from the region around the lower part of the central gyrus of Rolando. The incidence range changes between 7.1–21 per 100000 in a population younger than 15 years with male predominance [2]. The age of onset in 90% of cases between 1 and 10 years with a peak around 6–7 years and recovery occurs before the age of 15–16 years [2, 3, 4].
Self-limited epilepsy with centrotemporal spikes is a syndrome of brief hemifacial motor seizures, frequently having associated somatosensory symptoms, usually without impairment of consciousness which tend to evolve into GTCS [3, 4, 5, 6]. Seizures are often related to sleep [7]. Genetic predisposition is frequent, and there is male predominance [3, 5, 8, 9]. An interictal EEG has normal background activity with biphasic high-voltage centrotemporal spikes, often followed by slow waves that are activated by sleep and tend to spread or shift from side to side [10]. Neurological and mental status before the debut of epilepsy is normal. There are no specific abnormalities on brain MRI or CT. Many studies reported that RE may cause transient or long-lasting cognitive and behavioral disturbances [4, 5, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40].
Panayiotopoulos described a concept of benign childhood susceptibility syndrome (BCSSS) to unify RE, Panayiotopoulos syndrome (PS) and childhood occipital epilepsy of Gastaut (ICOE-G) outlined the common features, course of diseases, prognosis, and the possible genetic predisposition in this group of associated syndromes [5].
1989 ILAE classification recognized three “age-related and localization-related epilepsies and syndromes”: (1) benign childhood epilepsy with centrotemporal spikes (BCECTS); (2) childhood epilepsy with occipital paroxysms; (3) primary reading epilepsy [10].
ILAE Commission on Classification and Terminology lists three childhood idiopathic focal epilepsy syndromes: (1) benign childhood epilepsy with centrotemporal spikes (BCECTS); (2) Panayiotopoulos syndrome, and (3) late-onset childhood occipital epilepsy (Gastaut type) [13].
Rolandic epilepsy had undergone significant terminological and classification changes. RE had been previously considered as benign childhood epilepsy. Frequently reported cognitive and language impairments and behavioral disturbances in children with RE led to the replacement of the terms “benign” and “idiopathic” by the “self-limited” in the new classification proposed by Sheffer I. and colleagues [41].
The role of genetic factors in RE has been presumed since the first high incidence of centrotemporal spikes in family members of patients with RE was reported in 1964 [42]. RE and related syndromes with atypical features do not follow a Mendelian inheritance mode [43]. The clinical and genetic studies have shown complex inheritance [43, 44, 45, 46, 47, 48, 49, 50, 51].
The genetics of CTS is not the same as the clinical genetics of RE [52]. Although CTS is the primary EEG characteristics of RE or ARE, they are also observed in healthy children [53] or the children with autistic spectrum disorders without seizures [54]. Only 10% of EEG trait carriers had seizures [55, 56]. An autosomal dominant mode of inheritance of CTS on EEG has been reported by several authors [42, 55, 56] but it is still debated [52]. The linkage of CTS to ELP4-PAX6 region on 11p13 and chromosome 15q13 [57], 16p12–11.2 [58], and 15q14 [59] have been identified.
Doose et al. investigated the broad spectrum clinical and EEG manifestation of 147 children with RE and their 1266 family members revealed a high incidence of febrile convulsion and afebrile GTCS in patients and their relatives suggested multifactorial inheritance. EEG recordings of probands and their siblings showed a high rate of generalized EEG traits [43].
A multicentral twin study of eighteen twin pairs (10 MZ, 8 DZ) based on a twin database done by Vadlamudi and colleagues demonstrated that the etiology of RE and its inheritance mode is much more complicated than considered before [44].No twin pairs were concordant for RE. Only one monozygotic twin pair has shown centrotemporal spikes on EEG without seizures. Another intriguing finding from this twin data was that all twin pairs with atypical features RE, had a co-twin with seizures although discordant for RE, which emphasized that genetic factors may be more important in atypical cases of RE.
The genetic basis of RE/ARE is polygenic and complex, the interaction of environmental factors or other genes should be considered in etiology of RE spectrum epilepsy syndromes [60, 61]. A number of genes were found to follow the Mendelian inheritance and be associated with RE/ARE (Table 1).
Mutations | Protein function | Special features | Reports |
---|---|---|---|
KCNQ2/KCNQ3 | Voltage-gated potassium channel | BFNS plus RE | Maihara et al. [62] |
BFNS CTS trait | Coppola et al. [63] | ||
BFNS plus RE, RE, CTS trait | Neubauer et al. [64] | ||
SRPX2 | E2A/HLF fusion | RS, oral and speech dyspraxia, MR | Roll et al. [65] |
ELP4 | Elongator subunit | CTS trait, speech disorder, behavior disturbances, ADHD | Strug et al. [57] |
GRIN2A | NMDAR subunit | Atypical RE (LKS/CSWS), intellectual disability, various dysmorphic features | Lemke et al. [66] |
RBFXO1/3 | ATAXIN 2-BINDING PROTEIN 1/HEXARIBONUCLEOTIDE-BINDING PROTEIN 3 | RE, atypical RE, CTS trait | Lal et al. [67] |
DEPDC5 | GATOR complex | RE, atypical RE | Lal et al. [50] |
GABRG2 | GABA receptors | RE, atypical RE | Reinthaler et al. [68] |
CAMK2A | Subunit of calcium/calmodulin-dependent protein kinase II | Atypical RE, intellectual disability and autism | Rudolf et al. [45] |
GRIN2B | NMDAR subunit | Atypical RE, epileptic encephalopathy, intellectual disability | Rudolf et al. [45] |
CHRNA4 | Neuronal nicotinic acetylcholine receptor α4 subunits | RE (familial case) | Neng et al. [69] |
Genetic mutations associated with RE/ARE spectrum.
Lemke et al., have identified GRIN2A mutations in 20% of patients with ARE associated with neurocognitive disturbances [66].
Although mutations in PRRT2, KCNQ2, KCNQ3, RBFOX1, and DEPDC5 genes with an autosomal dominant transmission reported in patients with RE spectrum epilepsy syndromes, they have not been confirmed by the studies based on large case series [70].
With the exception of GRIN2A and ELP4, many genes currently associated with RE/ARE, including KCNQ2, KCNQ3, CHRNA4, DEPDC5, RBFOX1/3, BDNF, and GABAA-R, were initially linked to other neurogenetic conditions, and later their phenotypes were expanded to RE/ARE.
The main seizure type in RE according to the ILAE 2017 seizure classification is focal aware seizure consisting of motor-hemifacial tonic or clonic contractions, oro-pharyngo-laryngeal symptoms, sensory symptoms represented by unilateral numbness or paresthesia of tongue, lips, gum, and inner part of the check, and associated with speech arrest, hypersalivation, and focal to bilateral seizures [2, 3, 4, 41, 71]. Hemiconvulsions and bilateral tonic–clonic seizures are less frequently observed ictal features, mainly seen in younger children due to rapid distribution of focal onset seizures [2, 3, 4, 6]. Hemiconvulsions may be followed by post-ictal Todd’s hemiparesis in 10% of cases [8, 72].
Seizures are brief, usually last from 30 sec to 2–3 minutes or longer if turn into bilateral tonic–clonic seizures [6, 18, 72]. Seizures mainly occur during night sleep or drowsiness, whereas the probability of awake seizures is less than 10% [73, 74]. Seizure frequency is low, most patients have less than 10 seizures, 10%–20% of patients have a single seizure [75]. Consciousness is completely preserved in around 60% of patients with RE [5].
Focal motor seizures in approximately one-third of cases manifest as unilateral oral-facial tonic or clonic contractions. These are brief (few seconds −1 min), a sudden burst of clonic contractions of the face, which may be entirely localized in the lower lip or spread to the ipsilateral upper and very rare to the lower extremities [1, 2, 3, 4, 5, 71, 76].
Tonic deviation of the mouth is frequently observed ictal motor manifestation [5].
Oro-pharyngo-laryngeal symptoms are mostly motor ictal phenomena with the involvement of the (epi-) glottis and pharynx (> 50%) produce guttural bizarre sounds, resembling gargling, grunting, wheezing [72]. These may be accompanied by contractions of the respiratory and abdominal muscles (vomiting like contractions) which appear in more than half of seizures [77]. They consist of unilateral sensory and motor manifestations inside the mouth, tongue, inner cheek, gums, teeth, and pharyngolaryngeal regions [3].
Speech arrest occurs in >40% of seizures with dys - or anarthria [3, 72]. The child usually is aware, with preserved receptive language, attempts to communicate with gestures, but unable to produce a single intelligible word [3, 5]. Speech arrest is considered more as a motor ictal manifestation associated with the loss of the power and coordination for the articulation of words [3]. There is no impairment of the cortical language mechanisms [4, 5].
Focal non-motor seizures commonly observed in RE.
Sensory symptoms may manifest as unilateral numbness or paraesthesias like tingling, prickling, freezing and their variations in the parts (rarely involve the whole area) of oral-facial-pharyngeal area, usually tongue, inner cheek, gum, teeth, lips [3, 4, 6]. Sensory seizures often occur in combination with motor seizures and hypersalivation [3, 5, 72].
Hypersalivation is one of the most characteristic autonomic ictal symptoms of RE, occurs in one-third of cases [2, 3, 4, 5, 71]. It is frequently associated with hemifacial motor symptoms. As well as the awareness is not disturbed in most of the cases, children usually are able to describe their sensations as sudden filling of the mouth with saliva and air, difficulty in pronouncing words, a lot of saliva flowing from the mouth [5].
Other autonomic ictal manifestations as ictal emesis and ictal syncope may observe rarely in RE. Although autonomic seizures are the cardinal symptom of Panayiotopoulos syndrome, they are reported in RE [74, 78, 79, 80, 81, 82]. The overlap of the clinical and EEG features of PS and RE has been widely investigated by several authors [5, 74, 79, 80]. The cases where two different types of childhood focal seizures presented at the same time or one form of epilepsy progressed to another have been thoroughly reported by different investigators [74, 79, 80, 81, 82, 83, 84, 85].
Focal to bilateral tonic–clonic seizures are a frequent seizure type present in one to two-thirds of children with RE. FBTCSs mostly appear during night sleep [86].
Status epilepticus is seen rarely and usually associated with an atypical course of the disease [87].
Focal motor SE occurs more often than generalized convulsive SE [3]. This state consists of unilateral or bilateral hemifacial contraction, subtle perioral myoclonus, speech arrest, dysarthria, excessive drooling, swallowing difficulties [88, 89, 90, 91, 92, 93].
The EEG picture is distinctive in Rolandic epilepsy. The background activity is almost always preserved in an awake state and during sleep [91]. The characteristic interictal EEG pattern- centrotemporal spikes (CTS) or rolandic spikes are regarded as the neurobiological markers of RE. CTS is high-amplitude (usually > than 150 mkV) biphasic spikes or sharp waves of ∼ 70–80 milliseconds duration frequently followed by a slow activity on the central-mid temporal region (C3/C4, T3/T4) [2, 3, 4]. More posterior localization of CTS is often observed in the youngest patients [94]. The spikes may occur isolated or in clusters, in one or both hemispheres [95, 96] (Figure 1A and B). A focal rhythmic slow activity over the centrotemporal region is occasionally observed [2]. The most typical finding of the rolandic spikes is their significant increase in frequency during NREM sleep [74] (Figure 1C-E). The spikes appear only in sleep in about a third of children [97].
7y.old boy with rolandic epilepsy. (A) Interictal awake EEG. Left centrotemporal spikes with rapid spreading to the right frontal-anterior temporal region. with maximum negativity over the C3T3,F7 and maximum positivity on the right-midline frontal-parietal region F4Fp2FzPzCz(AV montage, sensitivity 20Mkv/mm, paper speed 30mm/sec, LFF 70 Hz, HFF 0.5 Hz, Rejector 50 Hz). (B) Increased paper speed clearly demonstrates propagation of left centrotemporal spikes to the right frontal-anterior temporal region (AV montage, sensitivity 20 Mkv/mm, paper speed 120 mm/sec, LFF 70 Hz, HFF 0.5 Hz, Rejector 50 Hz). (C and D) Interictal 1NREM sleep EEG. The frequency of CTS of the same distribution is increased. (E) The same EEG sample in bipolar longitudinal montage demonstrates phase reversal on both right and left centrotemporal region (C3C4T3T4).
EEG and magnetoencephalography (MEG) studies show a stable horizontal dipole coming from the lower rolandic region with maximal electronegativity in the centrotemporal region and electropositivity in the frontal region, usually seen unilateral or bilateral [98, 99, 100]. Spikes may often appear in the central, parietal, midline, or even occipital regions which do not exclude a diagnosis of RE [98].
Somatosensory stimulation by the tapping of hands or feet or electrical stimulation of fingers at 1 Hz may activate CTS and somatosensory evoked potentials (SEPs) on the contralateral hemisphere [101].
Brief interictal generalized bursts of 3–5 Hz slow waves with intermixed small spikes distinctive than a pattern 3 Hz spike–wave seen in CAE may observe in about 4% of patients with RE [85, 102].
Many studies have tried to identify the source of rolandic discharges using topographic analysis, source modeling techniques, dipole tracing method, magnetoencephalography (MEG), and functional MRI (fMRI) investigations [99, 100, 101, 103, 104, 105, 106]. The functional MRI (fMRI) triggered by EEG of the rolandic spikes as well as MEG showed activation of the sensorimotor area [104], mainly in the orofacial division of the primary sensorimotor cortex [105]. However, it is challenging to distinguish the precentral or postcentral origin of CTS [103]. Ishitobi et al., suggested the precentral origin of rolandic spikes explained this theory by the continuity of cortical surface polarity from negative gyral cortex to the surface positive interhemispheric fissure based on the combination of scalp EEG and MEG [103]. Gregory and Wong analyzed 12 independent foci in 10 patients with RE assumed that the generator of a dipole discharge was located halfway between the maximum negative and positive poles, and was most likely situated at the depth of the lower rolandic fissure or Sylvian fissure [107]. The propagation pattern of rolandic spikes first studied by Jung et al., suggests that spike propagation was caused by intracortical spreading a single dipole across the central sulcus [108].
CTS are diagnostic markers of RE only in a suggestive clinical presentation [74]. It has been widely reported that 1.2 to 3.5% of normal healthy children population between 5 and 12 years old [109, 110], 6–34% of siblings and relatives of patients affected by RE [9, 111, 112], children with migraine, behavior disturbances, ADHD, variety of organic brain diseases with or without seizures, such as cerebral tumors, Rett syndrome, fragile X syndrome and focal cortical dysplasia [84, 113] also show CTS in routine EEG recording.
The first described ictal patterns are characterized by a quite monomorphic sequence of rhythmic sharp waves or spikes without significant post-ictal slowing [91, 114]. In 1990, Gutierrez et al. described an ictal event with speech arrest only characterized by a short train of ictal alpha activity, and then two multiple spikes and wave complexes originated from the left centrotemporal region followed by marked attenuation of the left hemispheric background [115]. Subclinical rhythmic discharges of spike and wave in the centrotemporal region have been documented by several authors in RE [116, 117]. Saint-Martin et al. in 2001 described a series of patients presenting with typical and also atypical ictal manifestations such as falls, negative myoclonus and observed that positive motor phenomenon correlated to the spike component preceding a negative motor phenomenon, correlated with the slow-wave component of the spike and wave complex [118].
Capovilla et al. recorded 34 seizures in 30 patients with RE and described four electrographic seizure patterns thus emphasizing that ictal pattern for RE is not unique [116]:
low-voltage activity of fast rhythmic spikes, increasing in amplitude and decreasing in frequency observed in the majority of patients,
a discharge of spikes intermixed with sharp waves increasing in frequency and amplitude,
monomorphic theta which progressively formed a discharge increasing in amplitude and decreasing in frequency,
initial focal depression of the electrical activity, followed by one of the three above described patterns.
Ictal EEG source analysis of 3 patients with RE demonstrated the activation of the opercula-insular area, time-locked to the contralateral focal myoclonic jerks [119].
In most cases, children with RE have a good prognosis regarding both seizures and neurodevelopment [120, 121]. The remission of seizures usually occurs before the age of 18 years [11, 98]. The cognitive and behavior problem may happen in an active period of disease which is reversible in most patients [11, 12].
Rolandic seizures occur in a period of significant brain maturation. The dysfunction of neuronal network activities such as focal discharges may be associated with neuropsychological problems, including, linguistic, cognitive, and behavioral impairment [28, 29, 30, 122]. The frequent spike discharges in sleep may boost language and attention processing problems [120, 123, 124, 125].
Mood and behavioral disorders were present in nearly a third of children (30.9%) with RE [126, 127, 128]. Retrospective studies have proposed that early age at onset pretends a more aggressive seizure course [18, 129, 130, 131].
Functional MRI study revealed CTS density caused hemodynamic changes even during wakefulness can interfere with the normal brain-language network and the bilateral insular cortex [132].
The neuropsychological tests such as Wechsler Intelligence Scale for Children-III (WISC-3rd), verbal fluency test, Wisconsin card sorting test, attention deficit diagnostic scale, and child behavior checklist scale are usually administered to measure a wide range of skills and cognitive functions of RE patients [35, 36, 37, 38, 133].
Many researchers showed a variety of neuropsychological deficits, behavioral and emotional difficulties in a limited cohort of patients with RE range from 19 to 67% [22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 134, 135]. The series of reported children with uncomplicated RE were described lower average results on neuropsychological tests involving visuomotor coordination, some executive functions, sustained attention, and language issues like spelling, reading aloud, reading comprehension, memory, and learning of auditory–verbal material, delayed recall, and verbal fluency, compared with controls. However, the Full-Scale Intelligence Quotient (FSIQ) was not significantly low in most of them.
D’Alessandro et al. investigated the neuropsychological data of 44 children with RE who did not have a seizure for more than 6 months without treatment. Attention, language, and visuomotor coordination tasks problems were more severe in children with a bilateral epileptiform discharge. However, in a follow-up assessment for 4 years, a re-examination of 11 children had revealed the normalization of cognitive functions in all [21].
Several studies reported that cognitive abnormalities and behavioral impairments are associated with a high interictal spike frequency [24, 25, 35, 120], the number of interictal abnormalities in wake or sleep [136], activation of interictal spikes during sleep [118, 137], and the presence of non-tangential dipole spikes [73].
Piccinelli et al. [138], investigated the frequency of specific learning disabilities such as reading, writing, and calculation in patients with typical RE and possible related electroclinical findings. They reported children with RE who developed seizures before age 8 years and had epileptiform discharges more than 50% of the sleep EEG recording in several tracings over more than a year were at risk of developing academic difficulties [138].
EEG may predict educational and behavioral impairments in children with RE. The presence of an intermittent slow-wave focus during wakefulness, a high number of spikes in the first hour of sleep (and during whole night sleep), and multiple asynchronous bilateral spike–wave foci in the first hour of sleep are associated with learning problems in children with RE [16, 139].
RE can present or evolve to an atypical form, characterized by atypical ictal semiology, different EEG findings, and poor neuropsychological outcomes [19, 140, 141].
Massa et al. described 5 interictal EEG patterns that significantly correlated with atypical evolutions of RE: [41] intermittent slow-wave focus; [2] multiple asynchronous spike–wave foci; [3] long spike–wave clusters; [4] generalized 3-c/s “absence-like” spike–wave discharges; [1] conjunction of interictal paroxysms with negative or positive myoclonia, and abundance of interictal abnormalities during wakefulness and sleep [136].
Several studies have shown an association between atypical rolandic epilepsy and known genes (Table 1). The identification of de novo or inherited mutations of N-methyl-D-aspartate (NMDA) receptor subunit-encoding genes (GRIN2A and GRIN2B) linked to speech and language, cognitive impairment, and behavioral difficulties have been a significant breakthrough in the understanding of the nature of atypical RE [142, 143, 144, 145]. Another relevant gene is elongation factor protein 4 (ELP4), which is associated with language impairment, autism spectrum disorder, mental retardation, and epilepsy with centrotemporal spikes on EEG [146].
Atypical rolandic epilepsy (ARE) is a severe epileptic condition especially with regards to cognitive consequences. The first description of atypical features of RE was published by Aicardi & Chevrie in 1982 showed rolandic epilepsy presenting periods with new types of seizures, mainly atonic and myoclonic, associated with continuous spike-and-waves in slow-sleep EEG (CSWS/ESES), and transitory learning difficulties [147]. Doose and Baier described similar patients with atonic fits leading to daily falls which is the hallmark seizure type for Lennox–Gastaut syndrome and termed the condition “pseudo-Lennox syndrome” to differentiate this two distinct conditions [148]. Patients with ARE have significantly lower full-scale and verbal IQ than the patients with typical RE [149]. Neuropsychological impairment, which may sometimes be present before the onset of the disease, is constantly present during the clinical course, but in contrast to ESES and LKS, the cognitive outcome is always favorable [92, 150]. Clinical semiology consists of typical for RE focal seizures, generalized tonic–clonic seizures, atypical absences, myoclonic seizures, and atonic seizures. The atonic attacks may involve the whole axial musculature or be localized, causing repeated brief (0.5–2.0 s) atonic episodes in the head or a limb (epileptic negative myoclonus) that usually occur for periods lasting one to several weeks, separated by seizure-free intervals of weeks or months [6, 90, 92]. Such atonic attacks are associated with the slow-wave component of spike and wave complexes, and the location of the EEG discharges corresponds to that of the atonic episodes [151, 152]. Interictal awake EEG shows bilateral sharp and sharp-slow wave complexes with higher amplitude in the rolandic area, which increases during sleep with bilateral synchronization [90, 92, 116, 153].
Using carbamazepine may promote the diffusion of spike–wave activity from the rolandic focus to induce atonic seizures, atypical absences in patients with RE [154].
Rolandic status epilepticus refers to status epilepticus that can be convulsive or non-convulsive, and either generalized or focal lasting days or weeks including motor facial seizures, oromotor dyspraxia, anarthria with persistent drooling and swallowing problems [155]. The interictal EEG usually shows focally or bilaterally synchronous sharp waves or sharp and slow wave complexes predominant in the rolandic area with a tendency to become continuous during sleep [146, 155]. The condition can be resolved with a good neurocognitive outcome with appropriate treatment [146]. These seizures can persist for more than 1 month without treatment [156, 157].
The decision whether to treat children with RE or not requires a particularly careful risk–benefit analysis [2, 158, 159, 160, 161, 162, 163]. Many authors suggest that drug treatment is not necessary for typical RE because of its good prognosis, and usually infrequent nocturnal seizures [114, 154]. Moreover, in 40–50% of cases, the seizures are difficult to control with drugs [148]. Besides, the treatment with AED usually does not influence the duration of active epilepsy [163].
However, treatment may be indicated in patients with frequently recurring daytime seizures, generalized tonic–clonic seizures, young age at onset [164], or when the ictal events are disruptive to the patient or family [161, 163]. Furthermore, the presence of cognitive and behavioral disturbances, either transitory or persistent has to be considered [2, 5, 91, 92]. There is no single solution supported by definitive evidence which AED is more effective in the treatment of RE.
Internationally, carbamazepine (CBZ 20-40 mg/kg/d [165]) and valproate (VPA 20-30 mg/kg/d [166]) are the most often prescribed AED for children newly diagnosed RE [167]. However, the possible worsening of EEG in rolandic epilepsy by some drugs and particularly by CBZ, increasing epileptiform abnormalities during sleep, and inducing epileptic negative myoclonus have been reported [154].
Sulthiame, levetiracetam, and gabapentin were studied in a randomized controlled trial [158, 159, 167, 168, 169]. Sulthiame administered varied between 3.1 and 5.7 mg/kg/day was effective in controlling seizures in children with RE [159].
A prospective, open-label, pilot trial evaluating the efficacy and tolerability of levetiracetam (LVT 20-30 mg/kg/d) or oxcarbazepine (OXC 20-35 mg/kg/d) as monotherapy in two parallel groups of newly diagnosed RE patients demonstrated effectiveness in controlling seizures a follow-up period up to 2 years [162].
A randomized controlled multicenter trial comparing the effects of either Levetiracetam or Sulthiame on EEG in RE showed a reduction of epileptiform discharges after 12 weeks of treatment [158]. Persistent epileptiform discharges after 12 weeks of treatment are associated with recurrent seizures [158].
When the presence of ESES associated or not with negative myoclonus, clinical status, or acquired aphasia is detected in children with RE, a change of antiepileptic drugs should be considered. Class IV studies suggest that sulthiame, benzodiazepines, ethosuximide, and, in most severe cases, corticosteroids might be useful [91, 92].
Duration of treatment in RE should not exceed 1 year following the last seizure, regardless of EEG changes [2].
We are grateful to Dr. Sándor Beniczky for his valuable comments on EEG figures.
None of the authors has any conflict of interest to disclose.
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