Articles – Page 65

FROM THE EDITOR-IN-CHIEF
Implementation Science Arrives: A New Dimension for Advocacy

By Donald H. Silberberg

Donald H. Silberberg

The neurologic community should congratulate itself for achieving public recognition for our field by organizing public programs. This advocacy method began with the U.S. declaration of the Decade of the Brain in the 1990s. Many other countries followed with their declarations of a year or a decade for the same purpose. In 2013, the World Federation of Neurology designated July 22, the date of its founding in 1957, as World Brain Day. Neurological societies in several countries organized celebrations on that date in 2014, as reported in past issues of World Neurology. The purposes for these celebrations include increasing public awareness of neurological disorders and persuading governments to increase the resources needed to make care available, improve care and carry out essential research.

The huge question is how to move from advocacy to improving prevention and effective clinical care in all countries. As I described in the June 2014 issue of World Neurology, the Fogarty International Center at the National Institutes of Health (U.S.) is trying to help with this by developing a program to promote implementation science. This refers to the study of methods to promote the integration of research findings and evidence into health care policy and practice. The ways to achieve progress include calling attention to the impact of existing data (e.g., global burden of disease studies), designing new research studies that will be understandable and appeal to policymakers and exploring how the conversation with a policymaker or funding agency should be framed.

The Fogarty Center’s approach to implementation science is to learn what has worked, develop communication among neurologists and neuroscientists wherever there is interest and ultimately, perhaps, offer research funding designated for implementation science. This important initiative, as applied to our field, will be among the topics discussed in “Public Policy and Health Economics” at the 2015 World Congress of Neurology in Santiago, Chile, Oct. 31-Nov. 5. Please use this opportunity to share your thoughts about how to move from information to action.

Dementia: A New Perspective

By Vladimir Hachinski, MD

Vladimir Hachinski

As dementia rises in prevalence, new approaches must be adopted in the treatment of the condition and efforts to prevent it.

Dementia means the loss of brain capacity severe enough to result in the loss of self-sufficiency. The incidence of dementia, which is rising globally, is largely driven by the aging population. Although dementia increases with age, it is not inevitable with age. Dementia represents the end stage of several processes, for which some are treatable and preventable.

Brain blood vessels (vascular) and Alzheimer’s disease represent the two most common pathologies leading to dementia. The changes of Alzheimer’s disease are characterized by the deposition of amyloid protein plaques and of tau protein aggregation forming tangles in neurons. The changes that lead to Alzheimer’s disease begin about 20 years before any symptoms appear. Many elderly individuals die with plaques and tangles without having had any trouble in life as a result. Similarly, most vascular disease is insidious. For each stroke that affects the body, five affect the mind, usually with the person being unaware of them.

While Alzheimer’s and cerebrovascular pathology occur commonly with age, mostly without symptoms, the combination doubles the chances that the dormant pathologies will result in dementia. Although cerebrovascular disease is treatable and preventable, scant attention has been paid to this component, present in 80 percent of Alzheimer’s patients.

Instead, the declared intention is to find a cure or disease-modifying drug by 2025. The idea of giving one drug to an amalgam of pathologies broadly defined as Alzheimer’s disease may prove as disappointing as the litany of failed trials that took place in the late 1990s and early 2000s aimed at stopping the damage that follows a stroke with a single drug. Since dementia has multiple causes, one must try multiple therapies, including addressing the one component that can be treated and prevented: the vascular one.

Dementia is not a threshold but a continuum. The process begins decades before any symptoms appear, a phase termed the “brain-at-risk stage.” The earlier the risk factors are recognized and treated, the better the chance of success.

Knowledge accrues in pieces, but is understood in patterns. Specialization fosters fragmentation and fiefdoms. It turns out that all major brain diseases result from different combinations of half a dozen mechanisms. By integrating this knowledge, researchers may discover that drugs developed for one purpose in one field may have application in another. If we only knew what we already know.

The Need for Multiple Therapies

The diagnosis of Alzheimer’s disease is notoriously imprecise, mainly because most patients harbor multiple pathologies. Even if a drug were 100 percent effective in blocking amyloid deposition, its effect might be obscured or overwhelmed by concomitant pathologies, for example brain vascular disease and its interactions, such as inflammation, if not treated at the same time. This calls for multiple therapies and new methodologies, such as platform trials to evaluate multiple therapies simultaneously. The lack of precision in diagnostic categories can be overcome by identifying specific contributing mechanisms leading to dementia and treating them. It is now possible to image vascular disease, amyloid and tau protein deposition and inflammation in the brain. Each of these mechanisms can be treated individually or in combination.

The evaluation of drugs can be accelerated by developing protocols in close reciprocal interactions with experimental work in a few advanced centers. These would continue with extensive protocols and thorough evaluation of patients. Once experience has been gained, a protocol could be simplified so that large numbers of patients could be enrolled. At predetermined intervals, statistically valid samples of patients following the simplified protocol would be studied by those following the extensive study protocol to make sure that they were similar.

In the era of big data and electronic records, it may be possible to do more sophisticated post-marketing surveillance and gain real-world knowledge of the effectiveness of different treatments.

Unhealthy diets, physical inactivity and tobacco and alcohol addiction represent identifiable risks for stroke and dementia and other non-communicable diseases targeted by the United Nations resolution of September 2011. In order to succeed, a three-step approach is required:

Information
Motivation
Enablement

Good information is essential, but by itself is no more effective than New Year’s resolutions. Motivation matters but is seldom addressed. Healthy lifestyles require a healthy environment, and policymakers have a particular role in creating it. They also have a leadership role in introducing legislation to curb tobacco and alcohol use and limit the consumption of unhealthy foods. They also have a major role in ensuring that our air is breathable. Air pollution can harm the lungs, damage the heart and afflict the brain. What happens in Beijing matters at Schloss Elmau: We share the same biosphere. Policymakers can follow the lead of Finland in considering health in all policies. Public health could be enhanced considerably through the leadership of non-governmental organizations. Additionally, policymakers can get help from international brain organizations, which can provide expertise and patient support groups, and can help to mobilize the public toward healthier lifestyles and risk-factor control, which may prevent or postpone major chronic diseases, including dementia.

Conclusions

Dementia results most often from a combination of Alzheimer’s and cerebrovascular pathologies and their interaction. Cerebrovascular disease is both treatable and preventable.

The diagnosis of dementia is imprecise, but it is now possible to identify and target the different mechanisms leading to brain deterioration. This will require multiple interventions and new clinical trial methodologies.

Dealing with the challenges of dementia will require not only new resources, but new thinking and different approaches as well.

Vladimir Hachinski, MD, is the Distinguished University Professor at Canada’s University of Western Ontario. With John W. Norris, he founded the world’s first successful acute stroke unit. With David Cechetto, he discovered the role of the brain’s insula in sudden death, and, joined by Shawn Whitehead, they established a treatable link between Alzheimer’s disease and stroke. He has authored, co-authored or co-edited 17 books and more than 600 widely cited publications. He was president of the World Federation of Neurology from 2010 to 2013 and the founding and past chair of the World Brain Alliance.

Article originally published in G7 Germany: The Schloss Elmau Summit. www.g7g20.com.

AFAN Brings New Era in African Neurology

By Raad Shakir

Raad Shakir

It was a momentous day in the history of African neurology. For more than 40 years, the continental neuroscience organization was an amalgamation of neurologists and neurosurgeons. The Pan African Association of Neurological Sciences (PAANS) served its purpose, and it’s time now for the establishment of an organization dedicated to neurology. Neurosurgeons already have established the Continental Association of African Neurosurgical Societies and held an inaugural meeting in Algiers.

The World Federation of Neurology (WFN) fully supported the views of neurologists across Africa to establish this association. This followed many years of discussions and consideration, since the establishment of the Task Force for Africa in 2006 during the presidency of Johan Aarli and the subsequent stakeholders’ meeting in Stellenbosch, South Africa, in 2008.

Representatives from 27 countries participate in the inaugural meeting of the African Academy of Neurology in Dakar, Senegal, in August.

The WFN designated a third of its profits from the Marrakesh World Congress to the Africa initiative. Part of this fund was released to gather delegates from as many African neurological societies as possible to hold an inaugural extraordinary meeting in Dakar, Senegal, in August. This was arranged by Prof. Gallo Diop, WFN trustee and chair of the WFN Africa initiative, and Prof. Riadh Gouider, WFN trustee and president of PAANS.

I had the honor of attending and participating in the proceedings. Representatives from 27 African societies were present: Benin, Burkina Faso, Cameroon, Congo Brazzaville, Congo DRC, Egypt, Ethiopia, Gabon, Ghana, Guinea, Ivory Coast, Kenya, Madagascar, Mali, Mauritania, Morocco, Niger, Nigeria, Rwanda, Senegal, South Africa, Sudan, Tanzania, Togo, Tunisia, Uganda and Zambia.

The bylaws and constitution, which were drafted by Prof. Mostafa Elaloui, Morocco, were circulated in advance. After careful review, the draft unanimously was approved.

The following day and according to the bylaws, elections were held for the board of directors. Prof. Michel Dumas, Limoges France, and I supervised the proceedings. It is of note that Prof. Dumas is among the PAANS founders, and he was present to witness the founding of the African Academy of Neurology (AFAN).

The AFAN elected board of directors are:

President; Mansour Ndiaye, Senegal
President Elect: Youmi Ogun, Nigeria
Secretary General: Augustina Charway, Ghana
Treasurer: Lawrence Tucker, South Africa
Five Regional Vice Presidents: Central Africa, Alfred Njamnshi, Cameroon; East Africa, Osheik Seidi, Sudan; North Africa, Foad Abd-Allah, Egypt; South Africa: Alain Tehindrazanarivelo, Madagascar; and West Africa: Agnon Balogou, Togo

The AFAN council of delegates approved the establishment of a permanent secretariat in South Africa, with all the necessary legal implications and registration as a non-profit organization. Lawrence Tucker, AFAN treasurer, will undertake the task.

With the formation of AFAN, the sixth chain of the WFN regional organizations is now complete. I am sure that all of us wish our African colleagues all the best in their tireless work to promote and deliver neurological care in Africa.

A Continuum Course in Vietnam

By Nguyen Huu Cong

Nguyen Huu Cong

Through the aid of the World Federation of Neurology (WFN) and the American Academy of Neurology (AAN), the continuum courses have been carried out for many years in Vietnam. The courses are conducted one to two times a year, with the topics chosen by members of the executive committee of the Ho Chi Minh City Neurological Association, based on the issues delivered annually by the WFN and AAN. These courses are usually organized in Ho Chi Minh City, the largest city in Southern Vietnam. On May 6, the course took place for the first time outside Ho Chi Minh City in the Mekong Delta.

The Tien Giang Neurological Association (TNA) was founded one year ago in My Tho, a beautiful small town in the Mekong Delta and the capital of Tien Giang Province. It now has 60 members. Most of them are neurologists from the surrounding provinces in the Mekong Delta, but some are internists working in rural areas without neurologists. Dr Nguyen Van Thanh, chief of the department of neurology at Tien Giang Hospital and president of the Tien Giang Neurological Association, is actively working and running the association.

The Vietnamese Association of Neurology and the Ho Chi Minh City Neurological Association have been assisting the TNA by sending our experts to attend lectures there. This year’s continuum course, held at Tien Giang General Hospital, covered the topics of epilepsy, as presented in Continuum: Lifelong Learning in Neurology, Vol. 19, Issue 3, June 2013. The lecturers were Prof. Pierre Jallon, former professor of neurology at the University of Geneva; Le Van Tuan, MD, PhD; and Tran Quang Tuyen MD, from the Ho Chi Minh City Neurological Association. The lectures focused on these articles:

The 2010 Revised Classification of Seizures and Epilepsy
Antiepileptic Drug Treatment: New Drugs and New Strategies
EEG and Epilepsy Monitoring

Prof. Pierre Jallon presented the old and new definitions, compared previous to revised classifications and analyzed the advantages of new terms and concepts and their limitations. Then the professor also talked about the differential diagnosis, especially in difficult cases from the features of syncope convulsions, hypoglycemia and psychogenic nonepileptic seizure. Subsequently, Dr. Le Van Tuan introduced the article, “Antiepileptic Drug Treatment: New Drugs and New Strategies,” speaking about the targets of epilepsy treatment, classical AEDs and newer medications available in our country. He also showed how to select antiepileptic drugs appropriate for each types of seizure. The topic of AED treatment fascinated all of the attendees, and they started a fervent conversation with the lecturer. Dr. Tran Quang Tuyen introduced the usefulness of video EEG in supposing epilepsy diagnosis. On this occasion, Drs. Le Van Tuan Tran Quang Tuyén, took turns to report on the situation of using EEG in Vietnam for epilepsy monitoring. Once again, many questions related to clinical practice were asked of the lecturers. After the presentations, attendees were encouraged to discuss the contents of the articles, “Patient Management Problem” and “Patient Management Problem — Preferred Responses.”

The discussion lasted past the fixed hours. At the end of the course, Dr. Nguyen Van Thanh, president of the TNA, expressed the gratitude of the Tien Giang Association members to the lecturers, AAN and WFN. The participants showed their gratitude in kind and suggested similar educational courses in the Mekong Delta in the future.

The CME course with Continuum — Lifelong Learning in Neurology has contributed to improving the knowledge of epilepsy in our neurologists in Tien Giang and the surrounding provinces.

The issues of the Continuum: Lifelong Learning in Neurology, with articles written by experts from the American Academy of Neurology, are useful for our neurologists, especially young members of our associations in Vietnam. We are planning to organize one additional course in August 2015 on “Peripheral Nervous System Disorders.” We believe that afterward we will continue to have the assistance of the World Federation of Neurology.

Nguyen Huu Cong is an associate professor; deputy chairman of the neurological department at Pham Ngoc Thach University of Medicine, lecturer of the neurological department of Ho Chi Minh City University of Medicine and Pharmacology; president of the Vietnam Association of Electro Diagnostic and Neuromuscular Medicine; and vice president of the Neurological Association of Ho Chi Minh City.

Longtime INPC Continues in Croatia

By Vida Demarin, MD, PhD, FAAN, FAHA, FESO

55th INPC Opening Ceremony.

The 55th International Neuropsychiatric Congress (INPC) May 27-30 in Pula, Croatia, was held under the auspices of the president of the Republic of Croatia, her excellency Kolinda Grabar Kitarovic.

The organizer of the congress is the Society for Neuropsychiatry, and the co-organizers are the department of medical sciences of the Croatian Academy of Sciences and Arts and the Central and Eastern European Stroke Society.

The Congress was endorsed by the World Federation of Neurology (WFN), European Academy of Neurology, WFN Applied Research Group on the Organization and Delivery of Care, European Psychiatric Association and Croatian Stroke Society. The main sponsors of the congress were the Ministry of Science, Education and Sports of the Republic of Croatia, City of Graz, City of Pula and Istria County. There were more than 350 participants from Austria, Albania, Bosnia and Herzegovina, Montenegro, Kosovo, the Czech Republic, Croatia, China, Greece, Iran, Italy, Ireland, Hungary, Macedonia, Germany, Poland, Romania, Russia, South Korea, Slovenia, Serbia, Thailand, Ukraine, United Kingdom and the United States.

From left: Prof. Hrvoje Hecimovic; Prof. Vida Demarin, INPC president; and Prof. Raad Shakir, WFN president.

The congress kicked off with an academic lecture on “WFN: The Way Ahead,” given by our special guest, Prof. Raad Shakir, president of the WFN. The main theme was “Highlights in Neurology — What Have We Learned in the Last 55 Years” in stroke, post-stroke depression, multiple sclerosis, epilepsy, headache and pain and neurorehabilitation, presented by experts in the field, Professors Franz Fazekas, Kurt Niederkorn, Francesco Paladin, Wai Kwong Tang, Vesna Å eric and Vida Demarin.

Main topics in psychiatry were “Evolutionary Perspectives in Psychopathology” and “Controversies and News in Psychiatry,” organized by Prof. Karl Bechter and Francesco Benedetti. There were also numerous symposia, in particular: Challenging Child and Adolescent in Modern Society, Fourth European Summer School of Psychopathology, International Sports Psychiatry Meeting, Eighth International Symposium on Epilepsy, Fourth Symposium on the Interface Providers in Neurorehabilitation, Symposium on the Activities of the Association of Public Health Andrija Å tampar, and symposia about stress management and acute stroke treatment.

Joint meetings with Alps-Adria Neuroscience Section, WFN Applied Research Group on the Organization and Delivery of Care, and Central and Eastern European Stroke Society, chaired by Professors Leontino Battistin and Vida Demarin on the current status of stroke management in the region and on perspectives and new approaches in neurorehabilitation, also were organized as a part of the INPC. Prof. Anna Czlonkowska from Warsaw gave a special lecture on Wilson’s Disease, with original data from their registry.

During the congress, there were 66 lectures within 14 symposia, which were given by 55 lecturers from around the world, and a poster session with many interesting posters. Awards for best posters were given by the City of Graz and by INPC Kuratorium.

We are proud of this unique congress, being one with the longest traditions in the world. During the past 55 years, INPC has become a beloved place of meeting, a venue for continuing education in topics of neurology, psychiatry and related disciplines, and a point of scientific and professional exchange of experience for a large number of scientists and professionals from all over the world, continuing on the original idea of sciences and humanity. We hope to keep this success in the upcoming years.

Vida Demarin, MD, PhD, FAAN, FAHA, FESO, is president of the INPC.

Armauer Hansen: The Controversy Surrounding his Unethical Human-to-Human Leprosy Transmission Experiment

By Douglas J. Lanska, MD, MS, MSPH, FAAN

Douglas Lanska

In 1873, Norwegian physician Gerhard Armauer Hansen (1841-1912) [below]discovered rod-shaped bodies — Mycobacterium leprae — in leprous nodules. Initially unable to stain these bodies, he only tentatively suggested that they resembled bacteria, which led to a later priority dispute with Albert Neisser (1855-1916) when Neisser was able to stain the organisms and then claimed priority for the discovery. Although Hansen was convinced that leprosy was an infectious disorder, he was unable to cultivate the organism and unable to transmit the disease to animals, despite 12 failed attempts to transmit the disease to rabbits by inoculation.

In 1875, Hansen had been appointed as medical officer of health for leprosy in Norway and as the resident physician at the Bergen Leprosy Hospital. After corresponding with German physician and pioneering microbiologist Robert Koch (1843-1910) in Breslau, Hansen decided to attempt human-to-human inoculations, and specifically to inoculate leprous tissue from a patient with lepromatous (multibacillary) leprosy into patients with tuberculoid (paucibacillary) leprosy [below right] to determine whether he could produce manifestations of lepromatous leprosy.

Robert Koch. Public domain. Courtesy of the U.S. National Library of Medicine.

While Hansen had already achieved some professional renown for his studies of leprosy, his patients found him aloof and high-handed. On Nov. 3, 1879, while on rounds at the Bergen Leprosy Hospital, Hansen instructed a 33-year-old patient with the “anesthetic type of leprosy,” Kari Nielsdatter Spidsøen, to accompany him to his office as he indicated he wanted to speak to her. There, she saw that he had a sharp-cutting instrument in his hand which he brought up to her eye, while she held him off with her arms. After she was calmed down by one of the other doctors in the room, Hansen succeeded in his goal of inoculating leprous material from another patient under the conjunctiva of her eye with a cataract knife.

Gerhard Armauer Hansen. (Public domain. Courtesy of the U.S. National Library of Medicine)

The patient reported this to the hospital pastor, Pastor Grönvold, who in turn forwarded the complaints to legal authorities who charged him with causing bodily harm to an innocent patient. According to the transcript of the court proceedings, Hansen “admitted that he was not justified in carrying out the operation as he had neither obtained her permission in advance, nor told her of his aim in doing it. He had omitted seeking informed consent for the procedure “as he took for granted that the [patient] would not regard the experiment from his point of view, and if something happened [e.g., a lepromatous lesion developed that might threaten her vision], he was sure he could get the affection under control.”

Despite the criminal complaint against him, Hansen boldly expressed to the court his self-righteous belief that he was justified in these actions: … even if the subject should have some pain, because he had chosen a subject who had suffered from leprosy for many years, and therefore would not be exposed to a new disease. He was quite sure that there was no risk of loss of vision, even if the inoculation should have resulted in a nodule. He himself had several times extirpated nodules from eyes without any trouble, and had succeeded in saving the eyesight. … The great scientific and national importance of finding the answer to the question [of the transmissibility of leprosy] had therefore forced him to act as he did.

Maculo-anesthetic (tuberculoid or paucibacillary) leprosy (left) and lepromatous (multibacillary) leprosy (right). Tuberculoid leprosy is characterized by hypopigmented skin macules and anaesthetic patches from damaged peripheral nerves, while lepromatous leprosy is characterized by symmetric skin lesions, nodules, plaques and thickened dermis with detectable nerve damage typically late in the illness. (From Walker, 1905)

Although Hansen’s colleagues supported him with various post hoc justifications, it was clear to the court (with Hansen’s own admission) that, in his zealousness to prove the infectious nature of leprosy, he had misused his position of authority by trying to intentionally transmit a disease to a patient placed in his care without the patient’s consent.

Hansen was convicted and in consequence lost his post at the Leprosy Hospital in Bergen, but in a legal-political compromise he retained his position as chief medical officer for leprosy in Norway. The case had little effect, though, on Hansen’s professional reputation, and he continued with his scientific studies. Nevertheless, as Norwegian microbiologist and historian Thomas M. Vogelsang (1896-1977) concluded, the legal decision emphasized “that even a celebrated scientist is bound to obey the law of the land, and that it is the court’s duty to protect every citizen also against encroachments from more influential persons.”

Douglas J. Lanska, MD, MS, MSPH, FAAN, is with the Veterans Affairs Medical Center, Great Lakes Veterans Affairs Healthcare System, Tomah, Wisconsin.

Peter J. Koehler is the editor of this history column. He is neurologist at Atrium Medical Centre, Heerlen, the Netherlands. Visit his website at www.neurohistory.nl.

References
Blom K. Armauer Hansen and human leprosy transmission: Medical ethics and legal rights. Int J Lepr 1973;41:199-207.
Lock S. Research ethics – a brief historical review to 1965. J Intern Med 1995;238:513-520.
Marmor MF. The ophthalmic trials of G.H.A. Hansen. Survey Ophthalmol 2002;47:275-287.
Vogelsang TM. Gerhard Henrik Armauer Hansen: 1841-1912: The discoverer of the leprosy bacillus. His life and his work. Int J Lepr 1978;46:257-332.
Walker NP. An Introduction to Dermatology. Third edition. Philadelphia: William Wood and Co.

Report of the Cairo Department Visit

By Steven Lewis and Wolfgang Grisold

Prof. Mohamed El Tamawy of the department of neurology at Cairo University.

The department of neurology of Cairo University will be a World Federation of Neurology (WFN) Teaching Center for neurology training beginning in 2015. On March 20, the dean and departmental leadership and faculty welcomed an educational visiting committee of Steven Lewis, chair of the Education Committee; Wolfgang Grisold, co-chair and secretary general; and Riadh Goudier, trustee and president of the Panafrican Society.

As part of the application to become a WFN Teaching Center, and prior to the site visit, the department had been asked to write structured reports on their clinical teaching program, including detailed written surveys from faculty members and trainees.

On the day of the visit, the faculties of the clinical and research departments were introduced, followed by personal interviews by the visiting committee with trainees and teachers. These interviews focused on work conditions and cooperation within the department and the hospital. A formal round through the wards followed these interviews. Patient wards, investigational laboratories and outpatient facilities were explained and inspected by the WFN committee. In a final conference, an exchange of opinions, discussion of structure and academic discussions took place. Subsequently, the trustees of the WFN received a report from the site visit team recommending the establishment of a WFN Teaching Center in Cairo.

WFN Teaching Centers are being established to improve training of neurologists in Africa, and Cairo will join the first Teaching Center in Rabat, Morocco, which has been running a WFN teaching program since 2014. The WFN is convinced that supporting high-quality Teaching Centers in Africa will improve the quality of training neurologists and lead to additional high-quality institutions of training in neurology in Africa.

The WFN is grateful to the leadership and faculty of the department of neurology at Cairo University for its commitment to improving the training of neurologists within Africa, and I look forward to a long and successful collaboration in these efforts.

Report on the UEMS/EBN/EAN Examination in Berlin

By Wolfgang Grisold

Successful candidates and examiners.

The seventh European Board Examination in Neurology was held June 19 in Berlin, one day prior to the 1st Congress of the European Academy of Neurology (EAN) (http://www.uems-neuroboard.org/). There were observers from the World Federation of Neurology, including from Prof. Wolfgang Grisold.

The examination included a written part, with 80 multiple-choice questions (MCQ) and 50 extended matching questions (EMQ). These questions were developed by European specialist sections and were prepared by education standards in an extensive review process.

As a new feature, the candidates had to prepare an essay on a neurology-related public health or ethics related topic. This topic was presented by each candidate and orally discussed with the examiners. In addition, a critical appraisal of a neurological topic (CAT) had to be prepared and also was orally discussed with the examiners.

Topics of the essays were diverse and covered disease-based topics, such as driving with epilepsy or work- and health system-related topics. The CATs were mainly directed at diseases and therapies, and the presenters were expected to provide a qualified and thorough review on the topic chosen.

The examination was well organized and started with the MCQ examination. The rest of the day was spent with parallel sessions of EMQ testing and oral presentations. Two examiners who discussed the presentations and also asked questions in regard to the CAT took the oral presentations. Examiners were paired and selected with regard to language proficiency.

The results of the written examinations and the oral judgments were analyzed on the same day, and the results were presented on the same day.

Eighty-five candidates showed their interest, 72 sent submissions, 63 showed up, and 55 passed.

The following countries participated: Europe and Turkey (34): Austria, Belgium, Italy, Portugal, UK, Germany, Slowenia, Sweden, Greece and Turkey; Non-Europeans (29): Israel, Saudi Arabia, Egypt, Denmark, Pakistan, India, Iraq, Morocco, Tunisia, Cameroon and South Africa.

It was the seventh examination of the UEMS/EBN, now with the newly created EAN. This examination introduced the structured essay and CAT, which replaced the prior self-presentation. This new development added a new dimension to the examination, as the candidates were expected to give structured opinions on topics related to diseases and health as well as ethical topics.

At present, the European Board Examination is a sign of excellence, and it is hoped that increasingly European countries will accept the European Board Examination as equal to the national examination, or even replace their national examination with the European Board exam.

Editor’s Update and Selected Articles From the Journal of the Neurological Sciences

By John D. England, MD

John D. England

The editorial staffs of the Journal of the Neurological Sciences (JNS) and our publisher, Elsevier, have strived to enhance the quality of the journal. I am pleased to inform you that Elsevier soon will introduce the Journal Workflow Modernization Programme. This new workflow program, which Elsevier has named LeMans, will result in authors receiving proofs of their articles within 24 to 72 hours after acceptance. The LeMans program will significantly decrease the production time for all of our accepted articles. As an editor, I know that authors desire to see their articles published as quickly as possible, and this new feature will accelerate the publication process.

Additional steps for enhancing publication such, as the introduction of Article-Based Publishing (ABP) are planned. ABP, which should be introduced in late 2015, will post articles online as soon as they are finalized without waiting for an entire journal issue to be compiled.

All of these advancements reflect the ongoing transition from print to electronic publishing. JNS, like many Elsevier journals, is moving quickly toward a totally electronic publishing platform.

In our ongoing attempt to enhance accessibility of JNS articles for members of the World Federation of Neurology (WFN), we have selected two more free-access articles, which are profiled in this issue of World Neurology.

Stephanie Brown and Andrew Stanfield provide an excellent review of the fragile X tremor ataxia syndrome (FXTAS), which is an increasingly recognized movement disorder. Affected individuals carry the permutation allele of the FMR1 gene. This permutation is an expansion of the nontranslated 5′ CGG repeat region of FMR1 from the normal range, which is less than 45 repeats to between 55 and 200 repeats. Affected individuals are usually men over 50 years of age who have progressive symptoms of tremor, ataxia and cognitive decline. Other manifestations include Parkinsonism, peripheral neuropathy, autonomic dysfunction and endocrine changes. The authors detail the clinical, molecular and neuroimaging manifestations of the disease. They emphasize the neuroimaging characteristics of FXTAS, which include increased T2 signal intensity in the middle cerebellar peduncle (MCP sign), thinning of the corpus callosum and white matter atrophy.

Brown SSG, Stanfield AC. Fragile X premutation carriers: A systematic review of neuroimaging findings. J Neurol Sci 2015;352:19-28.

Alexander Slade and Sinisa Stanic reviewed the literature regarding the usefulness of salivary gland irradiation for managing sialorrhea in patients with amyotrophic lateral sclerosis (ALS). Many patients with ALS have difficulty controlling salivary secretions. Although oral anticholinergic medications or botulinum toxin injected into the salivary glands can be helpful, patient intolerance or unacceptable adverse effects may occur. This review concludes that the majority of ALS patients with sialorrhea respond well to salivary gland irradiation and experience minimal side effects. Thus, clinicians should consider this treatment option for patients with ALS who are troubled by excessive salivation.

Slade A, Stanic S. Managing excessive saliva with salivary gland irradiation in patients with amyotrophic lateral sclerosis. J Neurol Sci 2015;352:34-36.

John D. England, MD, is editor-in-chief of the Journal of the Neurological Sciences.

Worldwide Variations in Brain Death Declaration

By Torrey Boland, MD

Torrey Boland

Despite the publication of evidence-based practice parameters for the declaration of brain death by the American Academy of Neurology (AAN) in 1995 and updated in 2010, there remains much variation worldwide in how the actual determination of brain death is performed. Not only does the practice, but also the perceptions of brain death, vary widely across countries.

In 2002, an international review identified significant variations in the practice of determining brain death. However, studies such as this have reported primarily on higher-income regions.

Earlier this year, several researchers reported in Neurology the results of the largest study to date attempting to assess and characterize both the practices in determination of brain death worldwide as well as the perceptions of brain death among countries. This study aimed to gather data not only from high-income countries, but to gather a comprehensive worldwide dataset. It confirmed that significant variations continue to exist worldwide in both the perception and practice of declaration of brain death. This lack of agreement could become challenging as organ transplantation networks are becoming increasingly internationalized, and supports the need for a stronger international consensus on brain death.

The authors conducted an electronic survey, which was distributed to individuals who practiced medicine and had interactions with patients who could become brain dead. The target participants comprised physicians in 123 countries. The survey sought to query individuals with knowledge and expertise in brain death within each country. Included in the survey were members of the Neurocritical Care Society; country representatives of the World Federation of Neurology, who were asked to either complete the survey or forward it to appropriate colleagues, authors of publications on brain death; and international personal contacts of the authors. Responses were tallied from 91 countries, including the African, Eastern Mediterranean, European, Pan-American, Southeast Asian and Western Pacific World Health Organization regions.

The findings of the study showed that there remains significant variability in both the perception of brain death as a concept as well as dissimilarity in the practice of the declaration of brain death. Most countries noted a legal provision (70 percent) and an institutional protocol for the declaration of brain death (77 percent), but high-income countries were significantly more likely to have an institutional protocol than low-income countries.

In addition, the majority of countries in Africa lacked institutional brain death guidelines. Legal provisions for brain death were more likely in countries with organized transplant networks, even when adjusting for income. This may be related to advances in medical technology, which has led to an increase in organ transplantation in more middle- and low-income countries. This rise in transplantation indicates a corresponding need for understanding of the concept of brain death.

A small portion of countries (14 percent) reported a lack of brain death declarations in their hospitals, citing a lack of intensive care or advanced technology, lack of expertise in brain death, and uncertainties regarding the concept of brain death. This may represent a need for further
medical education in some areas of the world. Interestingly, 57 percent of respondents disagreed with the statement that brain death equates to cardiac death. This response did not appear to be associated with country income level, which may reflect variations among individuals related to personal or cultural beliefs. Nearly all physicians from countries with designated transplant networks agreed that brain death was an established concept at their hospitals.

Variations in the practice of declaring brain death were noted as well. While the majority (66 percent) of respondents reported that an attending physician trained in neurology, neurosurgery or intensive care must make the determination of brain death, 25 percent of those polled stated that a resident-level trainee could independently declare brain death. Discrepancies were noted in the clinical examination, with more than half of the countries citing an institutional protocol that was discordant with the 2010 American Academy of Neurology criteria. Apnea testing was another area in which much variability existed, with differences in blood gas requirements, number of tests required and equipment.

There did not seem to be an association between the use of ancillary testing and country income level. The authors hypothesized that this finding may be explained by the increased use of ancillary tests by physicians who are unfamiliar with brain death, as the AAN practice parameter does not require ancillary testing in straightforward cases.

While this study depended on the responses of individuals, there may be a bias in the nature of self-reported perceptions and practices. The results may not reflect each country as a whole, and there may be regional differences within each country that were not assessed. In addition, the survey was presented in English only, which may have led to some misinterpretation in the questions by responders. In the lowest income countries, the need for a policy surrounding the declaration of brain death may be irrelevant as there are more urgent public health issues. Overall, however, this study represented a large, diverse and worldwide sample.

Variations in brain death declaration both in practice and as a concept persists despite advances in communication and education worldwide. This study identifies the challenges in developing a worldwide consensus on the determination of brain death. It is unclear whether creating an international standard for brain death is a feasible goal. In addition to variations in medical education, cultural beliefs and legal codes worldwide, there are also individual differences in practice and beliefs, as evidenced by variations in practice within the United States, which may be related to individual biases and opinions. This study highlights the need for the international community to work together to create a more uniform approach to brain death, especially as the practice of organ donation continues to expand.

Torrey Boland, MD, is an assistant professor in the department of neurosciences at Rush University Medical Center, Chicago.

Reference
Wahlster S, Wijdicks EFM, Patel P, et al. Brain death declaration. Neurology 2015; 84:1870-1879.