Articles – Page 72

Immune-Mediated Neurologic Disorders Recognition and Management in LMCs

FROM THE EDITOR-IN-CHIEF

Donald H. Silberberg

By Donald Silberberg

Since Josep Dalmau’s 2007 report of Anti-N-Methyl-D-Aspartate Receptor Encephalitis associated with ovarian teratoma¹, there have been several studies that make it clear that he and his colleagues uncovered just the tip of the iceberg. Clinical presentations ranging from seizures, movement disorders, autonomic dysfunction, behavioral changes to frank psychosis and disordered consciousness are being reported in association with a variety of antibodies, mostly directed against synaptic molecules². Some proportion of what is being termed Pediatric Autoimmune Neuropsychiatric Disorders (PANS) also may be antibody-mediated³.

Although these seem to be uncommon disorders, their epidemiology at present is unknown. However, as is the case with virtually all neurologic disorders, immune-mediated disorders are likely to be at least as common in low- and middle-income countries (LMCs). In those situations in which a preceding infection plays a role, these disorders may even be more common. Their recognition depends on physician or other health care provider awareness; diagnosis depends entirely on sophisticated laboratory testing. This raises the problem of how to get affordable diagnostics and interventions to LMCs where they are needed.

This problem, of course, extends far beyond this group of disorders. More than 100 genetically determined disorders can now be detected before birth. Even more neurologic disorders can be accurately diagnosed throughout childhood and adolescence where the genetic testing is available.

Advances in the capacity to deal with this issue in LMCs will depend on raising awareness at many levels, training diagnostic laboratory personnel, lowering the cost of the reagents that are needed, and through developing partnerships with centers in wealthy countries.

World Neurology Online welcomes your commentary concerning this issue.

References

1. Dalmau J, Tuzun E, Wu HY, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61:25

2. Panzer JA, Gleichman AJ, Lynch DR. Glutamatergic autoencephalitides: an emerging field. Neuro Oncol. 2014 Mar 16. [Epub ahead of print]

3. Murphy ML, Pichichero ME, MD Prospective Identification and Treatment of Children With Pediatric Autoimmune Neuropsychiatric Disorder Associated With Group A Streptococcal Infection (PANDAS). J Neural Transmission. 2014 Jan 9 [Epub ahead of print]

Asia Initiative: Neurology Service and Beyond

Ryuji Kaji MD, PhD

In his 2010 inauguration speech as the president of World Federation of Neurology (WFN), Prof. Vladimir Hachinski conveyed a message: “Asia has more than 60 percent of the global population, yet in some areas, the education of neurology to young neurologists does not keep up with the patients’ needs of neurological care.” He organized the Asia Initiative as a part of WFN to bring attention to this region. As the chair of this initiative, I have met many Asian neurologists and have begun to realize that there are unique problems that require attention from the rest of the world.

The 14th Asian & Oceanian Congress of Neurology was held in March in Macao, China. This is an official meeting of Asian Oceanian Association of Neurology, whose history dates back almost half a century ago. Charles M. Posner, a WFN representative, toured the Asian and Oceanian countries and challenged local neurologists to form an association that would promote and foster the advancement and exchange of information within the area. In response, Shigeo Okinaka, then the executive of Japanese Society of Neurology, invited the region’s neurologists to a planning meeting in Tokyo. This gave birth to the Asian and Oceanian Association of Neurology (AOAN) on June 26, 1961. Its official meeting, Asian & Oceanian Congress of Neurology (AOCN), had been held every four years until the current 14th meeting in China, which followed the 13th meeting in Melbourne in 2012 by two years. There was uncertainty over the financial and scientific outcomes of this short-interval meeting, but thanks to the dedication of the Hong Kong Society members, it became an unprecedented meeting with the largest number of international participants ever. Its surplus funds should significantly contribute to the activities of AOAN, chaired by Dr. Man Mohan Mehndiratta from India. The next AOCN will be held in 2016 in Malaysia.

On March 4, there was a plenary session on “Special Issues in Asia,” in which three speakers spoke. Prof. Chong-Tin Tan from Malaysia gave a talk on education in Asia. He serves as the editor of Neurology Asia, the official journal of AOAN and Association of Southeast Asian nations Neurological Association (ASNA). He pointed out that Asia accounts for 60 percent of the world population, but less than 20 percent of neurologists in the world, and stressed that education is the key to development of neurology in Asia. Next, Dr. Li-Ping Liu from China emphasized rapidly advancing frontiers of neuroscience research in China. At the last of this session, I gave a talk on neurology service in Asia. Asian countries are rapidly exploding in population and economy. Some are facing unique problems not experienced in other regions¹.

Figure 1. Projected increase of the aged (>65) populations among nations.
Adapted from Current Status and Predictions for an Aging Society with Fewer Children, Japanese Ministry of Education, Sports, Culture, Science and Technology (with permission).

Figure 1 depicts the projected increase of the population over 65 years among nations. While Western countries have a linear increase of the aged over years, Asian countries (Japan, Korea and China) have S-shaped curves indicating a steep surge of the aged population during 2000-2020 (Japan) and 2020-2030 (Korea and China). India will probably join this group by the end of this century.

Japan is the first to be exposed to this surge, and medical needs for the aged people are highlighted. Among these, neurological disorders such as stroke and Alzheimer’s disease came to the forefront. For instance, the number of stroke survivors has steeply increased from 1.7 million in 2000 to 2.8 million by 2013. Stroke was the No. 2 killer in the 1970s, but it is currently the fourth, following cancer, heart disease and pneumonia. Although the number of stroke attacks is six times as frequent as heart attack, it has largely become non-fatal, although it typically leaves disabilities; two- thirds of the patients are unable to return to pre-morbid activities.

From the global point of view, stroke is the second leading cause of death after ischemic heart disease, with an estimated 5.5 million people dying from stroke every year worldwide. Two thirds of these deaths occur in countries with limited resources². Approximately 80 percent of patients survive the acute phase of stroke, but are left with varying degrees of chronic disability.

Not only the number of deaths, but also the quality of life after stroke is an important aspect. Disease-adjusted life years (DALYs) are defined as number of years of healthy life lost by disease³. They reflect the impact of a disease in the aging societies.

Figure 2. Disease-adjusted life years (DALYs) of neurological diseases with respect to nation’s income status. Data from WHO (2005).

Figure 2 shows DALYs among various neurological diseases with respect to the economic status of a nation. Stroke is more important than Alzheimer’s disease, because many Asian nations are still in the state of low-middle income by World Bank Criteria. Japan had been among those with limited resources with high mortality of stroke, but her economy grew up in a short period to high income state, and other Asian nations should follow this path.

The drop of stroke mortality in Japan is due to change in diet, western lifestyle and the efficient social health care system. However, the main factor is the control of hypertension by medication, which decreased the number of fatal massive hemorrhages³. The impact of thrombolytic therapy is limited, rather increasing those disabled by preventing stroke deaths⁴.

The expense related to caring for stroke survivors is now exceeding $25 billion (U.S. dollars) per year in Japan. Now the Japanese economy is revolving around those aged patients and their care. In fact, a major diaper maker saw sales of adult diapers outpace infant diapers. Stroke centers with staff dedicated to thrombolytic therapy are urgently needed, and we are investing effort into increasing awareness of stroke among Asian people. Educating health professionals in neurology is the Asian Initiative’s first priority.

The roots of neurology lie in Europe and this specialty matured in the U.S. Asia has its own priorities in coping with neurological diseases. In this regard, I propose “autonomy” as a key word for activating regional neurological organizations. We need a forum to discuss the problems in each region, and to provide unique educational opportunities for neurologists, general practitioners, allied health professionals and the patients. With these aims in mind, the WFN has decided to make the best use of the existing regional neurological organizations such as AOAN to fulfill its mission.

Another key for success would be “synergy”. Symposia and workshops were held in collaboration with international organizations such as the Movement Disorder Society (MDS) and the International Federation of Clinical Neurophysiology (IFCN). These joint activities provided the financial support for the meeting and increased the attendance. The event also helped the supporting organizations increase their visibility. I hope that the March 2014 meeting in Macao becomes a model for the future meetings in Asia, and the meeting will serve as an equivalent to EFNS, ENS or American Academy of Neurology meetings.

Alzheimer’s disease is a little harder to tackle. Traditionally, Asian people had a large family, three generations living together. The aged people lived with younger family members. In my childhood in 1950s, the aged are naturally thought to have memory loss to some degree. It is still a virtue that children respect and take care of the parents by Asian standard. Re-appraisal of this system in the face of increasing Alzheimer’s disease might be a solution for countries with limited resources. For the aged, it would be appropriate to prepare for the intellectual decline.

Steve Jobs, the former CEO of Apple Computer, gave a speech at the commencement at Stanford in 2005. Facing the recurrence of cancer, his message on coping with imminent death might be a hint: in the morning he thought about his life as if ending in the evening. Whenever he thought the activities of the day were not what he really wanted to do, he changed his life. These were actually the words of the old Chinese philosopher, Confucius (551–479 BC), which he probably was familiar with. Alzheimer’s disease is still unpreventable and incurable probably for the next decades to come. “Think Oriental” might be the key for the societies and the neurological community in the world.

References

1. Kaji R Asian Neurology and Stroke (Perspectives) Neurology, in press

2. WHO, W. Neurology Atlas, (2003).

3. Murray, C.J. et al. Disability-adjusted life years (DALYs) for 291 diseases and injuries in 21 regions, 1990-2010: a systematic analysis for the Global Burden of Disease Study 2010. Lancet 380, 2197-223 (2012).

4. Kubo, M. et al. Secular trends in the incidence of and risk factors for ischemic stroke and Its subtypes in Japanese population. Circulation 118, 2672-8 (2008).

Kaji, is the Chair, Asia Initiative, World Federation of Neurology, Professor and Chairman, Department of Neurology, Institute of Health-Bioscience, Tokushima University.

ENS + EFNS = EAN

Wolfgang Grisold

By Wolfgang Grisold

Earlier this year, the first Joint Congress of European Neurology took place in Istanbul. This was the final stage in the process of merging the two European neurological societies — the European Neurological Society (ENS) and the European Federation of Neurological Societies (EFNS) — into the European Academy of Neurology (EAN). The first elections of the new society were held during the congress, naming G. Deuschl, Germany, as the first president of the EAN, and F. Fazekas, Austria, as its first vice president. The merger of the two societies was a logical step and now that it has occurred, it seems to be a natural development, but it took a huge effort and the engagement of many individuals to make it possible.

The ENS was the first European neurological society, founded in 1986. It held its first congress in Nice in 1989. That congress was organized by G. Said.

Said along with his co-founders, P. K. Thomas and Anita Harding, were the heart of the ENS at its founding. Their idea was to create a European society based on individual membership, with a strong emphasis on science and a structure similar to that of the American Academy of Neurology (AAN). As a consequence of this philosophy, the ENS traditionally has been less engaged in political aspects of neurology, fostering individual rather than national representation. The ENS, apart from holding excellent congresses, increasingly engaged in education through teaching, scholarships and internationalsupport for countries in need. Its major publication was the Journal of Neurology, which was supplemented by informative newsletter that appeared regularly.

The EFNS was founded in Vienna in 1991 by Prof. Gerstenbrand who had a vision of creating a large European society based on prior activities in Eastern Europe, such as the International Danube Symposia and the proposed Pan-European Society of Neurology. The philosophy of the EFNS was federal in constitution and structure, where individual European countries were the constitutional members. This construction was effective. Since its founding, the EFNS has contributed greatly to European neurology. The EFNS sponsored a number of activities, many of which were originally conceived within the EFNS, promoting education, CME accreditation and supporting neurology in the former Eastern European countries, by teaching courses, holding lectures and helping in the establishment of national neurological organizations. The primary publication of the EFNS is the European Journal of Neurology, which will become the official journal of the EAN. Neuropennews is an online publication serving the purpose of a newsletter.

Joint Congress of European Neurology Due to differences in their philosophies, the two societies were effectively in competition, resulting in what many European neurologists concluded was an unnecessary duplication of effort. The initial event of their coming together was the creation of the European Board Examination in Neurology, where the examination’s creator, the UEMS – European Board of Neurology (UEMS/EBN) invited both societies to participate in its development. The presidents of the two organizations at that time — De Reuck of the EFNS and Moonen of the ENS — were both from Belgium. That facilitated the process of initiating a proposal for the creation of a joint EAN.

There were many additional steps necessary for the eventual merger. One major step was the agreement of the European delegates of the EFNS to this proposal in Florence 2009 and the subsequent agreement of ENS members. A joint task force was then formed that meticulously prepared the details for the merger and also created a new constitution and voting system.

The EAN combines the best of the developments of both societies. It is expected to foster neurology within Europe for the best of patient care, and to support and encourage science, teaching and education, as well as to be a transparent, open-minded and member-oriented society that provides an essential service for its members.

The WFN acknowledges this development toward a strong European society, and we hope to cooperate in further projects, especially where those involve furthering neurological education in countries in need of additional resources.

Grisold is from the Department of Neurology, Kaiser Franz Josef Hospital, Vienna, Austria.

Maynard M. Cohen (1920-2014)

Maynard M. Cohen, MD, PhD

Great scientist, one of the pioneers of the American modern neurology, lover of philosophy and culture, philanthropist. Maynard M. Cohen, MD, PhD, professor and chairman emeritus in the Department of Neurological Sciences at the Rush-Presbyterian-St. Luke’s MedicalCenter in Chicago, and past AAN president, died on Feb. 18, 2014, in Miami in his 94th year.

As the world-renowned neurologist, he was the U.S. delegate to the World Federation of Neurology (1985-1989). At the delegates’ preparatory meeting (1987) in New Delhi for the XIVth WCN, he strongly supported the organization of the symposium, proposed by Yugoslav and Indian neurologists: “Neurology in Developing Countries” during the New Delhi WCN in 1989. After the successful symposium, in which our respected friend gave a beautiful contribution, the then-WFN President Richard Masland and the then-Chairman of the WFN Research Council John Walton proposed to form a new Research Group on Organization and Delivery of Neurological Services (WFN RG ODNS) in order to search for best solutions in delivery of neurological services around the world (both in industrial and developing countries), respecting local and regional, social and economic conditions.

Cohen accepted to serve as secretary treasurer of the new research group, participating in the new activities in the Federation. As co-editor of the book, he cared that contributions from less developed countries find a place in it. He concluded his own chapter with visionary words: “This symposium, the support of the World Federation of Neurology, and the formation of the Research Group on Organization and Delivery of Neurological Services are but the initial steps. The larger task still lies ahead.” (Neurology in Developing Countries, B.I. Churchill Livingstone, 1991, p.75).

Despite his years, he showed remarkable enthusiasm and energy as co-organizer of our meetings (New Delhi, Vienna, Marrakesh, Vancouver and others), as well as initiatives in many parts of the world. He organized an important symposium, “Ethical Problems in Neurology,” concerning problems in managing neurological ailments due to various cultural and religious traditions (XVth WCN Vancouver 1993), engaging the leading experts in the field from the U.S. and other countries of the globe.

During his service (until 1995), he supported activities of the group. When we needed new, experienced colleagues for the organization, he proposed new active members for the RG Committee: Donna Bergen and Don Silberberg, who have stayed active in the WFN. His wife, Doris Vidaver, participated kindly with her experience in humanities and in medicine, especially in neurology.

The World Federation of Neurology will memorialize his activities, for which he received in 1993 the WFN Certificate of Appreciation. They are now being continued also as an integrative part of the WFN mission and programs. Many neurologists active in those years still remember our early initiatives and the humane care of Maynard M. Cohen for people around the world.

Bosko Barac, MD, DMS, is the founding chairman of the WFN RG ODNS (ret.).

Theodore L. Munsat, MD: A Tribute from the Sociedad Neurologica Argentina

Ted Munsat worked with the WFN to improve education in developing countries like Argentina in 1997.

Never too late for a tribute, especially for a man like Theodore Leon Munsat (1930-2013). He was an extraordinary man and a natural educator who gave the opportunity to improve neurology worldwide.

Among other educational activities, Munsat helped launch the American Academy of Neurology’s (AAN´s) continuing education publication, Continuum, was chairman of the World Federation of Neurology (WFN) Amyotrophic Lateral Sclerosis (ALS), chairman of WFN Research and Education Committees, served as president of the AAN (1989-91), and was emeritus professor of neurology at Tufts University School of Medicine, between other relevant activities. Indeed, exceptional merits for only one man.

He was honored with the A.B. Baker Award for Education from the AAN, the Sheila Essey Award for ALS Research and the Lifetime Achievement Award from the WFN Research Group on Neuromuscular Diseases.

He developed a number of successful educational programs. More than 42 countries all over the world were benefited with a continuing medical education (CME), using the journal Continuum, generously donated by the AAN with an extraordinary local impact.

He worked within the WFN to improve neurology and education in many developing countries, including educational programs in Honduras and Argentina. He directed his energies to teaching neurology internationally.

I met Munsat at the WFN World Congress of Neurology 1997 in Argentina with his wife Carla. We invited him to an International Symposium as part of the scientific activities after the congress, in Tucumán, CalchaquiesValleys, in the North of Argentina, where several conferences of ALS took place, with Alan Mac Comas, Roberto Sica and other international leaders in neuromuscular diseases.

In that opportunity began the idea to develop an educational program for Argentina.

The educational program began in 2003 and benefitted over a decade of continuity to hundreds of neurologists in our country with a significant educational imprint.

I want to highlight his passion for education, intelligence, humor, kindness with a great ability to make easier the hardest things, a natural leader, a gentleman, an honorable man. He had a warm and sincere personality. he loved helping others every chance he could.

I admired and respected him as a man, as a professional and as a teacher. He has left a profound mark on the world of neurology. The Sociedad Neurológica Argentina wishes to give this tribute and extend our gratitude. Dear Ted, thank you very much for all you have done; we shall remember your legacy always.

Editor’s Update and Selected Articles From JNS

By John D. England, MD

John D. England, MD

On behalf of the Editorial Board, I would like to thank all of the individuals who review articles for the Journal of the Neurological Sciences (JNS). The integrity of a scientific journal such as JNS depends heavily upon the quality of independent peer-review.

I continue to be impressed with the high quality and thoroughness of the reviewers’ critiques of manuscripts. I am especially impressed and thankful that such busy and committed individuals still take the time to review articles. One of my goals for this year is to seek advice from the Editorial Board and Elsevier about how we might be able to recognize the efforts and importance of our reviewers in a more tangible manner.

Most readers are becoming aware of the fact that Elsevier, the publisher of JNS, now provides free access to selected articles from JNS for members of the World Federation of Neurology. In consultation with members of the Editorial Board, I select two “free-access” articles, which are profiled in each issue of World Neurology.

In this issue, we feature two paired articles:

Hellmann, et al. reviewed the response to maintenance intravenous immununoglobulin (IVIg) in a cohort of 52 patients with myasthenia gravis (MG) who had not responded adequately to pyridostigmine, prednisone, azathioprine, or combinations of these medications. Fifteen of the patients did not respond to an initial trial of IVIg, and were not treated with additional doses of IVIg. Thirty-seven patients responded to the initial trial and were treated with maintenance IVIg (0.4 g/kg every three to six weeks) for an average of 5.9 years (range 1 to 17 years). Twenty-three patients achieved mild improvement, and 14 patients achieved moderate improvement as measured by the Myasthenia Gravis Foundation of America (MGFA) clinical classification scheme. A beneficial response was associated with bulbar onset, seropositivity and high titer of acetylcholine receptor antibody, and older age of disease onset. Probably the most important observation in this study was that none of the patients achieved full remission with maintenance IVIg. Although IVIg improved symptoms of MG and allowed reduction of other medications, it did not provide a sustained effect or true remission of the disease. (Hellmann MA, Mosberg-Galili R, Lotan I, Steiner I. Maintenance IVIg therapy in myasthenia gravis does not affect disease activity. J Neurol Sci 2014;338:39-42)
Marinos Dalakas provides a thoughtful editorial on the above-noted article and places its results into the perspective of current treatment options for myasthenia gravis (MG). He emphasizes the point that IVIg appears to exert only a short-term and transient immunomodulation in MG and does not provide immunosuppression of the disease. At this time, the evidence supports restricting the use of IVIg in MG to short-term treatment of unstable patients until immunosuppressive treatments become effective. However, only a prospective trial of IVIg therapy for MG will provide definitive conclusions regarding its long-term effectiveness in managing this disease. (Dalakas M. IVIg in the chronic management of myasthenia gravis: Is it enough for your money? J Neurol Sci 2014;338:1-2)

England is editor-in-chief of the Journal of the Neurological Sciences.

What Have You Learned at the AAN Annual Meeting That You Will Take Back to Your Practice?

“I attended the Global Health Challenges: Neurology in Developing Countries. It was eye-opening to learn about the global burden of disease and specifically the difficulties in treating epilepsy in developing countries.

It won’t affect my current practice but reminds me of the need to think outside of the small world in which I practice. It also reignited an interest in participating in short-term medical mission work like I was able to do in medical school.”

David B. Watson, MD
Morgantown, West Virginia

“I live in Zambia and there are only two neurologists in Zambia for 13 million people. It’s a very small community! Coming here, I get to meet the people face-to-face that I’ve emailed, spoken to and trained with over the years. We are talking about collaboration and building our global health programs. This AAN meeting — it’s neurology on steroids!”

Omar Siddiqi, MD
Lusaka, Zambia

“I like that the issue of ethics was addressed in the Presidential Plenary Session and its impact to medicine. We are all living with ethics in our daily lives.”

Barbara A. Dworetzky, MD
Boston

“I attended a very educational series of debates on the controversies pertaining to ICU EEG monitoring of critically ill patients. It was great to hear from experts in the field arguing their viewpoints on an important unanswered question in neurocritical care. This has inspired me to review the literature myself and come to my own conclusion. Clearly, there is more research that needs to be done.”

Krishnan Vaishnav
Boston, MA

“The clinical sessions, especially those on the new agents for multiple sclerosis, are very good. MS treatment has been closed, very routine and patients are getting tired of the same injections.

It’s good to see that clinical research is progressing in this area. Hopefully in a few years — not 10 years — there will be new drugs available for MS patients. Patients are being included earlier in studies and being treated better and earlier.”

Douglas Sato, MD
Sendai-Miyagi, Japan

“I really appreciate the speakers who gave a high level interpretation of the clinical trials. We all know the data. It’s the interpretation that really helps us start a discussion and collaborate nationally.”

Ishida Koto, MD
New York

“The ICU monitoring session was very helpful. I work at the V.A. We are short-staffed, and we are short on staff qualified to perform multiple tasks such as ICU monitoring. The ideas I learned about ICU monitoring is directly applicable to my facility.”

Gabriel Bucurescu, MD
Philadelphia

“For me, it’s learning the research ideas for the future. I’m a junior resident so I’m enjoying all the sessions, especially the genetic talks.”

Janice Wong, MD
Boston, MA

“What I’m hearing is confirmatory: In stroke, there often is no clear decision. So, it’s confirmation to what’s been discussed here.”

Markus Naumann, MD
Augsburg, Germany

Book Review: Neuroanatomy of Language Regions of the Human Brain

Academic Press, 2014; 186 pages

The human brain contains billions of neurons, and these neurons interact in a variety of ways that are only beginning to be understood. One of the great challenges that humans confront is determining the way in which the human brain can support complex behaviors such as reading and understanding this text. We can begin to confront this challenge by improving our understanding of human neuroanatomy.

Michael Petrides is an internationally renowned neuroanatomist. His new, large format book is titled, “Neuroanatomy of Language Regions of the Human Brain.” The book is generously illustrated in color, including unique illustrations from his own work. The illustrations are clearly labeled. The accompanying text is authoritative and describes the relevant anatomical features in clear language.

The book is divided into three major sections. The first section of the book focuses on gross anatomy of the human brain. There is a comprehensive discussion of the gross morphological features of the brain. This is accompanied by images that can be obtained with MRI. Petrides illustrates gross anatomy in axial, sagittal and coronal orientations with a T1 sequence obtained at 3 tesla with 1 mm isotropic voxels, but few additional details of the imaging sequence are provided. These illustrations are useful since most volumetric imaging is obtained at 3 tesla with 1 mm3 voxels, although a 7 tesla scanner may have illustrated the anatomy with additional detail. Slices are provided at approximately every 4 mm. Each slice is associated with an orienting location in the space of the Montreal Neurological Institute ICBM152 generation VI average brain, and most sulci are labeled on each slice of each image.

The second section of the book focuses on cytoarchitecture. Large format images are provided that illustrate the layers of the cortex from each of the critical areas of the brain. Brodmann labeling is used for most illustrations, although Economo and Koskinas labels are used for some critical areas. The location of most samples is illustrated with images of gross location on a brain illustrating Brodmann’s areas, and some corresponding anatomic loci in the macaque monkey brain are also provided.

The third section of the book describes the named, long white matter projections of the human brain. Corresponding projections in the macaque monkey brain are illustrated as well. White matter connectivity is illustrated primarily within the left hemisphere, and hemispheric differences are not detailed. In addition to the averaged results of white matter projections, in situ tractography in individual subjects is provided to demonstrate each of the major white matter fasciculi. The text provides an important discussion of the distinction between the superior longitudinal fasciculus and the arcuate fasciculus, and considers in detail the dual-route hypothesis the postulates dorsal and ventral projections between anterior language regions in the frontal lobe and posterior temporal language regions.

Petrides provides an interesting historical discussion of the development of our understanding of the neuroanatomy of language, beginning with Pierre Paul Broca’s revolutionary presentation of Leborgne and Lelong. He also provides interesting illustrations of MRIs of the brains of these two patients. Major figures such as Wernicke, Marie and Dejerine are mentioned as well, although other seminal early investigators such as Lichtheim are not acknowledged. Petrides also reviews the seminal electrical stimulation studies of Penfield. Indeed, interesting historical perspectives are provided throughout the book.

It is appropriate that a volume about neuroanatomy focuses on language since this topic is the historical origin of our study of human cerebral anatomy. Petrides notes at the beginning of the book that most areas of the brain are arguably related to language. The focus of the book is tilted toward the language regions of the brain, but Petrides generously describes essentially all cerebral regions and provides key illustrations from macaque monkey.

A truly positive feature of this book is the multiple perspectives that are adopted in a single, comprehensive volume. An additional perspective might have been provided by the anatomically based neurotransmitter work from Zilles and Amunts.

This book was reviewed by Murray Grossman, professor of neurology, director of the Fronto-TemporalDementiaCenter at the University of Pennsylvania School of Medicine.

Stroke in Literary Works Around the World

By Axel Karenberg

Axel Karenberg

There are few other neurological disorders with such a constant presence in literary works as apoplexy. As early as 1600, the notions “apoplexy” and “apoplejàa” appear in dramas written by Shakespeare and Lope de Vega. More detailed descriptions of the disease enrich popular novels of the 19th century. Authors such as Balzac, Dumas, Flaubert and Zola must be mentioned here as well as the epical sagas of Dostojewski and Tolstoi ^1,2. The American author John Steinbeck used a “stroke” in his work East of Eden as did Philip Roth in his morbid narratives. In the German-speaking world, stroke is dealt with in more than 100 fictional works.

Naturally, literary productions treating this subject resort to contemporary medical knowledge³. Until the middle of the 20th century, literature focused mainly on two aspects of apoplexy: its typical symptoms and explanations of its origin. The presentation of symptoms abide by a strict code: sudden onset, motor deficiency and unfavorable outcome. The majority of authors was deeply impressed by the loss of the ability to produce speech — above all after Goethe’s early depiction of motor aphasia in Wilhelm Meister’s Apprenticeship (VII,6; 1795/96): “Altogether unexpectedly my father had a shock of palsy; it lamed his right side, and deprived him of the proper use of speech. We had to guess at everything that he required; for he never could pronounce the word that he intended … His impatience mounted to the highest pitch: his situation touched me to the inmost heart.”

Johann Wolfgang von Goethe (1749-1832), author of “Wilhelm Meister’s Apprenticeship.”

The diverse causes of the disease taken up in the “belles lettres” reflect its multifactorial origin conceived in premodern medicine. Melancholic “gloominess” or “thick blood” was grounded on the ancient idea of an abnormal blending of the bodily humors. In this perspective, the disease could be provoked by the “cold and moist evening air” or by “taking a bath at 9° C.” (e.g. in E.T.A. Hoffmann, 1825 and in Theodor Fontane’s Effi Briest, 1894/95). Only approaching present times, the thematic focus and the narrative perspective shifted slowly but constantly. Along with a more detailed description of the symptoms, the story is now set in hospitals and rehab centers and supplemented by technical diagnosis and therapeutic options. To exemplify this shift, there are George Simenon with his Non-Maigret book The Bells of Bicetre (1962) and Kathrin Schmidt’s novel You Won’t Die (2009).

In literary works, apoplexy serves different goals. The sudden outbreak of the disease can set the plot going, let take it the defining turn or end a story line. Above all, in recent prose texts, the symptoms bring the fictional patients to get to the bottom of their former life. But the illness also can assume a metaphoric function. The recurrent cerebral insults of the protagonist Oblomow in Iwan A. Gontscharows’ homonymous epic (1859) were meant to illustrate the agonizing czaristic feudal system. In a similar way, the American author John Griesemer in his New York novel Heart Attack (2009) fixed the acute symptoms of his protagonist on 9-11 in order to link individual and collective fate, facts and fiction to a meaningful picture.

Effi Briest by Theodor Fontane. Original cover of the first edition (1896).

Fictional texts are never bare clinical case histories, they never render exclusively neurological textbook knowledge. It is the components that lie beyond the medical horizon that arouse the interest of physicians and a broader public. The anthropological dimension, the look at human despair and hope can further the understanding of a patient’s fate and help to support present-day stroke medicine.

References

Perkin JD. The neurology of literature. In: Bogousslavsky J et al. (eds.): Neurological Disorders in Famous Artists, Part 3. Basel: Karger 2010, pp. 227-37.
van den Doel EM. Balzac’s serous apoplexies. Arch Neurol 1987; 44:1303-5.
von Engelhardt D. Neurologische Erkrankungen im Medium der Literatur. In: Kömpf D (ed.). 100 Jahre Deutsche Gesellschaft für Neurologie. Berlin: DGN 2007, pp. 346-53.

Karenberg is from the Institute for the History of Medicine and Medical Ethics, at the University of Cologne, Germany.

President’s Column: Neurology Cooperation Around the World

Since I wrote my last column, many events have occurred. The neurological world is moving so fast. The WFN remains at the forefront of developments of international activities and is leading in cooperation and promotion of neurology.

In January, the Sudanese Neurological Society held its ninth annual meeting in Khartoum. I had the privilege to be invited as well as the president of the Pan African Association of Neurological Sciences, Prof. Riadh Gouider, and the president of the Pan Arab Union of Neurological Societies Prof. Mohammad Tamawy. The attendance and interest was large and intense. There was an impressive eagerness to learn among young neurology trainees in all topics, and the hands-on workshops with bedside patients were fully subscribed. It is heartening to see that relatively small societies in Africa can provide so much high-quality teaching and care. Congratulations to Prof. Ammar El Tahir and Prof. Osheik Seidi for their efforts.

The 15th Cairo Neurology Congress was held in February and again the topics and attendance were impressive. Prof. Wolfgang Grisold, WFN Secretary-Treasurer General attended the meeting. Tamawy and Prof. Osama Abdul Ghani are to be congratulated for an excellent effort. I am sure that across the world many national societies have had their annual congresses and this only enriches the field. The WFN will be delighted to be involved in any way and to help promote and advertise these congresses.

Figure 1. (From left to right) Jonas Yeung, president of the Hong Kong Neurological Society; Man Mohan Mehndiratta, president of AOAN; Wai-Sin Chan, deputy director, Health Bureau, Macau Special Administrative Region; Chin Ion Lei, director, Health Bureau, Macau Special Administrative Region; Patrick Li, president of the Hong Kong College of Physicians; Ping Wing NG, co-chair of AOCN 2014; Leonard Li, co-chair of AOCN 2014; Lawrence Wong, secretary of AOAN and chair, Scientific Committee, AOCN 2014.

From national to regional congresses. The Asian Oceanian Congress of Neurology (AOCN) 2014 was organized by the Hong Kong Neurological Society and held in Macau. The congress was attended by members from all over Asia. The Hong Kong Society in collaboration with the Chinese Neurological Society was instrumental in producing an excellent program. Prof. William Carroll, WFN first vice president, attended the congress, and Man Mohan Mehedirata, AOAN president, was also present; the organization was excellent. Profs. Wing-Ping Ng and Laurence Wong are again to be congratulated. (See Figure 1.)

It seems that wherever in the world neurologists meet, there is always a sense of camaraderie and togetherness. It is also clear that topics vary in their scientific slant and their emphasis on training; but the eagerness to learn among neurologists in training is the same across the world. Teaching courses with live and videotaped cases attract a huge interest and create lively discussions.

The WFN grants round is now open, and we hope to receive as many applications as possible. The Grants Committee will start work after the closing date, and decisions will be conveyed to the applicants immediately. The plan is that the WFN will partner with other organizations to increase the amounts of the grants.

The Vienna World Congress was not only a scientific success but also a great financial success for the WFN. I, on behalf of the WFN, am most indebted to the Austrian Society for its hard work; and to the EFNS which suspended its annual congress for 2013 to allow just one major neurology congress to take place in Europe. The financial returns to all indeed exceeded expectations, which bodes well for the financial survival and strength of the WFN.

As this issue is being published, the amalgamation of the EFNS/ENS in the joint meeting in Istanbul will have taken place. This will create a most solid association. The WFN looks forward to the birth of the EuropeanAcademy of Neurology (EAN) and the elections of its officers so that our relations and close collaboration will continue as before with its two predecessors. The WFN’s strength and ability to reach its goals can only be achieved with the help of strong regional associations willing to collaborate to further the cause of advancing neurology globally. If one reads the EAN’s Purpose and Values, these goals are well laid out in Article 4 of its bylaws.

The WFN was born on the July 22, 1957, and during the Vienna World Congress, the Council of Delegates voted to commemorate that day every year as the World Brain Day. The task was given to Prof. Mohammad Wasay, chair of the Public Awareness and Advocacy Committee. The details are in the April issue of World Neurology. By the time this is published, all delegates should have received further correspondence.

The WFN history is rich and diverse. Prof. Johan Aarli, WFN past president, is the author of The WFN History: The First 50 years, published by Oxford University Press. By the time this issue of World Neurology is distributed, the book will be launched during the joint EFNS/ENS meeting in Istanbul. The book is essential reading for all.

The WFN and other peer organizations have created the World Brain Alliance. See http://www.wfneurology.org. This was started during the previous presidency, and I had the honor of being present during its inception in 2010. The presidents of peer societies last met in Vienna and will meet again to formulate a structure and proceed as the force speaking for all those involved in brain health.

Other activities to report are the collaboration with the WHO. This has matured and is progressing well. The Department of Mental Health and Substance Abuse is where neurology lies in the WHO structure. Shekhar Saxena and Tarun Dua are major contributors to the success of the collaboration. Moreover, Oleg Chestnov, WHO’s assistant director general, has agreed to talk to the attendees of the World Congress of Neurology in 2015 in Santiago, Chile. The WFN is a major funder to our WHO activities and will continue to be so. The ICD11 process is being finalized, and the process is on target. The WFN and the WHO are again collaborating in the production of the successful Neurology Atlas, second edition, as the first edition is now 10 years old. This process involves gathering information from Ministries of Health and all WFN member societies so that the data are verified and are useful tools for all.

The involvement of the WFN with the Non Communicable Diseases (NCDs) declaration is vital for the future of neurology. There is now a clear perception that the WHO is moving from the preventative mode, which has fully dominated its activities, to the area of disease management and appreciation of the huge burden of neurological diseases in the world. This, when it evolves further, is a seismic shift in thinking, and the WFN should be ready when it happens. The close collaboration and financing of many projects through the WHO is crucial for neurology, and the WFN should be at the top table in the decision-making process.

Many tasks lie ahead for the WFN trustees. For examples, finding and hiring a PCO when the contract with the current PCO Kenes expires with the last contracted congress in WCN 2015 in Santiago; and finding a publisher for JNS when the contract with Elsevier expires at the end of 2014. These are important decisions, and the trustees will have to look at all of the options and come up with the most suitable ones for the WFN.

As delegates were informed, the Nominating Committee is soliciting nominations for the post of elected trustee. Prof. Gustavo Roman will finish his second term and is not eligible for re-election. His contributions to the WFN as a trustee and as chair of the Latin America Initiative are immense. I, on behalf of all trustees, committees and member societies, would like to thank him for a wonderful job, which was done with grace, elegance and professionalism.

The next Council of Delegates meeting will be held in September in Boston, Massachusetts. This will be during the joint meeting of the American and European MS societies, and we look forward to seeing as many society representatives there as possible.

Raad Shakir
President