VOL. 23 • NO. 2 • JULY 2008 World

THE OFFICIAL NEWSLETTER OF THE WORLD FEDERATION OF NEUROLOGY

Welcome to the New WORLD NEUROLOGY! INSIDE From Germany: tarting with this is- scientific advances. What view of a brief, little-known top- As the German sue, WORLD NEUROL- interests you may well in- ic of interest in clinical neurolo- Society for Neurology SOGY will have new terest others. gy. The first installment is on turns 100, a look content and a new look. As In this inaugural issue, the Ramsay Hunt syndromes. back on the history the newsletter of the World you will find some new fea- Can you describe all four with- of the specialty in Federation of Neurology tures. One of these is “Pro- out cheating? Deutschland. PAGE 8 (WFN), WORLD NEUROLO- files in Neurology.” In it, I We also will publish cases. GY is the only publication hope to spotlight different Some of these may be disorders going to all neurologists in medical care practices from that are seen only in certain parts the world. around the world, giving of the world or that are more The newsletter will con- one neurologist his or her common in particular places. From Brazil: tinue to feature news from chance at “fifteen minutes Others may be rare but important EWS A case series of N the WFN. However, the of fame,” as the expression to keep in mind. So send in your dengue fever shows new version will have other goes. I am grateful to Dr. suggestions! EDICAL why neurologists content of interest to neu- M Rawiphan Witoonpanich We expect that cases, as well as should be wary rologists, including some of Mahidol University, the other articles, will be illus- LOBAL of this infectious neurology news, and will G Bangkok (Thailand), for trated with photographs or oth- disease. PAGE 10 be published by Elsevier. starring in the first of er images—an interesting MRI, LSEVIER

We are all grateful to Prof. E these. for example. And with all articles, Jagjit Chopra, who has New WORLD NEUROLOGY editor in chief Dr. We’re also interested in we will want to have a photo- done a spectacular job of Mark Hallett plans to revamp the newsletter. news from the National So- graph of you, too. editing WORLD NEUROLOGY cieties that make up the WORLD NEUROLOGY should be a From Thailand: for the past decade. . Presently, I WFN. For example, the German useful and educational newsletter. Dr. Rawiphan So who am I? I am a neurolo- served as an associate editor for Society just celebrated its 100th Send your feedback and ideas to Witoonpanich gist at the National Institute of the journal Brain as well as for a anniversary. In this issue, Dr. [email protected]. writes about her Neurological Disorders and new journal, Brain Stimulation. Günther Deuschl and his col- Let’s keep each other informed practice in Thailand, , at the National Institutes Although I hope to assemble leagues present a brief history of and knowledgeable, so we can home of the 2009 of Health in Bethesda, Md., an editorial board to help me in the society. We will highlight oth- bring good neurologic practice to World Congress on where I do clinical research. My the coming months, as the new er national societies in upcoming our patients and have some fun Neurology. PAGE 12 interests are clinical neurophysi- medical editor, I will be looking issues. along the way. ology and movement disorders. I for input from everyone. So This issue also includes the Mark Hallett recently completed an 8-year please send in local news, or first “Neurologic Pearl.” This Editor in Chief term as editor in chief of Clinical news of interesting medical and column will give an in-depth World Neurology Oral Agent for Form of Multiple Sclerosis Shows Promise

BY DOUG BRUNK Comi of the institute of experi- terview, “This is a first step in the study was the mean cumulative surprised the researchers, con- Elsevier Global Medical News mental neurology at the Vita- direction of identifying a therapy number of GdE lesions per scan sidering that the 0.3-mg dose Salute San Raffaele University, for use early in the course of mul- in the last four scans. demonstrated efficacy, compared atients with relapsing remit- Milan. tiple sclerosis which is convenient, The researchers reported that with placebo, in a previous study Pting multiple sclerosis who Developed by Teva Pharma- safe, and effective. Whether oral patients in the laquinimod 0.6 (Neurology 2005;64:987-91). took daily oral laquinimod over a ceutical Industries Ltd., laquini- laquinimod meets these charac- mg daily group demonstrated a One possible explanation could 36-week period achieved a 40% mod is an oral immunomodula- teristics remains to be seen.” 40% reduction in the mean num- be that the previous study used a reduction in gadolin- tory drug that is In a study conducted at 51 cen- ber of GdE lesions over the last triple dose of gadolinium, “which ium-enhancing le- believed to exert anti- ters in nine countries, Dr. Comi four scans, compared with those increases the harvest of active sions compared with inflammatory activity and his associates randomized in the placebo group (a mean of multiple sclerosis lesions by 60%, patients who took in the relapsing re- 102 patients with relapsing re- 2.6 vs 4.2, respectively). Patients and, as a consequence, increases placebo, according to mitting form of MS mitting multiple sclerosis to in the laquinimod 0.3 mg daily the statistical power of MRI- phase II trial results. by the Th1-Th2 shift. placebo, 98 to laquinimod 0.3 mg group had a mean number of monitored trials,” the researchers “Overall, the effi- To date, approved daily, and 106 to laquinimod 0.6 GdE lesions similar to the place- cacy and safety pro- drugs for MS are all mg daily for 36 weeks (Lancet bo group (3.9). The latter finding See Multiple Sclerosis • page 2 file emerging from injectable and in- 2008;37:2085-92). this and from a pre- clude glatiramer ac- Patients were aged 18-50 years vious phase II clini- DR. GIANCARLO etate, interferon-β, and underwent brain MRI and cal trial, in combina- COMI natalizumab, and mi- clinical assessments 4 weeks be- tion with the oral toxantrone. fore the study started, at baseline, route of administration, make Prof. Alastair Compston, head and then every 4 weeks for 9 laquinimod a promising thera- of the department of Clinical months. The researchers mea- peutic opportunity for patients at the University sured the number of gadolinium- with relapse remitting multiple of Cambridge (), who enhancing (GdE) lesions at weeks sclerosis,” reported the re- was unaffiliated with the study, 12, 16, 20, 24, 28, 32, and 36. searchers, led by Dr. Giancarlo told WORLD NEUROLOGY in an in- The primary end point of the 2 • WORLD NEUROLOGY WWW.WFNEUROLOGY.ORG • JULY 2008

GdE Lesions In an accompanying editorial, Dr. B. Mark New Editor in Chief Keegan and Dr. Brian G. Weinshenker of the Fell 40% department of neurology at the Mayo Clinic, Mark Hallett Takes Over Rochester, Minn., noted that many patients Multiple Sclerosis • from page 1 would prefer an oral medication such as he process of reformatting world. WORLD NEUROLOGY will explained. “GdE lesions visible only on triple- laquinimod and recommended that a head-to- WORLD NEUROLOGY is over, serve as a support mechanism to a dose scans are usually smaller and with a less- head analysis of the drug with existing im- Tand the inaugural issue, which sense of solidarity, of knowing what severe blood-brain barrier dysfunction than munomodulatory medications be conducted you now hold in your hand, is the is going on in neurology every- those enhanced after a standard dose.” (Lancet 2008;37:2059-60). They also called for new link that will connect neurolo- where. The current trial used a standard dose of careful investigations of safety, “because seri- gists all over the world. Therefore, WORLD NEUROLOGY gadolinium. Until the Chinese Society of Neu- will provide information about cur- In terms of safety, two adverse events po- rology joined the World Federation of rent developments in modern neu- tentially attributable to laquinimod occurred. THIS IS THE FIRST STEP Neurology last year, we served a rology, with dissemination of thera- One patient in the laquinimod 0.6 mg daily TOWARD DEVELOPING A membership of about 25,000. peutic advances and discussions on group had Budd-Chiari syndrome and was That number is now considerably hot topics in the neurologic com- found be heterozygous for the factor V Leiden CONVENIENT, SAFE, AND EFFECTIVE higher, and I am proud to say that the munity. mutation, “which is associated with up to THERAPY FOR MS. We are delighted to have Mark 30% of cases of venous thrombosis,” the re- Hallett as our new editor in chief. searchers noted. “This case highlighted the THE NEWSLETTER’S Because he was the chair of the fi- possibility of an increased risk for thrombosis ous adverse effects are commonly not evident nance committee until he took over in patients with pre-existing thrombophilia until phase II studies are started (as with lino- FUNCTION IS TO CONVEY his new position, Mark Hallett exposed to laquinimod, which needs to be fur- mide) or until after approval (as with natal- INFORMATION FROM AND TO knows the World Federation of ther explored.” izumab).” WFN’S CENTRAL Neurology very well. Dr. Hallett did One patient in the laquinimod 0.3 mg daily Teva Pharmaceuticals sponsored and fund- his neurology training at Massachu- group had persistent elevation in liver enzymes ed the study. Dr. Comi disclosed receiving ADMINISTRATION. setts General Hospital, Boston, and during the early phase, but did not show clini- compensation from Teva and other pharma- has a broad background in neurolo- cal signs of liver injury. ceutical companies. ■ gy and clinical neurophysiology. He World Federation of Neurology has currently serves as the chief of the become a global organization with Medical Neurology Branch of the members in 102 countries, in which U.S. National Institutes of Health, WFN Committee Has Named more than 50 different languages are and chief of its Human Motor Con- spoken. trol Section. Candidates for Trustee Post The function of the newsletter, Dr. Hallett has also been the pres- WORLD NEUROLOGY, is to conduct ident of the Movement Disorder So- he following candidates in accordance with the Fed- Chandos St., , W1G news to and from our many global ciety and vice president of the Amer- Tare recommended by the eration’s Memorandum and 9DR, United Kingdom. members. The redesigned WORLD ican Academy of Neurology. Nominating Committee of Articles of Association. Nominations must be re- NEUROLOGY will help to distribute in- In conclusion, it is a real honor for the World Federation of Neu- It is open to anyone to ceived by September 22, 2008. formation from and to the central the World Federation of Neurology rology for the Elected Trustee make additional nominations Recommended candidates: administration, with its various re- to have Mark Hallett as the editor in post, which is falling vacant in by securing the supporting Prof. Natan Bornstein search groups and committees, as chief of our international newsletter, 2008 as Prof. Marianne de signatures of five or more au- (Israel) well as inform the regional neuro- especially at this crucial time when Visser, from the Netherlands, thorized delegates and sub- Dr. Antonio Culebras logic communities. our organization is expanding and retires. mitting the name(s) of the in- (Spain/USA) However, the newsletter also aims coming to new crossroads. The nominees are recom- dividual(s) in question to the Dr. Gustavo Román to be a medium through which the Johan A. Aarli mended to the membership following address: Secretary- (Colombia/USA) individual neurologist can stay in President through their representatives Treasurer General, c/o the Dr. Stephen Sergay contact with colleagues around the The World Federation of Neurology on the Council of Delegates, London Secretariat Office, 12 (USA) ■

ELSEVIER GLOBAL MEDICAL NEWS WORLD A Division of the International Medical News Group President IMNG Alan J. Imhoff EUROLOGY Executive Director, Editorial Mary Jo M. Dales N Executive Editor, IMNG Denise Fulton WORLD FEDERATION OF NEUROLOGY WORLD NEUROLOGY, an official publication of the World Federation Executive Editor, EGMN Kathy Scarbeck Editor in Chief Dr. Mark Hallett of Neurology, provides reports from the leadership of the WFN, its Editor Sally Koch Kubetin EDITORIAL ADVISORY BOARD member societies, neurologists around the globe, and news from the Associate Editor Denise Napoli Dr. Jagjit S. Chopra cutting edge of clinical neurology. Content for WORLD NEUROLOGY is VP, Medical Education Sylvia H. Reitman Dr. Daniel Truong provided by the World Federation of Neurology and Elsevier Global Senior Director, Marketing and Research Janice Theobald Dr. Theodore Munsat Medical News. Dr. William M. Carroll Circulation Analyst Barbara Cavallaro Executive Director, Operations Jim Chicca WFN OFFICERS Disclaimer: The ideas and opinions expressed in WORLD NEUROLOGY President: Dr. Johan Aarli do not necessarily reflect those of the World Federation of Neu- Director, Production and Manufacturing Yvonne Evans First Vice-President: Dr. Vladimir Hachinski rology or the publisher. The World Federation of Neurology and El- Production Manager Judi Sheffer Secretary-Treasurer General: Dr. Raad Shakir sevier, Inc., will not assume responsibility for damages, loss, or Creative Director Louise A. Koenig claims of any kind arising from or related to the information con- ELECTED TRUSTEES tained in this publication, including any claims related to the prod- EDITORIAL OFFICE Prof. Marianne de Visser; Prof. Werner Hacke; Dr. Ryuji Kaji ucts, drugs, or services mentioned herein. 5635 Fishers Lane, Suite 6000 CO-OPTED TRUSTEES Rockville, MD 20852 Editorial Correspondence: Dr. Roger Rosenberg; Dr. Niphon Poungvarin Send editorial correspondence to +1-240-221-4500 Fax: +1-240-221-2541 REGIONAL DIRECTORS WORLD NEUROLOGY, 5635 Fishers Lane, Suite 6000, US ADVERTISING Dr. Jacques De Reuck (Europe); Dr. Steve Sergay (North America); Rockville MD 20852, U.S.A.; [email protected]; Aaron Wattenberg Dr. Mario Tolentino-Dipp (Latin America); Dr. Ashraf Kurdi (Pan Arab); Phone +1-800-798-1822 60 Columbia Rd., Building B Dr. Gilbert Avode (Pan Africa); Dr. Bhim Sen Singhal (Asia Oceania) Address Change: +1- Fax change of address to 973-290-8245 Morristown, NJ 07960 EXECUTIVE DIRECTOR POSTMASTER: Send change of address (with old mailing label) +1-973-290-8200 Fax +1-973-290-8250 Keith Newton nd World Federation of Neurology to WORLD NEUROLOGY, Circulation, 60 B, Columbia Rd., 2 Fl., INTERNATIONAL ADVERTISING 12 Chandos Street Morristown NJ USA 07960 Martin Sibson London W1G 9DR UK WORLD NEUROLOGY, ISSN: 0899-9465, is published quarterly by Business Development Executive Tel: +44 20 7323 4011 Fax: +44 20 7323 4012 Elsevier Inc., 60 B, Columbia Rd., 2nd Fl., Morristown NJ Elsevier Pharma Solutions [email protected] 07960. Phone 973-290-8200; fax 973-290-8250. London, UK EDITOR OF JOURNAL OF NEUROLOGICAL SCIENCES +44 (0) 207 424 4963 Dr. Robert Lisak ©Copyright 2008, by the World Federation of Neurology [email protected] JULY 2008 • WWW.WFNEUROLOGY.ORG WORLD NEUROLOGY • 3

THE PRESIDENT’S COLUMN WFN and Regional Neurologic Groups Share a Close Link

he World Federation of rological Association, the Euro- and have the possibility of closer There are two bids for the 2011 will take place in Europe. The Neurology (WFN) is one pean Federation of Neurological contact with the corresponding Congress. One possible venue is European Federation of Neuro- T of the neurologist’s mem- Sciences (EFNS), and the World regional World Health Organiza- Cape Town, South Africa. The logical Sciences, which up to now ber organizations, membership to Federation of Neurology. tion offices in Brazzaville (Con- other is Marrakesh, Morocco. has organized successful annual which is obtained through his or The regional neurologic asso- go), Cairo (Egypt), , regional congresses, has decided her national neuro- ciations, including Manila, New Delhi, and Wash- that the World Congress that logic association. the American Acade- ington. ‘WE LOOK FORWARD TO year will also serve as an EFNS However, most neu- my of Neurology, The World Federation of Neu- congress; there will be no inde- rologists also are usu- the AOAN, the rology looks forward to closer CLOSER CONTACT WITH pendent EFNS meeting in 2013. ally members of lo- EFNS, the Latin contact with the regional neuro- THE REGIONS AND TO The venue for that joint meeting cal, regional American Neurolog- logic organizations in part be- FUTURE EXCHANGE has not yet been chosen; the professional organiza- ical Region, the Pan cause of the coming geographi- EFNS will have the strongest tions. An Indian neu- African Association cal rotation of world congress PROGRAMS.’ voice in that decision, and we rologist, for example, of Neurological Sci- venues. will let our members know. might be a member ences, and the Pan The 2009 World Congress of The ultimate goal is to increase of the Indian Acade- BY JOHAN A. AARLI Arab Union of Neu- Neurology, as you likely know, is Both are extremely fascinating the visibility of the global re- my of Neurology, the PRESIDENT, WFN rological Sciences, set for Bangkok, in Thailand. The and attractive cities. The ultimate sponsibilities of the World Fed- Asian Oceanian Asso- are strong and inde- 2011 Congress will take place on decision of congress venue will eration of Neurology. We look ciation of Neurology (AOAN), pendent organizations. They or- the African continent. In 2013, be made at the Council of Dele- forward to closer contact with and the World Federation of Neu- ganize annual or biennial region- the World Congress of Neurolo- gates Meeting in New Delhi in the regions and to future ex- rology. A Norwegian neurologist, al congresses. The presidents of gy is scheduled to be held some- October, 2008. change programs for neurolo- on the other hand, might be a the regional associations are ex- where in Europe, and in 2015, in As I mentioned, the World gists from different parts of the member of the Norwegian Neu- officio WFN regional directors Latin America. Congress of Neurology in 2013 world. ■ World Neurology Foundation European Board Examination Launches New Web Site In Neurology Is Scheduled t is my pleasure as webmaster to an- ucation in countries in need—and neu- Inounce the launch of the new Web site rologic care purposes. To Commence in 2009 for the World Neurology Foundation. An About Us section that contains The Web site was released on April 27, content regarding mission, foundation he European Union of Medical Spe- that they are eligible can be admitted. 2008. For immediate access and to check overview, leadership, and staff profiles. Tcialists’ Section of Neurology and the Because their practical skills will be con- out what’s new, please visit www.world- A 2008 Projects feature, which de- European Board of Neurology will estab- firmed by the candidates’ national training neurology.org. scribes the World Neurology Foundation’s lish and administer a European board ex- society, the examination will be a multi- The World Neurology Foundation projects, projects’ locations, overviews on amination in neurology, beginning in step process. was incorporated in 1999 as a charitable project toolkits for Africa, Neuroshare, 2009. This certification exam is part of a Although many European countries arm of the World Federation of Neu- and 2008 affiliate membership. broader European effort to improve train- have their own certification system, and rology. Information about Partnership Pro- ing in neurology. the UEMS /EBN examination will have no The new Web site will serve the orga- grams, including lectureships, continu- To further assure the quality of neurol- nization internally by providing infor- ing medical education, and certification ogy training, the UEMS/EBN has formed mation about the mission, program, and training. a committee to visit neurology depart- SUCCESSFUL EBN EXAMINATION meetings of the foundation to the orga- Opportunities for you to make do- ments throughout Europe. Residents of nization’s staff, its 12-member volunteer nations that will go towards helping the centers that are visited will receive a CANDIDATES WILL BE AWARDED board of directors, and the six-member support various foundation projects, in- reduction in the examination fee. THE TITLE OF FELLOW OF THE advisory council. cluding the capacity to donate for use in The examination will consist of two EUROPEAN BOARD OF The new Web site will also provide an specific programs. parts. One will contain both written and essential external marketing function by Congratulations to the World Neu- multiple choice questions; the other will NEUROLOGY. engaging others in the foundation’s mis- rology Foundation on this new Web site. be an oral examination in which the can- sion and projects, thus promoting finan- Please don’t hesitate to direct any ques- didates will answer structured questions cial support. tions about the site to the World Neu- about four specific cases. legal power, it is hoped that countries Users can expect the following high- rology Foundation’s executive director, Candidates can earn extra points by without a system will adopt this exami- lights when accessing the new Web site: Carrie Becker, who can be reached at: presenting either one of their own cases nation as a standard. A home page that emphasizes the [email protected]. or results from scientific research done The EBN examination is being devel- World Neurology Foundation’s central John Johnson during their training. oped as a measure of excellence, such as mission—to serve as a catalyst for the World Neurology Foundation Scientific panels from the European those already established by many other promotion of neurological care and ed- Webmaster Neurological Society (ENS) and European UEMS sections and boards. Federation of Neurological Societies The successful candidates will be (EFNS) will collaborate to develop the awarded the title of Fellow of the Euro- Please Visit the Updated 2009 World exam. Scientific committees from the ENS pean Board of Neurology. and EFNS have teamed with the In cooperation with the big European Congress of Neurology Web Site UEMS/EBN to form a joint European ex- neurologic societies (EFNS and ENS), the amination committee, which will have an UEMS/EBN will launch this important he Web site for the official general information about the advisory role to the UEMS/EBN. step in training in neurology. T19th World Congress of Congress. The Web site will be The application for the examination is For more information, visit the UEMS’ Neurology, to be hosted by updated continuously as the available from the UEMS/EBN Web site Web site at www.uems-neuroboard.org, the World Federation of Congress approaches, so (www.uems-neuroboard.org/ebn). or e-mail the VMA office in : Neurology, October 24-30, check back often as more is At the time of the implementation of [email protected]. ■ 2009, in Bangkok, Thailand, added. the examination, only those candidates has now been updated. Infor- Visit the Web site today, at from EU/EEA (European Economic By Prof. Wolfgang Grisold, who is chair of mation is now available regard- www.wcn2009bangkok.com, to Area) countries and who have either the education committee at the EFNS and ing the preliminary program and see what’s new. passed their national examination or have president of the EBN and Prof. Svein confirmation from their national society Mellgren, who is the EBN chair. 4 • WORLD NEUROLOGY WWW.WFNEUROLOGY.ORG • JULY 2008

Meeting Report: WFN Junior Traveling Fellowship 2008 From Shanghai to Chicago, the WFN group meeting, along with the “Anterior Horn” platform sessions, supports its members around the globe. that I learned about up-to-date re- search and therapeutic progress in Editor’s Note: 2008 from the World Federation the MND/ALS field that will bring This year, Dr. Xiang-Jun Chen was of Neurology made it possible for hope to my patients. one of several young neurologists to me to attend. The Junior Traveling Fellowship receive a World Federation of Neu- The AAN annual meeting is helps young neurologists in low- rology Traveling Fellowship, which among one of the world’s largest and lower-middle income coun- he used to attend the 2008 annual neurology meetings. This year, the tries attend an international meet- meeting of the American Academy AAN celebrated its 60th anniver- ing, reflecting the spirit of the of Neurology in Chicago. At the re- sary meeting in Chicago from WFN mission. The professional HEN quest of WORLD NEUROLOGY, he April 12 to 19, bringing together knowledge gained from this mem- C UN has written a report, which appears more than 10,000 orable meeting will definitely im- -J

below, on the experience and what professionals from all over the prove my practice in the future. IANG . X he gained as a result of his award. world. As planned, I partook of all More importantly, the spirit of the R five intriguing plenary sessions fea- WFN will be in my mind through- D ast year, I completed my turing the latest neurologic re- out my career inneurology. OURTESY

postdoctoral training in the search. Among the popular Inte- Xiang-Jun Chen, M.D., Ph.D. C Lfield of neurogenetics at the grated Neuroscience Sessions, the attending neurologist, Pictured from right to left: Dr. Chen, WFN Secretary Susan Bilger, University of Chicago and began “Mitochondria in Diseases” ses- Huashan Hospital, WFN Executive Director Keith Newton, and Jane Aberle, the my long-pursued career in acade- sion was most attractive to me be- Fudan University, China development associate of the World Neurology Foundation. mic neurology as a faculty mem- cause I have been working on a ge- ber in the neurology department netic neuropathy mouse model at Fudan University Huashan with a defect of mitochondrial WFN Traveling Fellowships Application Criteria Hospital (Shanghai, China). To transport. During this session, I me, attending the American Acad- met many of my neurologist peers ach year, the World Federation of Neurolo- sumé, bibliography, and an estimate of ex- emy of Neurology (AAN) annual involved in neuroscience research Egy offers 10 Junior Traveling Fellowships penses, to a maximum of £1,000; and enclose meeting is always an excellent way and exchanged scientific view- for young neurologists from countries classi- a letter of recommendation from the head of to refresh and update my knowl- points with them in our fields of fied as low- or lower-middle income by the the department, plus an abstract if a paper or edge of clinical neurology. How- common interest. Through the in- World Bank to attend an international meet- poster is to be presented. Awards are assessed ever, as a young neurologist now troduction of Dr. Raymond Roos ing. The candidates for the awards must hold a by the Executive Committee of the WFN’s living in Shanghai, the expense of of the University of Chicago, I post no higher than associate professor and Education Committee. traveling to the United States to at- also had the opportunity to attend must be under 42 years of age. Applicants Nominations for the 2009 fellowships are not yet tend this meeting was intimidat- an ALS Association-sponsored must state the names and dates of the pro- being considered. Check WORLD NEUROLOGY for ing. Fortunately, the generous gift Drug Company Working Group posed meeting they wish to attend; send a re- updates on when applications will be accepted. of a Junior Traveling Fellowship Meeting. It was from this small Vladimir Hachinski Is a Leader of Many Accomplishments

BASED ON AN INTERVIEW WITH “vascular cognitive impairment” to en- (England), and three ent part of the world—the premise being DR. VLADIMIR HACHINSKI compass the broad spectrum of cognitive doctor honoris causa from the universities that such meetings have the greatest im- impairment caused by or associated with of Salamanca, Buenos Aires, and Cordo- pact in the region in which they are held, ince Dr. Vladimir Hachinski, professor vascular factors. He developed the Hachin- ba, respectively. He was the first recipient and that we need to reach neurologists or Sof neurology and Distinguished Uni- ski Ischemic Score (2,150 citations) to iden- of the Trillium Award, which is given to potential neurologists who cannot travel versity Professor at the University of West- tify the vascular component of cognitive the best clinical researcher in the province internationally. The greater frequency of ern Ontario, London, was impairment, and led, along with of Ontario, and he also received the in- meetings also allows a platform for in- elected first vice president of Gabrielle Leblanc, a group of ternational Mihara Award, given every 4 creased activities in the years between the WFN, he has done some experts in developing common years to someone who has made major them. impressive work for the WFN. standards for describing and contributions to the field of stroke. Dr. Hachinski currently is fostering co- But Dr. Hachinski has long studying cognitive disorders. He As Canada’s delegate to the WFN and operation between the WFN and other in- been one of the more presti- also helped develop a model to chairman of its steering committee for two ternational organizations, specifically a gious members in the field of study the interaction between terms, Dr. Hachinski was presented with a collaboration with the International Brain neurology. and diploma whose citation reads in part, Research Organization (IBRO). Born in Europe and raised in Alzheimer’s. “Brought parity and fairness in the inter- He is encouraging a survey of the mem- Latin America, Dr. Hachinski Dr. Hackinski has also pub- relationship of the WFN with regional and bership, particularly of national delegates, received his MD from the Uni- VLADIMIR lished 17 books and more than national groups.” to identify how the WFN can better serve versity of in 1960. Af- HACHINSKI, M.D. 600 scientific papers, book As first vice president, he initiated the its members, broaden the base of those ter his there, he was chapters, editorials, and other process that led to the decision to hold a participating in its activities, and involve a fellow at the National Hospital for Ner- works. As editor in chief of Stroke, he ini- world congress every 2 years in a differ- neurologists in the organization. ■ vous Diseases at Queen Square, London tiated its translation into Spanish, Russian, (England), and the University of Copen- Italian, Portuguese, Chinese, Japanese, hagen. After his initial return to the Uni- and Korean, and a special edition for read- Dr. Hachinski Named to Order of Canada versity of Toronto in the early 1970s, he ers in India. He also started the Author joined the faculty at Western Ontario in Mentoring Program, through which he r. Vladimir Hachinski has recently Dr. Hachinski. “When I was informed 1980. With John Norris, he established the welcomes manuscripts from contributors Dbeen named to the Order of Cana- that I was to be named to the Order first acute stroke unit in Canada for the ur- in developing countries and, at an au- da, the country’s highest honor, for his of Canada, I was surprised, and felt gent care and systematic study of patients thor’s request, will assign a member of the lifetime contributions in neurology. honored and humbled to join such a with “brain attack.” He codesigned the editorial board. He arranged free sub- The award was announced just as distinguished company of Canadi- Toronto Stroke Scale with John Norris, scriptions for readers in select countries. WORLD NEUROLOGY went to press. ans,” he commented. and discovered the role of the right insula In 2004, Dr. Hachinski led a working “When I learned that the govern- The Order of Canada recognizes a of the brain in mediating cardiac compli- group that resulted in the establishment of ment was trying to track me down, I lifetime of outstanding achievement, cations of stroke, including sudden death. World Stroke Day (Oct. 29) and the devel- was in China and a few unpleasant dedication to the community, and Dr. Hachinski proposed the concept of opment of a world stroke agenda. possibilities crossed my mind,” joked service to the country. “multi-infarct ” and later that of Dr. Hachinski received an ScD from the JULY 2008 • WWW.WFNEUROLOGY.ORG WORLD NEUROLOGY • 5

Is Gene Therapy Feasible for Parkinson’s Disease?

BY MICHAEL J. AMINOFF, M.D. nondividing human cells, shows low im- 12 months; fluorodeoxyglucose–positron- A third approach involves delivery of a munogenicity, and is not associated with emission tomography (FDG-PET) showed trophic factor to the nigrostriatal system, harmacologic therapy is the main- oncogenesis. Most humans have been in- reduction in thalamic metabolism on the in the hope of slowing disease progres- stay of the symptomatic treatment fected naturally, but the mode of infec- treated side at 1 year, similar to after DBS. sion. Neurturin (NTN) is a naturally oc- Pof Parkinson’s disease; deep brain tion is unclear. The virus, when injected No adverse effects were related directly to curring structural and functional analogue stimulation is reserved for those with com- into the striatum, is taken up selectively gene therapy. of glial-derived neurotrophic factor; it sup- plications. An alternative to both may be by neurons. An alternative approach in- ports survival of dopaminergic cells in vit- gene therapy. Gene therapy by transfec- volves the gene for aromatic ro and, in animal models of PD, prevents Gene therapy works by inserting a nor- tion of the subthalamic nucle- l-amino acid decarboxylase degeneration of nigrostriatal neurons and mal gene into a nonspecific location with- us (STN) with AAV containing (AADC), which converts lev- improves function. In a phase I study, in the genome in order to replace a non- the gene for glutamic acid de- odopa to dopamine. As PD AAV-NTN was delivered intraputaminal- functional gene or to establish a new carboxylase (GAD) leads to advances, many patients re- ly bilaterally in a low or higher dose to 12 function or role for the cell. Release of the synthesis of the inhibitory neu- quire increasingly more lev- patients with stage III or IV PD and re- gene product then acts on the affected or rotransmitter γ-aminobutyric odopa to maintain benefit. In sponse fluctuations (Lancet Neurol. neighboring cells. The approach has ad- acid. This reduces STN output a phase I study, 10 patients 2008;7:400-8). There were no major ad- vantages. Depleted neurotransmitters can and hence its inhibitory effect with moderately advanced verse events, and significant clinical im- be replaced in a more physiologic manner on the thalamus. The rationale MICHAEL J. PD and response fluctuations provement was seen, compared with base- so that certain complications are less like- is thus similar to treatment by AMINOFF, M.D. received either a low or high line. ly. Therapies can be delivered in a site-spe- DBS of the subthalamic nucle- dose of AAV-AADC into the These results suggest that gene thera- cific way, so that other side effects (like the us, but the gene therapy avoids hardware- postcommissural putamen bilaterally py for PD is feasible and appears safe. mental side effects of dopaminergic drugs) based complications, programming and (Neurology 2008;70:1980-3). Patients were Whether it is efficacious remains to be are avoided. One treatment may provide maintenance. assessed clinically and by PET imaging demonstrated. And the question re- sustained benefit. And unlike DBS, there In an open-label safety study, 12 pa- with fluoro-metatyrosine (FMT), a tracer mains: is it really necessary to develop are no device complications. tients with advanced PD received unilat- that is more specific than fluorodopa for gene therapy simply to duplicate what Gene therapies have been studied in an- eral infusion of AAV-GAD into the sub- AADC activity. Clinical and PET scan im- can already be achieved by a standard op- imal models of parkinsonism for the last thalamic nucleus (four patients each in provements were noted. There were no ad- eration? ■ 20 years, but only in the last 5 years have three dose cohorts). Patients were assessed verse effects related directly to the thera- clinical studies been undertaken in hu- clinically on and off medication (Lancet py, although the surgical procedure led to DR. AMINOFF is director of the Parkinson’s mans. The usual vector for gene delivery 2007;369:2097). There were significant im- intracranial hemorrhage in two instances Disease Clinic and Research Center and the to the target cells is adeno-associated provements in motor Unified Parkinson (asymptomatic in one). It was possible to executive vice chair of the department of virus type 2 (AAV2). This virus is not as- Disease Rating Scale scores contralateral reduce dopaminergic therapy in several pa- neurology at the University of California, sociated with human disease, can infect to surgery at 3 months. These persisted to tients without loss of therapeutic benefit. San Francisco. 6 • WORLD NEUROLOGY WWW.WFNEUROLOGY.ORG • JULY 2008

Chemo Plus Radiation Cuts Glioma Recurrence at 2 Years Living beyond 2 years was significantly more likely week cycles of chemotherapy (125 pa- The researchers also performed a con- tients). Radiation was given to localized ditional probability analysis for overall sur- for patients on radiotherapy plus PCV. treatment fields and consisted of 30 1.8- vival, asking what the likelihood of living Gy fractions (54 Gy total). Postradiation longer than 2 years was for those patients 2 BY KERRI WACHTER ble for the two groups (85%-87%), but at chemotherapy consisted of 60 mg/m who had lived to 2 years. “The likelihood Elsevier Global Medical News 5 years, overall survival was 63% for the oral procarbazine (Matulane) on days 8- of living beyond 2 years was significantly radiotherapy arm and 72% for the com- 21, 110 mg/m2 oral lomustine (CeeNU) better for patients who were treated with C HICAGO — Progression-free survival bination arm (HR 0.72, P = .33), Dr. Ed- on day 1, and 1.4 mg/m2 IV vincristine on radiotherapy plus PCV versus radiation begins to improve after 2 years when ward G. Shaw reported at the annual days 8 and 29 (no greater than 2 g per therapy alone [HR 0.52, P = .03],” said Dr. patients with high-risk, low-grade glioma meeting of the American Society of Clin- dose). Shaw. are treated with chemotherapy and radia- ical Oncology. Median follow-up was 6 years. Median A similar analysis for progression-free tion rather than with radiotherapy alone, He presented these findings from the progression-free survival for the radio- survival resulted in an even more pro- according to the results of a phase III trial. Radiation Therapy Oncology Group therapy group was 4.4 years, but has not nounced advantage to those on combina- Progression-free survival rates were sim- (RTOG) protocol 9802 study for high-risk been reached for the combination arm. tion therapy (HR 0.44, P = .002). ilar at 2 years, whether patients were treat- patients who had newly diagnosed adult Likewise, median overall survival for pa- Severe toxicity (grade 3/4) was more ed with radiation alone or with radiation supratentorial low-grade gliomas (World tients in the radiation therapy arm was 7.5 common for the combination arm. Grade plus a chemotherapy regimen of procar- Health Organization grade II) and a years; median overall survival has not yet 3 toxicities occurred in 8% of patients in bazine/lomustine/vincristine (PCV)— Karnofsky performance score of at least been reached by patients in the combina- the radiotherapy arm, compared with 51% 74% and 75%, respectively. 60. High risk was defined as either being tion arm. Patients who survived beyond 2 in the combination arm. Likewise, grade The groups began to separate after 2 at least 40 years of age or being a patient years tended to be younger (less than 40 4 toxicities occurred in 3% of patients in years, however. Progression-free survival of any age with an incomplete resec- years), to have undergone resection (versus the radiotherapy arm, compared with 15% at 5 years was 46% for the radiotherapy tion/biopsy. Results for low-risk patients biopsy), and to have oligodendrogliomas/ in the combination arm. “Most of the arm and 63% for the combination arm were previously reported. oligoastrocytomas, said Dr. Shaw, who is toxicities were hematologic and re- (hazard ratio 0.60, P = .06). A total of 251 patients were random- chair of the radiation oncology depart- versible,” said Dr. Shaw. Overall survival showed a similar trend. ized either to radiation therapy alone ment at Wake Forest University, Winston- Dr. Shaw reported that he had no con- Two-year overall survival was compara- (126 patients) or to radiation plus six 8- Salem, N.C. flicts of interest. ■ Psoriasis Independently Increases Stroke Risk

BY BRUCE JANCIN cluding an estimated 4.5 million tients with severe psoriasis had and the Society for Investigative of the rates of cardiovascular, Elsevier Global Medical News U.S. adults. higher rates of obesity and smok- Dermatology. cerebrovascular, and peripheral Five percent of psoriasis pa- ing than did controls, while rates To examine the possibility that vascular disease in the same study K YOTO, JAPAN — Severe pso- tients have severe disease as de- of these and other traditional the excess stroke risk seen in se- population. riasis appears to be a potent risk fined by a need for systemic ther- cardiovascular risk factors were vere psoriasis was a function of The rationale for this addition- factor for stroke independent of apy or phototherapy. similar in patients with mild pso- toxicities of treatments for the al analysis was that MI and stroke the traditional stroke risk factors, Dr. Azfar and her coinvestiga- riasis and in controls. disease rather than being intrin- are acute thrombotic events, and Dr. Rahat S. Azfar said at an in- tors had previously shown psori- After adjustment for the it would be informative to ternational investigative derma- asis to be an independent risk major stroke risk factors— see if psoriasis is also asso- tology meeting. factor for acute MI, also using the diabetes, hyperlipidemia, ciated with increased rates Dr. Azfar presented a case-con- GPRD. But the relationship be- smoking, obesity, hyper- of chronic atherosclerotic trol study drawn from the U.K. tween psoriasis and stroke had tension, age, and gender— diseases as reflected in the General Practice Research Data- never before been studied. patients with mild psoriasis appropriate diagnostic base (GPRD) in which she found The GPRD is an extensive elec- were found to have a sta- codes, as well as procedure that severe psoriasis was associ- tronic medical record including tistically significant 6% per codes for coronary revas- ated with an excess stroke risk more than 9 million U.K. patients year increased relative risk cularization, carotid en- amounting to one additional under the care of general practi- of stroke. darterectomy, and periph- stroke per 530 patients per year tioners/family physicians in 450 In contrast, the stroke DR. RAHAT S. MR. DANIEL B. eral vascular intervention. beyond background levels of tra- primary care practices. Dr. Azfar risk in patients with severe AZFAR SHIN This indeed proved to be ditional stroke risk factors. reported on 129,143 patients with psoriasis was increased by the case. As for stroke, the “Given the prevalence of pso- mild psoriasis in 1987-2002 and 43% per year, compared with sic to severe psoriasis itself, the in- associated risks generally were riasis worldwide, these numbers 496,666 contemporaneous con- matched controls. vestigators reanalyzed the data af- greater with severe than with carry a potentially significant im- trols without psoriasis, along The attributable risk of stroke in ter excluding methotrexate users mild psoriasis, noted Mr. Shin, a pact on public health,” observed with 3,603 patients with severe patients with mild psoriasis was 2.4 or restricting the analysis to pa- medical student at the university. Dr. Azfar, of the University of psoriasis and 14,330 separate con- per 10,000 person-years, tients treated with oral retinoids. (See chart.) Pennsylvania, Philadelphia. trols. The mean follow-up was and with severe psoriasis it was 1.9 It didn’t have any significant im- There was, however, one glar- Psoriasis affects roughly 2.5% about 4 years. strokes per 1,000 person-years. pact upon the results. Neither ing exception to the broad trend. of the population worldwide, in- As found in other studies, pa- A caveat: Data audit suggested did exclusion of psoriatic arthri- How to explain the lack of asso- up to 15% of patients catego- tis patients. ciation between severe psoriasis rized in the GPRD as having mild A German dermatologist in and increased peripheral vascu- Odds Ratios of Atherosclerotic Diseases psoriasis may actually have had the audience questioned the reli- lar disease? In Psoriasis Patients at 4-Year Follow-Up moderate disease. ability of psoriasis diagnoses in “I’ve asked some of my col- Mild psoriasis Severe psoriasis If so, truly mild psoriasis may the GPRD. In his country, he leagues in cardiovascular medi- (n = 129,143) (n = 3,603) not be associated with any sig- added, general practitioners get it cine, and they think peripheral nificant excess in strokes, ac- wrong at least 30% of the time. vascular disease is significantly un- 1.05 1.08 1.31 1.11 1.00 1.23 cording to Dr. Azfar. Dr. Azfar replied that she and derdiagnosed,” commented Dr. Control 0.99 The working hypothesis is that her coworkers have formally val- Joel M. Gelfand, who is the senior groups the link between psoriasis and idated the ability of U.K. general investigator in the GPRD studies stroke—and MI as well—lies in practitioners to reliably diagnose and medical director of the clini- EWS N Th1/Th17-mediated systemic in- the disease. cal studies unit in the department flammation, a prominent shared In any event, severe psoriasis of dermatology at the university. Cardiovascular Cerebrovascular Peripheral EDICAL disease disease vascular disease M feature, she explained at the was typically diagnosed by a con- The ongoing GPRD studies are Notes: Odds ratios adjusted for traditional factors. There were 496,666 meeting, sponsored by the Euro- sultant dermatologist. partially funded by an unrestrict- LOBAL controls for mild psoriasis and 14,330 controls for severe psoriasis. G pean Society for Dermatological Elsewhere at the conference, ed grant from Centocor. Source: Mr. Shin Research, the Japanese Society Daniel B. Shin, Dr. Azfar’s coin- The investigators reported hav-

LSEVIER ■ E for Investigative Dermatology, vestigator, presented an analysis ing no conflicts of interest. Leading resources in clinical neurology!

The offi cial journal of the Acute Pain World Federation of Neurology Alzheimer’s and Dementia Autonomic Neuroscience: THE journal for the prompt publication of Basic and Clinical studies on the interface between clinical Brain & Development neurology and the basic sciences. Clinical Neurology and And did you know.. Journal of the Neurosurgery Neurological Sciences is your ultimate resource for the latest developments and Clinical Neurology News research on , Stroke Clinical Neurophysiology and Multiple Sclerosis! Epilepsy & Behavior Epilepsy Research www.elsevier.com/jns European Journal of Paediatric Neurology EDITOR R.P.LISAK Volume 250, issues 1–2 December 1 2006 ISSN 0022-510X European Journal of Pain Experimental Neurology Journal of Clinical

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Review Regional Anesthesia and Neuromuscular disorders in critical illness – L. Pandit, A. Agrawal (Mangalore, India) 621 Original articles Laterality does not influence early mortality in MCA ischemic stroke – I. Mateo, A. Pinedo, I. Escalza, J.C. Garcia-Monco (Vizcaya, Spain) 628 Vascular cognitive impairment in patients with late-onset seizures after an ischemic stroke – J. De Reuck, M. De Clerck, G. Van Maele (Ghent, Belgium) 632 Cardiovascular risk factors in patients aged 85 or older with ischemic stroke – A. Arboix, M. Miguel, E. Císcar, L. García-Eroles, J. Massons, M. Balcells (Barcelona, Spain) 638 Pain Medicine The interleukin-10 levels as a potential indicator of positive response to interferon beta treatment of multiple sclerosis patients – H. Bartosik-Psujek, Z. Stelmasiak (Lublin, Poland) 644 Medically refractory epilepsy associated with temporal lobe ganglioglioma: Characteristics and postoperative outcome – A. Radhakrishnan, M. Abraham, V.V. Radhakrishnan, S.P. Sarma, K. Radhakrishnan (Trivandrum, India) 648 Alleviation of intracranial air using carbon dioxide gas during intraventricular tumor resection – T. Beppu, K. Ogasawara, A. Ogawa (Morioka, Japan) 655 Clinico-pathological and immunohistochemical characteristics associated to recurrence/regrowth of craniopharyngiomas – M.L. Tena-Suck, C. Salinas-Lara, R.I. Arce-Arellano, D. Rembao-Bojórquez, D. Morales-Espinosa, J. Sotelo, O. Arrieta 661 Case reports Parkinsonism Respiratory failure in a patient with antecedent poliomyelitis: Amyotrophic lateral sclerosis or post-polio syndrome? – S.-i. Terao, N. Miura, Seizure: European Journal A. Noda (Aichi, Japan), M. Yoshida, Y. Hashizume, H. Ikeda, G. Sobue (Japan) 670 & Related Disorders Bilateral C5 motor paralysis following anterior cervical surgery—a case report – K.S. David, R.D. Rao (Milwaukee, WI, USA) 675 Correlation of magnetic resonance images with in acute Wernicke’s encephalopathy – Y.-T. Liu, J.-L. Fuh, J.-F. Lirng, A.F.-Y. Li, D.M.-T. Ho, S.-J. Wang (Taipei, Taiwan) 682 Subacute aseptic meningitis as neurological manifestation of primary SjÖgren’s syndrome – R. Rossi, M. Valeria Saddi (Nuoro, Italy) 688 Official Journal of the WFN Research Group Thin corpus callosum and amyotrophy in spastic paraplegia—Case report and review of literature – B. Winner, C. Gross, G. Uyanik, on Parkinsonism and Related Disorders W. Schulte-Mattler, R. Lürding, J. Marienhagen, U. Bogdahn (Regensburg, Germany), C. Windpassinger (Graz, Austria), U. Hehr, J. Winkler (Regensburg, Germany) 692 of Epilepsy Camptocormia or Pisa syndrome in multiple system atrophy – J. S2awek (Gdan´sk, Poland), M. Derejko (Warszawa, Poland), P. Lass, M. Dubaniewicz (Gdan´sk, Poland) 699 “Frontal variant Alzheimer’s disease”: A reappraisal – A.J. Larner (Liverpool, UK) 705 Transient tetraplegia after cervical facet joint injection for chronic neck pain administered without imaging guidance – J.G. Heckmann, C. Maihöfner, S. Lanz, C. Rauch, B. Neundörfer (Erlangen, Germany) 709 Adie’s pupils in paraneoplastic ganglionopathy with ANNA-1 – J.V. Campellone, A. Hageboutros (Camden, NJ, USA) 712 Book reviews IN THIS ISSUE Head injury: Pathophysiology and Management – D. Van Dam (Wilrijk, Belgium) 715 (Contents continued on OBC) Sleep Medicine Parkinson’s disease and family GAITRite system evaluation of history parkinsonian bradykinesia Clinical characterization of Estimating the value of novel LRRK2 G2019S Parkinson patients interventions for Parkinson's disease

Mutations in the parkin Gene ERP and cognition in PD Psychosis and depression in PD Vascular parkinsonism Sleep Medicine Reviews Paroxetine in multiple system Central pontine myelinolysis and atrophy extrapontine myelinolysis

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NEWS FROM THE NATIONAL SOCIETIES German Society for Neurology Celebrates 100th Anniversary

BY DR. STEFAN BRANDT, of expulsion from the profession. tensive care units or provide DR. GÜNTHER DEUSCHL, Some were deported to concen- emergency neurologic treatment AND DR. AXEL tration camps or chose suicide. in interdisciplinary intensive care KARENBERG The long list of emigrants reads wards. like a who’s who of Central Eu- Another noteworthy aspect of Editor’s note: As the German So- ropean neurology: Josef Gerst- the growth of neurology in Ger- ciety for Neurology celebrates its mann, Kurt Goldstein, Sir Ludwig many is the ongoing development 100th year, we present a look back. Guttmann, Friedrich Heinrich of neurologic rehabilitation as a Lewy, Adolf Wallenberg, and subspecialty. Many severely hand- The Founding Years Robert Wartenberg. icapped patients, particularly “We call ourselves neurologists Because the Third Reich sought STEFAN GÜNTHER AXEL KARENBERG, those with stroke, receive addi- (Nervenärzte) and are proud to to stop or even reverse medical BRANDT, M.D., PH.D. DEUSCHL, M.D., PH.D. M.D., PH.D. tional treatment in rehabilitation declare ourselves representatives specialization, the neurologic as- hospitals because of the shorten- of this science. We are united by sociation was forcibly “reunited” guilty of crimes against humanity, man neurologists to be intro- ing of the inpatient period after our love of the profession.” When with psychiatry in 1935, a step the hospitals were destroyed, and duced to modern techniques of acute neurologic events. Rehabil- spoke that thwarted any further aspira- our international reputation in the clinical and experimental re- itation is also becoming more these words on Sept. 14, 1907, to tions of autonomy. field had been lost. With Germany search. German neurologists popular as a research field at Ger- open the first meeting of the As- More appalling was the in- divided after the war, the Cold have done pioneering research, man universities. A major break- sociation of German Neurologists volvement of German brain re- War limited contact between neu- particularly in the field of stroke, through came with the invitation (Gesellschaft Deutscher Ner- searchers, neuropathologists, and rologists on the two sides of the movement disorders, pain, mul- of the WFN to host the 13th venärzte), the discipline could al- neurologists in the ideologically country. In West Germany, neu- tiple sclerosis, epilepsy, and many World Congress of Neurology in ready look back on a long tradi- other fields; clinical neurophysi- in 1985. tion. In 1840 Moritz H. Romberg ology is a closely related disci- published the world’s first text- pline whose research is promot- The DGN Today book that described all of the dis- ed by the German Society for The most important event of the orders of the nervous system that Clinical Neurophysiology. second half of the last century for were then known. the country as well as the DGN In subsequent decades, physi- The Years of Prosperity was the reunification of Ger- cians interested in neurology The Joint Congresses, first with many in 1989. The DGN today produced impressive research re- French neurologists in 1966-1970 has almost 6,000 members and sults. In 1874, Carl Wernicke de- and then with British neurolo- holds annual congresses. In 2007, scribed sensory aphasia and in gists in 1968-1971, were among more than 5,000 people came to 1884, Wilhelm Erb discussed the first indications of the grow- the Anniversary Congress in muscular dystrophy. In 1891, ing acceptance of German neu- Berlin. Our meetings are com- Heinrich Quincke reported the rology after the war. Many of bined with those of a national first successful spinal tap; in the 41 university departments in Academy of Neurology and cov- 1906, Alois Alzheimer published 36 German universities were es- er all aspects of the specialty. The a case of presenile dementia. tablished between 1960 and 1980. DGN is engaged in setting the EUROLOGY

Shortly before World War I, N During that time, neurology in- standards for neurologic care. It Max Nonne in Hamburg and Ot- creasingly was accepted as an aca- has developed and constantly up- fried Förster in Breslau set up in- demic specialty and a desperate- dates guidelines for therapy and OCIETY OF dependent departments. In con- S ly needed discipline for daily diagnosis of many neurologic dis- trast to those in France, Great patient care. In 1968, neurology eases. ERMAN

Britain, and the United States, G became an autonomous medical During recent years, the DGN German medical schools and Founders of the German Society for Neurology. Clockwise from speciality in West Germany, with changed its internal structure community hospitals long con- left: Heinrich-Moritz Romberg (1795-1873), Alois Alzheimer board examination and certifica- and now is organized as a con- sidered clinical neurology a sub- (1864-1915), Carl Wernicke (1848-1905), Hermann Oppenheim tion, while in East Germany, neu- temporary society with its per- discipline of psychiatry or internal (1858-1919), Max Nonne (1861-1959), and rology and psychiatry remained manent professional headquar- medicine. (1840-1921). combined as a single discipline. ters in Berlin-Mitte. Our future During the first quarter-centu- Two new West German neurol- challenge is to maintain high ry, membership of the Association motivated and systematic murder rologists were rechristened in 1950 ogy journals (EEG-EMG and Ak- standards of care for the increas- of German Neurologists grew of the mentally ill. After 1940, as the German Society for Neu- tuelle Neurologie) were founded ing number of neurologic pa- quickly, to 720. Many scientific brains from patients who were rology (DGN). In East Germany, around 1970 and the number and tients, despite budget cuts for papers from international authors murdered within the framework the legal successor to Gesellschaft size of German-language neu- medical care in Germany. The fu- were published in German. The of the so-called euthanasia pro- Deutscher Nervenärzte was the rology textbooks grew. ture development of neurology main topics of the annual meet- gram were made available to re- reconstituted Society for Psychia- and neurologic care will depend ings between 1916 and 1930 re- searchers. Since the end of World try and Neurology, in which neu- German Neurology: The 1980s on the success of research to flected the questions of the day: War II, German science has had rologists and psychiatrists re- Since the 1980s, the DGN has cure or postpone the handicaps shell shock (Kriegszitterer), brain to come to terms with this hor- mained in the same organization. promoted recognition that care of neurologic disease. This task trauma, neurosyphilis, epilepsy, rific component of its past, The recovery of neurologic care, for stroke was a critical need. Re- is an international one, and the and new diagnostic methods. It through trenchant historic re- education, and society was an out- cent research and the develop- DGN is open for international was in this period that Hans Berg- search and an appeal for forgive- standing achievement of the first ment of care facilities have led to collaboration. ■ er described the EEG, implemen- ness to the victims. The role two generations of neurologists the advent of a nationwide net- tation of which in clinical practice played by neuropathologic re- after the war. work of stroke units to provide DR. BRANDT is of the department was an international scientific search in Nazi Germany has Since then, German universi- primary care for stroke patients. of neurology at Humboldt- achievement. made clear to all that there can be ties continue to be among the This was only possible through University Berlin. no progress without morality and most important institutions for the installation of almost 400 de- DR. DEUSCHL is the chairman of The Dark Years humanity. neurologic research; our re- partments of neurology at large the department of neurology at, The advent of Nazism in 1933 searchers build global partner- and small community hospitals. Christian-Albrechts University, Kiel had a dramatic affect on scientif- After World War II ships, particularly in the United A second important advance (Germany), and also is president of ic leaders, morality, the quality of In 1945, German neurology States and Europe. Successful ex- was the development of inten- the German Society for Neurology. clinical care, and the science of reached a nadir in its history: The changes sponsored by the sive care neurology as a major fo- DR. KARENBERG is from the German neurology. For Jewish best neurologists had left the coun- Deutsche Forschungsgemein- cus of neurologic care. Many Institute for the History of neuroscientists, this was the be- try or had been murdered, some of schaft and other German funding university or large community Medicine and Medical Ethic at the ginning of the distressing history the remaining neurologists were organizations have allowed Ger- hospitals now have their own in- University of Cologne (Germany).

10 • WORLD NEUROLOGY WWW.WFNEUROLOGY.ORG • JULY 2008

HIGHLIGHTS FROM THE JOURNAL OF NEUROLOGICAL SCIENCES: Dengue Fever and the Central Nervous System

BY ALEX TSELIS, M.D., PH.D. a small number also showed incoordina- “In 2008, there were more than 120,000 rologists must be familiar with the poten- tion, Babinski signs, hemiparesis, and dengue cases registered in Brazil, with tial neurological manifestations.” ■ engue fever (DF) is an acute febrile ill- nuchal rigidity. More than half of the 13 more than 600 cases of dengue hemor- ness found in tropical and subtropi- patients who had their cerebrospinal fluid rhagic fever and almost 50 deaths. [Con- DR. TSELIS is an associate professor of Dcal parts of the world. It is (CSF) tested had positive sidering the fact that,] during epidemics, neurology at Wayne State University, Detroit. caused by a mosquito-borne fla- polymerase chain reactions many cases of patients with neurological He is the Reviews and Book Reviews Editor vivirus, and characterized by for viral RNA. In three, the involvement are expected to be seen, neu- for the Journal of the Neurological Sciences. high fevers, headaches, rash, and CSF PCR was positive while joint pains. So why does it con- the blood was negative (J. cern neurologists? Neurosci. 2008;267:36-40). Facts About Dengue Fever In years past, DF resembled The authors emphasize severe influenza, in which what isn’t known about DF, engue fever is carried by Aedes mos- cases rose from 46,458 in Southeast Asia prostration was thought to be such as whether there are Dquitoes. They had been suppressed, in 1986 to 218,821 cases in 1998. By 2006, part and parcel of the general characteristic findings on in the past, as a result of campaigns to that number had risen to 188,684, with “toxic” effect. Physicians now DR. R.B. imaging and the pathogene- eradicate yellow fever, which is caused an overall upward trend since 2003. realize that many patients are DOMINGUES sis of the neurologic compli- by another flavivirus. However, for var- More recently, new and more malig- frankly encephalopathic, cations. It also is not known ious reasons, these campaigns are no nant forms of DF have emerged, in which raised the question: Does the virus whether a direct viral infection of neurons longer pursued with the same vigor. which multiple organ failures and he- invade the brain and cause encephalitis? occurs or if there is an immunopatho- And with changes in populations and morrhagic manifestations have as- Dr. R.B. Domingues and colleagues genetic mechanism, such as acute parain- climate, the opportunity for further sumed a prominent role. These are evaluated 85 consecutive adults with fectious demyelination. spread of the disease increases. known as dengue shock syndrome dengue fever during the 2002-2003 sum- Contacted by WORLD NEUROLOGY, Dr. The incidence of dengue fever is sig- (DSS) and dengue hemorrhagic fever mer season dengue epidemic in Brazil. Domingues of the department of pathol- nificant and growing considerably. In (DHF). While DSS and DHF probably They found 18 (21%) with prominent neu- ogy at the Escola Superior de Ciências da 2005, the Pan American Health Organi- occurred sporadically during dengue rologic findings not attributable to other Saúde de Vitória (EMESCAM), Vitória zation reported 427,627 cases in the epidemics in the past, these have conditions. (Brazil), noted, “Dengue virus infection is Americas, and the World Health Orga- formed a substantial proportion of the Most had changes in mental status, and, a very common disease in the developing nization reported that the number of total number of cases. usually, confusion rather than drowsiness; world. New Study Will Combat Stigma of Epilepsy in Zambia

BY DENISE NAPOLI plans to set up a “somewhat bogus” week- Elsevier Global Medical News long intervention program, where the teachers will learn about the disease from r. Gretchen Birbeck first became an educator they have never met. At week’s Daware of the pervasive stigma experi- end, “the teachers will find out that this per- enced by Zambians with epilepsy in 1994, son they’ve been working with all week ac- while working in a clinic in the small, tually has epilepsy.” She hopes that devel- southern African country. People with oping a bond with the educator, perhaps epilepsy would come in for treatment all more than the lessons themselves, will the time—for a burn or another injury ex- break the teachers of discriminatory habits. perienced during a seizure—but not for Another example involves Zambia’s tra- the disease itself. Having epilepsy was not ditional healers. Dr. Birbeck’s previous re- something to be publicized, even to health search showed that one of the factors in care professionals. their discrimination against epilepsy pa- IRBECK

Many epilepsy patients around the B tients is whether they believe the disease is globe complain of stigma associated with medical or the result of witchcraft. She

the disease. In the western world, “Data RETCHEN hopes to teach healers the scientific causes . G

... strongly suggest that the stigma peo- R of epilepsy. ple with epilepsy have is often self-in- D Intervention in households, however, flicted. They have life limitations that will be more difficult, and will not be a fo- OURTESY they’ve often self-imposed,” said Dr. Bir- C cus of the present study. According to Dr. beck. But in Zambia, the limitations ex- From left to right: Dr. Alan Haworth, Henry Kansembe, Dr. Elwyn Chomba, Edward Birbeck, after her last study, “I don’t think perienced by epileptics are especially dra- Mbewe, Dr. Gretchen Birbeck, Dr. Masharip Atadzhanov, Dr. Phillimon Ndubani. we understand that dynamic well enough matic. And very real. to know.” For example, “People with epilepsy are against epileptic children by their own beck, will implement social, educational, For example, she said, sometimes it is very disadvantaged in education and em- parents. In a comparison of epileptic chil- and economic interventions that her team not uncommon for parents of children ployment but also in terms of marriage,” drens’ food intake and nonaffected chil- developed after analysis of the causes of with epilepsy to elect not to send their said Dr. Birbeck. It’s difficult to find a drens’ rations across households of simi- stigma and discrimination in her last study. child to school. “Sometimes it’s because partner, but perhaps even more troubling lar means, “relative to the child in the The interventions will be specifically tar- they don’t want to waste money” on a is that even after marriage, “the women, other household, the child in the house- geted to each of several “power groups”— child who, they presume, won’t amount to especially, were abandoned.” Findings hold with epilepsy has food deprivation,” those people with the most influence in so- much. from an observational study conducted by said Dr. Birbeck, associate professor and di- ciety. “We know that health care workers, But “sometimes it’s because they’re Dr. Birbeck and her colleagues from 2003- rector of the International Neurologic clerics, police officers, and teachers propa- worried the child isn’t safe at school.” In 2007 also revealed that single women with and Psychiatric program at gate [the stigma against epilepsy]. But those both cases, the child loses, but they are epilepsy felt vulnerable to sexual assault, Michigan State University. are also the individuals who could have the “very different motivating factors. We which prevented them from seeking Now Dr. Birbeck and her colleagues from largest positive impact,” said Dr. Birbeck. need to understand that better, so we can health care—and not unduly (Lancet Neu- Michigan State, the University of Zambia, In a targeted approach to overcoming dis- intervene,” said Dr. Birbeck. rol. 2007;6:39-44). “When we did our and several Zambian health care institu- crimination by teachers, Dr. Birbeck will Her upcoming study will be funded quantitive assessment, the rate for rape for tions will have an opportunity to affect employ the observation made in her pre- with a $1.38 million grant from the Na- epilepsy was 20%, compared to less than some of that stigma faced by Zambians vious study that teachers who personally tional Institute of Neurological Disorders 3% in our comparison group.” with epilepsy. The Epilepsy-Associated Stig- knew someone with epilepsy were less like- and Stroke of the National Institutes of There is even evidence of discrimination ma in Zambia study, to be led by Dr. Bir- ly to stigmatize other epileptics. Dr. Birbeck Health. ■ JULY 2008 • WWW.WFNEUROLOGY.ORG WORLD NEUROLOGY • 11

NEUROLOGIC PEARL WFN Now Accepting The Ramsay Hunt Syndromes Nominations for Two

BY MARK HALLETT, M.D. started using Dr. Hunt’s name to de- Ramsay Hunt syndrome type III New Medals Editor in Chief scribe patients with these symp- can be described as a compression toms. The use of Ramsay Hunt syn- neuropathy of the deep palmar he Trustees of the World Federation of Neurology or reasons that are not always drome to describe this disorder is branch of the ulnar nerve from an Thave decided to establish two new WFN medals, one clear, sometimes people’s confusing since there are so many occupational cause (Hunt JR. Oc- for Service to Neurology and one for Achievement in F names get attached to neuro- etiologies for this syndrome (Hunt cupational neuritis of the deep pal- Neurology. logic syndromes. One of the cham- JR. Dyssynergia cerebellaris my- mar branch of the ulnar nerve: a Each award will carry an honorarium of $5,000. pions in this regard is James Ramsay oclonica—primary atrophy of the well defined clinical type of profes- Nominations, which may be made either by World Fed- Hunt, who has at least four syn- dentate system. A contribution to sional palsy of the hand. [J. Nerv. eration of Neurology member societies or by individ- dromes named after him. Perhaps he the and symptomatol- Ment. Dis. 1908;35:673-89]). ual members of a member society, should be seconded gained this recognition because of ogy of the cerebellum. [Brain Finally, Ramsay Hunt syndrome by at least five neurologists. the superb detail of both his clinical 1921;44:490-538]). type IV, which is also called Ramsay Three of these individuals should be from other reports and correlative pathological Ramsay Hunt syndrome II, also Hunt paralysis, is a form of juvenile WFN member societies. studies. called Herpes zoster oticus, is a her- Parkinson disease with the patho- The nominated individual should have approved the The four Ramsay Hunt syndromes pes zoster infection of the geniculate logical substrate of neuronal de- nomination and the principal proposer should write a are sufficiently confusing that they ganglion with pain in the ear, herpetic generation in the globus pallidus. citation of no more than 300 words in support of the are now designated by type. blisters of the skin of the ear canal or Juvenile Parkinson disease had been nomination. Hopefully, the following list will auricle, and facial paralysis. There described, but at the time of this ar- The Medal Committee will be made up of the cur- help keep these often-perplexing can also be dysfunction of the senso- ticle there had not been any prior rent president of the WFN and two previous World Fed- syndromes straight. ry portion of the seventh nerve and neuropathologic correlation (Hunt eration of Neurology presidents or World Federation Ramsay Hunt syndrome I is a dysfunction of the eighth nerve with JR. Progressive atrophy of the of Neurology trustees of the president’s choice. progressive hereditary neurodegen- vertigo, hearing loss, and tinnitus. globus pallidus [primary atrophy of The first medals will be awarded during the 2009 erative disease characterized by This was an original description, and the pallidal system]. A system dis- World Congress of Neurology in Bangkok (Thailand). ataxia, myoclonus, seizures, and it is called type II even though its de- ease of the paralysis agitans type, All nominations should be sent to: cognitive decline. scription preceded that of type I by 14 characterized by atrophy of the The Medal Committee Dr. Hunt believed that he was de- years (Hunt JR. On herpetic inflam- motor cells of the corpus striatum. c/o the World Federation of Neurology London Office scribing cases similar to those de- mations of the geniculate ganglion: a A contribution to the functions of 12 Chandos St., London, W1G 9DR, United Kingdom scribed before him by Unverricht new syndrome and its complications. the corpus striatum. [Brain 1917; and Lundborg, but some people [J. Nerv. Ment. Dis. 1907;34:73-96]). 40:58-148]). ■ Nominations must arrive by October 10, 2008. ■

Organized under the aegis of: Asian Oceanian Association of Neurology (AOAN), Indian Academy of Neurology (IAN) World Federation of Neurology (WFN) Delhi Neurological Association (DNA) In association with Association of Indian Neurologists in America (AINA) Pre-Conference workshops on EEG, Multiple Sclerosis, Movement Disorder & Advocacy October 22, 2008 (Wednesday) Main Conference October 23-26, 2008 (Thursday-Sunday) CME Accreditation The 12th Asian Oceanian Congress of Neurology (AOCN 2008) & 16th Annual Conference of the Indian Academy of Neurology (IANCON 2008) have applied to Delhi Medical Council for accreditation of CME hours for the AOCN - IANCON Workshops and the Main Conference.

Venue: The Ashok Hotel, New Delhi, India Conference Secretariat – AOCN – IANCON 2008 For Further details please log onto our website: Dr. M. M. Mehndiratta, Organizing Secretary Professor of Neurology, Department of Neurology www.aocn2008.com Room No. 502, Academic Block, G B Pant Hospital, New Delhi-110002, India or email us at : Ph: +91-11-23232742 Telefax: +91-11-23234350 (fax accepted after 6 ring) E-mail: [email protected] Website: www.aocn2008.com, www.ianindia.com [email protected] 12 • WORLD NEUROLOGY WWW.WFNEUROLOGY.ORG • JULY 2008

PROFILES IN NEUROLOGY My Practice in Bangkok, Thailand

mon in our region, have become his issue’s column takes us to Bangkok, Thailand, and the practice less common nowadays because Tof Dr. Rawiphan Witoonpanich, who is a neurologist and associate people are more aware of hygiene professor and consultant neurologist in a medical school. Dr. Witoon- and health maintenance. Infec- panich obtained her medical degree from Ramathibodi Hospital in tions that we used to see quite of- Thailand, where she began her training. She had further training in ten were pyogenic meningitis, vi- England, where she worked at Newcastle General Hospital, St. ral meningoencephalitis, tuber- Thomas’ Hospital, and the National Hospital for Neurology and Neu- culous meningitis, cryptococcal rosurgery. Dr. Witoonpanich specializes in neurophysiology and has ex- meningitis, neurocysticercosis, pertise in electromyography (EMG). Dr. Witoonpanich is also interested eosinophilic meningitis from An- in neuromuscular disorders, particularly myasthenia gravis. She cur- giostrongylus cantonensis, eosino- rently teaches at Mahidol University, Bangkok, and is on the faculty of philic meningoencephalitis caused medicine at Ramathibodi Hospital. by Gnathostoma spinigirum, and OSPITAL

neurosyphilis. However, oppor- H tunistic infections such as tuber- y job consists of teach- I see a wide variety of patients culosis, cryptococcosis, and toxo- AMATHIBODI

ing, patient care, and re- because we do not have enough plasmosis are still prevalent R Msearch. I teach medical attending staff doctors for every- among patients with AIDS. Dr. Witoonpanich examines a patient with Kennedy’s disease at students and neurology resi- one to see only patients of one’s There are also occasional out- Ramathibodi Hospital as three neurology residents look on. dents and look after outpatients own subspecialty. In recent years, breaks of food-borne botulism. and inpatients. I also perform apart from ordinary epilepsy and The largest occurred 2 years ago diseases, and the clinical applica- weekend in private hospitals. and teach EMG. stroke, I have seen more motor and affected more than 100 peo- tion of EMG. This combination of hard work I enjoy teaching and I always neuron disease, especially amy- ple. The implicated food was im- In the past, I have served as pres- together with family commit- participate in academic activities, otrophic lateral sclerosis, multi- properly processed home-canned ident of the Neurological Society ment means there is not much which are essential parts of resi- ple sclerosis, and Alzheimer’s dis- bamboo shoots. of Thailand and organized a few time left for each individual to re- dency training. I tend to spend ease. There are opportunities to do international meetings, including lax or exercise. I am no longer in much time talking to and exam- In the field of neuromuscular clinical research here in Bangkok. the Asian and Oceanian Sympo- private practice now and, as a re- ining a patient to make an accu- disorders, we have many patients Research is encouraged but not sium on Clinical Neurophysiology sult, I am able to spare 3 evenings rate clinical diagnosis before send- with Guillain-Barré syndrome, compulsory in our institution; in February 2005 in Chiang Mai, a week for exercise, usually last- ing the patient for various tests. I myasthenia gravis, and inflamma- however, academic positions and also in Thailand. I am a member ing 2 hours at a time. am rather obsessive about this tory myopathy. Other common promotions depend on the of the executive committee of the I devote most of my time to clinical approach and try to em- diseases include diabetic polyneu- amount of research completed, Asian and Oceanian Myology teaching and taking care of pa- phasize this in my teaching. ropathy, chronic inflammatory de- having a certain number of pub- Center and president of the Asian tients, with some hours spared Communicating with the pa- myelinating polyneuropathy, con- lications and book chapters. and Oceanian Chapter, as well as for research. I insist on giving pa- tient can, to a certain extent, pro- nective tissue diseases with There is some limitation on ad- a member of the rule committee tients plenty of time so I can tect a physician from being sued mononeuropathy multiplex, mus- vanced research because of a of the International Federation of make an accurate clinical diag- as well. Being better educated cular dystrophy, thyrotoxic my- shortage of trained personnel Clinical Neurophysiology. nosis, provide good care, and es- and increasingly health con- opathy, periodic paralysis, and mi- and funding. I have one or two The salary of a government tablish a good doctor-patient scious, Thai people have a grow- tochondrial myopathy. half-days a week spared for my officer is quite low here. There- rapport. I enjoy my profession- ing tendency to sue their doctors Central nervous system infec- research, which is mainly on fore, most doctors have to prac- al life and try to relax and exer- for malpractice. tions, which used to be very com- myasthenia gravis, some muscle tice in the evening and over the cise regularly. ■ Nelly Chiofalo, M.D., 1929-2008

BY OSVALDO OLIVARES, M.D. advanced the field by organizing profes- in the professional development of her col- AND RENATO VERDUGO, M.D. sionals dedicated to electroencephalogra- laborators. She was always searching for phy, electromyography, evoked potentials, educational opportunities for Latin Amer- r. Nelly Chiofalo died in Santiago, Chile, and sleep disorders. Her concern for the ican neurophysiologists, encouraging Don April 18, 2008. Dr. Chiofalo was one specialty was evinced by her successful ef- them to apply for scholarships and awards, of the pioneers in the development of clin- forts in Chile for the professional develop- and urging them to improve their foreign ical neurophysiology in Latin America. ment and continuing education of electro- language performance. Born on March 24, 1929, in Mendoza, physiology personnel. Through the society, Dr. Chiofalo was married to Dr. Luciano Argentina, Dr. Chiofalo studied medicine at she promoted the creation of the Latin Basauri, a prestigious neurosurgeon. The the National University of Cordoba, Ar- American chapter of the International Fed- two were unstoppable travelers, visiting gentina, where she obtained her medical eration of Clinical Neurophysiology every continent, including Antarctica, and degree in 1954. In 1956, she moved to Chile (IFCN), founded in Guatemala during a climbing the Himalayas. He preceded her and gained admission into the Instituto de symposium she organized in 1995. The in this final trip by only 2 months. IBILS

Neurocirugía. Working with Professor Car- C statutes of the chapter were approved by The couple is survived by their two sons, los Villavicencio, she studied neurology the IFCN in Buenos Aires in 2001. Cristian, an engineer, and Rodrigo, a pub- ANIEL and electroencephalography there until D In 1977, Dr. Chiofalo organized a pio- licist; and eight grandchildren, two of 1959. In 1960, she continued her education Dr. Nelly Chiofalo died on April 18, 2008. neering workshop on brain death in Chile, whom are in medical school. in electroencephalography at Harvard the conclusions of which were the basis for Latin American neurologists and neuro- Medical School, Boston, and graduated in training for a team of neurosurgeons and a transplant law—at a time when the sub- physiologists acknowledge her importance 1961. She returned to the Instituto de Neu- neurologists to develop epileptic surgery. In ject was still controversial in Latin Ameri- in the development of the field in our rocirugía, also assuming a leading position 1978, the International League Against ca and the world. countries, and will long remember her and in the Chilean League Against Epilepsy. Epilepsy gave her the title of ambassador, During 1978 and 1979, she carried out miss her. ■ In the late 1970s, Dr. Chiofalo became which allowed her to organize symposia the first epidemiologic studies on epilepsy the first person in Chile to develop tech- and congresses. In the late 1980s, after 4 in Chile. Her works on the electroen- DR. OLIVARES is from the neurology unit at niques to measure blood levels of years as president of the league, she de- cephalographic abnormalities in the Hospital San José, at the Universidad de antiepileptic drugs. She also introduced veloped the study of sleep disorders and in- Creutzfeldt-Jakob disease are considered Santiago (Chile). DR. VERDUGO is an continuous electroencephalographic mon- troduced polysomnography to the country. classic. In addition, Dr. Chiofalo published associate professor of neurology on the itoring and, supported by Professor Jean The Chilean Society of Clinical Neuro- several papers on pediatric epilepsy. faculty of medicine, Universidad de Chile, in Bancaud of Sainte-Anne in Paris, secured physiology, which Dr. Chiofalo founded, Dr. Chiofalo showed enduring interest Santiago. JULY 2008 • WWW.WFNEUROLOGY.ORG WORLD NEUROLOGY • 13

Hereditary Neuroblastoma With Aura Linked Tied to ALK Gene Mutations To Increased CVD Risk BY PATRICE WENDLING cantly greater risk of MI (HR Three novel anaplastic lymphoma kinase somatic mutations in the ALK Elsevier Global Medical News 1.64), compared with those re- gene can occur during evolu- porting one migraine per month germline variations are identified. tion to the high-risk form of the C HICAGO — Migraine fre- (HR 0.94) or at least weekly mi- disease. The notion that ac- quency appears to be an indica- graine (HR 1.49). BY LAUREN SCOTT The investigators performed quired mutations of ALK are tor of increased risk of cardio- Women with the highest mi- ZOELLER genomewide linkage studies us- likely to activate oncogenes such vascular disease in migraineurs graine frequency had significant- Elsevier Global Medical News ing a panel of approximately as BRAF is consistent with the with aura, according to findings ly increased risk of stroke (HR 6,000 single nucleotide poly- two-hit model of oncogenesis. from a large cohort analysis. 2.74), although a nonsignificant C HICAGO — Mutations with- morphisms (SNPs). This pro- In a panel of 491 tumors as- The study, which used data increase was also seen among the in the anaplastic lymphoma ki- duced evidence of linkage to a sessed, 112 had focal unbalanced from the Women’s Health Study lowest frequency group (HR nase gene are associated with 16.1 megabase region of c2p23- gains of ALK, 16 of which had involving 27,798 women with no 1.44). The finding of increased hereditary neuroblastoma, and 24 (log score = 4.23) containing high levels of ALK amplifica- history of cardiovascular disease, stroke risk in high-frequency mi- acquired ALK mutations may 104 genes, including ALK and tion. ALK gain or amplification identified a mixed association be- graine patients is consistent with be related to the development of MYCN. (MYCN amplification is was associated with both high- tween migraine frequency and previous studies, he said. nonfamilial neuroblastoma, re- associated with poor prognosis risk disease (P less than .0001) major cerebrovascular disease. When analyzed based on aura searchers reported. in neuroblastoma.) and increased disease-related Compared with women with- status, the risk for stroke was sig- These findings have impor- Recombination events were mortality (P = .0003). out migraine, women with at nificantly increased in the high- tant implications in terms of ge- then mapped to this region to A resequencing of the tyro- least weekly were al- and low-frequency migraine netic screening and genetic determine potential neuroblas- sine kinase domain of ALK in most three times more likely to groups with aura, compared with counseling, and provide a novel toma predisposition genes. Ge- 167 of the 491 primary neurob- experience a stroke. Those with their counterparts without aura. target for the development of netic and pedigree analysis lastoma tumor samples showed migraines less than once a month The risk for MI was significantly therapies that disrupt ALK sig- (based on a mean of four indi- that 24 of them had ALK muta- were one-and-a-half times more increased in the low-frequency naling, according to Dr. Yael P. viduals with neuroblastoma per tions, including eight distinct likely to have a heart attack, Dr. group, compared with their Mosse, a pediatrician at the Chil- family) indicated that heritable single-base missense substitu- Tobias Kurth reported. counterparts without aura. dren’s Hospital of Philadelphia, mutations of the ALK tyrosine tions. Of nine tumors matched However, the increased risk of An audience member observed and her coinvestigators. kinase domain (exons 21-28) for germline DNA, eight had ac- disease according to migraine fre- that patients with low migraine The vast majority of familial were present in 8 of the 10 fam- quired and one had germline quency was apparent only for frequency were still at increased neuroblastomas are associated ilies studied. mutations of ALK. migraineurs with aura. risk for stroke, suggesting that with mutations in the tyrosine Three novel variations of ALK Further gene knockdown ex- “The clinical implications of migraine prevention may actual- kinase domain of ALK, which is germline mutations were iden- periments using small interfering our data at this point are unclear,” ly make things worse. located on chromosome 2p24- tified: R1275Q (P = .91), which RNA techniques showed a 40%- Dr. Kurth said at the annual meet- Dr. Kurth said the study raises 23, said Dr. Mosse at the annual activates the BRAF oncogene 80% inhibition of ALK mutated ing of the American Academy of many questions and that further meeting of the American Soci- and other protein kinases; or amplified neuroblastoma cell Neurology. “The strongest evi- research is needed to validate or ety of Clinical Oncology. G1128A (P = .95), which also ac- lines. Results suggested multiple dence from our study and others refute its findings. Earlier in the Mutations of the ALK tyro- tivates BRAF; and R1192P (P = mechanisms of ALK activation. is that migraine without aura is presentation, he cautioned that sine kinase domain have also .98), which has an undetermined Furthermore, the vast majority not associated with increased risk the data do not support the con- been shown to have oncogenic function. of somatically acquired ALK of cardiovascular disease. Future clusion that migraine prophylax- functions in several other can- Neuroblastoma is passed on mutations fell within mutation- studies should focus on identify- is will affect the risk of CVD. cers and are involved in trans- through the germline in only al “hot spots” observed in other ing patients with migraine and Audience members also ques- formation, typically via translo- 1% of the children affected, cancers (for example, MET, aura who are at particular in- tioned Dr. Kurth about the fact cation events, she noted. however. ERBB2, and EGFR kinase). creased risk of CVD.” that migraine status was defined ALK plays a role in regulating Therefore, the investigators Dr. Brian Kushner, a pediatric At the start of the study, 3,568 based on a series of questions the development of the central sought to determine the role of oncologist specializing in neu- women (mean age 55 years) had rather than through standardized and peripheral nervous systems, ALK mutations in somatically roblastoma at Memorial Sloan- migraine based on self-reported definitions. He acknowledged and is a highly conserved or- acquired neuroblastoma. Kettering Cancer Center, New questionnaires, of which 1,468 that the study’s classification of phan receptor. They speculated that acquired York, discussed the study, saying (41%) women reported had aura. migraines was “problematic,” that “ALK is a very important Overall, 2,315 women report- but noted that only those pa- marker in these familial neu- ed migraine less than once a tients who checked a box for aura DR. CHOPRA WINS AWARD roblastomas and a promising month, 1,073 reported one mi- were counted in that subgroup, target for therapy.” graine per month, and 180 re- suggesting that any misclassifica- In terms of developing new ported at least weekly migraines. tion potentially could have un- ALK-targeted drugs, Dr. Mosse After an average of 12 years derestimated the associations ob- said, “Fortunately, although follow-up, there were 706 cere- served in the study. neuroblastoma is a rare disease, brovascular events, 305 heart at- Finally, the pivotal question we are not starting from tacks, and 310 ischemic strokes. from the audience was whether it scratch. ALK is an oncogene In a multivariate analysis that is aura frequency rather than mi- EWS N which is implicated in other hu- adjusted for age, hypertension, graine frequency that matters, as man cancers, such as anaplastic smoking status, body mass in- only migraineurs with aura had IBERTY

/L large cell lymphoma. So there dex, total cholesterol, and histo- significantly increased disease risk NDIA I are many pharmaceutical com- ry of myocardial infarction, the when compared with migraineurs HOPRA C panies who are very interested risk for major CVD was increased without aura. “I agree with you

AGJIT in developing ALK inhibitors; among those with at least week- that it’s the aura and not the fre- . J

R some have actually developed ly migraines (hazard ratio 1.90), quency,” Dr. Kurth said. OVERNMENT OF D

/G ALK programs.” as compared with women with The study was supported by ALK is homologous to other migraine less than once a month grants from the Donald W. OURTESY ICTURES

C P kinases such as MET, an in- (HR 1.54) and those with one Reynolds Foundation, the Leducq Dr. Jagjit Chopra was awarded the Padma Bhushan, hibitor of which is now being migraine per month (HR 0.97). Foundation, and the Doris Duke one of the highest civilian awards in India, by the tested in phase I studies in A different emphasis emerged Charitable Foundation. Dr. Kurth President of India Mrs. Pratibha Patil on May 5, 2008. adults, she added. Researchers for risk of ischemic stroke and MI, disclosed relationships with the Dr. Chopra is the only Indian neurologist to receive working in Dr. Mosse’s labora- said Dr. Kurth of Brigham and National Institutes of Health, this award, which was given for Service to Medicine tory have cloned all of the key Women’s Hospital and Harvard Bayer AG, McNeil Consumer & and Neuroscience. Dr. Chopra served as Editor in ALK mutations they discovered, Medical School, both in Boston. Specialty Pharmaceuticals, Wyeth Chief of WORLD NEUROLOGY for the last decade. and are performing ongoing Women with the lowest mi- Consumer Healthcare, and i3 transformation assays. ■ graine frequency were at signifi- Drug Safety. ■ 14 • WORLD NEUROLOGY WWW.WFNEUROLOGY.ORG • JULY 2008

Calendar of International Events

2008 6th International Conference on 6th International Congress on 8th European Congress on Epileptology Frontotemporal Dementia Autoimmunity Sept. 21-25, 2008; Berlin NeuSIG Satellite to the Glasgow 2008 Sept. 3-5, 2008; Rotterdam, The Sept. 10-14, 2008; Porto, Portugal www.epilepsyberlin2008.org Netherlands www.kenes.com/autoimmunity World Congress on Pain 5th World Congress for www.ftd2008.org/site August 13-15, 2008; London World Congress on Treatment and NeuroRehabilitation www.kenes.com/neuropathic2008 6th International Congress on Research in Multiple Sclerosis Sept. 24-27, 2008; Brasília, Brazil Meningiomas and Cerebral Venous System (ACTRIMS+ECTRIMS+LACTRIMS) www.sarah.br/wfnr-rio2008 XXIII Congresso Brasileiro de Sept. 17-20, 2008; Neurologia Sept. 3-7, 2008; Boston www.msmontreal.org 6th World Stroke Congress August 16-21, 2008; Belém, Brazil www.themeningiomaconference2008.org Sept. 24-27, 2008; Vienna www.neuro2008.com.br 7th Mediterranean Congress of Physical www.kenes.com/stroke2008 European Headache and Migraine Trust and Rehabilitation Medicine 12th World Congress on Pain International Congress 2008 Sept. 18-21, 2008; Portorose, Slovenia XXVII Annual Congress of the European August 17-22, 2008; Glasgow, Scotland Sept. 4-7, 2008; London www.medcongress.prm08.org Society of Regional Anaesthesia and www.iasp-pain.org www.ehmticongress2008.com Pain Therapy Xth International Symposium on 12th Congress of the European 14th World Congress of Sept. 24-27, 2008; Genoa, Italy Psychophysiology Thrombolysis and Acute Stroke Therapy www.kenes.com/esra Federation of Neurological Societies Sept. 21-23, 2008; Budapest, Hungary August 23-26, 2008; Madrid Sept. 8-13, 2008; St. Petersburg, Russia 36th Annual Meeting of the www.world-psychophysiology.org/iop2008 www.kenes.com/tast2008 www.kenes.com/efns2008 International Society for Paediatric Neurosurgery (ISPN) Oct. 12-16, 2008; Cape Town, South Africa www.ispn2008.org 6th International Congress on Mental Dysfunctions and Other Non-Motor Features in Parkinson’s Disease Oct. 16-19, 2008; Dresden, Germany www.kenes.com/pdment2008 Dystonia Europe 2008 Oct. 17-19, 2008; Hamburg, Germany www.dystonia-europe-2008.org Before the researcharch 2nd World Congress on Controversies in Neurology is published... Oct. 23-26, 2008; Athens www.comtecmed.com/cony 9th International Congress of NeuroImmunology Oct. 26-30, 2008; Fort Worth, Texas www.isni2008.org Before the drug 19th International Symposium on ALS/MND is approved... Nov. 3-5, 2008; Birmingham, England www.mndassociation.org Child Neurology Society Annual Meeting Nov. 5-8, 2008; Santa Clara, Calif. www.childneurologysociety.org/annual_ meeting/accomodations Before the guidelineeline Neuroscience 2008 is issued... Nov. 15-19, 2008; Washington www.sfn.org/am2008

2009

2009 AAN Regional Conference Jan. 16-18, 2009; Orlando, Fla. You read www.aan.com/go/education/conferences 7th International Symposium of Asian and Pacific Parkinson’s Association it first in (APPA) Feb. 15-16, 2009; New Delhi www.aopmcindia.com/appa/appa_ invitation.html 2nd Asian and Oceanian Parkinson’s Disease and Movement Disorders Congress (AOPMC) Feb. 15-17, 2009; New Delhi THE LEADER www.aopmcindia.com IN NEWS 2nd European Brain Policy Forum AND Feb. 25-26, 2009; Brussels G www.kenes.com/ebpf2009 MEETINGE COVERA 9th International Conference on Clinical Neurology News Alzheimer’s and Parkinson’s Diseases: Advances, Concepts and New Challenges — We Write Medicine’s First Draft — March 11-15, 2009; Prague www.kenes.com/adpd www.clinicalneurologynews.com JULY 2008 • WWW.WFNEUROLOGY.ORG WORLD NEUROLOGY • 15

The Federation of European Neurological Societies

The EFNS supports the European logic societies. If your organization would The EFNS has expanded its activities, further Brain Council (EBC), which promotes like to work with us in our efforts to pro- promoting neurologic research and training. neurologic research programs within the mote the importance of neurologic re- European Union. The council, launched search to the World Health Organization, by Jes Olesen, M.D., is supported in its please contact the EFNS head office, Breite BY JACQUES L. DE REUCK The EFNS has traditionally organized brain research initiatives by various sci- Gasse 4-8, A-1070 Vienna, Austria, or e- European Regional Director of the WFN randomized clinical trials in Eastern Eu- entific societies; pharmaceutical compa- mail us at [email protected]. Our Web rope, where neurologic departments are nies; and the European Federation of site is www.efns.org. ■ he European Federation of Neuro- poorly developed and neurologists have Neurological Associations (EFNA), a pa- logical Societies (EFNS), which rep- difficulties participating in scientific sem- tient organization. The European Federation of Tresents 42 countries, has successful- inars outside their countries. The successful collaboration between Neurological Societies ly extended its international cooperation This program has been very successful the EFNS and the EFNA is demonstrated across the Mediterranean. for many years. by EFNA’s representation at EFNS con- Breite Gasse 4-8 The resulting collaboration among Eu- One of the EFNS’s main commitments gresses, where they organize sessions for A-1070 Vienna, Austria ropean, African, and Arabic countries will is to help expand neurologic departments patients. [email protected]. benefit us all as neurologists and advocates and to increase the quality of neuroscience The EFNS also supports international www.efns.org for the specialty. research within and outside of Europe. collaboration among established neuro- The EFNS has adopted as associated members the adjoining neurologic soci- eties of the Mediterranean basin: the Let These Images of Thailand Entice You to Join Us in new member so- cieties include those from Mo- Bangkok for the 2009 World Congress of Neurology rocco, Algeria, Tunisia, Libya, Learn more at: www.wcn2009bangkok.com Egypt, Lebanon, Syria, and Jordan. These societies now share the same practical ad- JACQUES L. vantages as those COM . COM DE REUCK in Europe. Neu- .

rologists in train- OTOLIA OTOLIA /F ing can participate in all the education and /F JIKGOE exchange programs of the EFNS, and are RSLER © eligible to apply for travel bursaries to par- E ticipate in EFNS congresses when their re- MITRY search is accepted for presentation. They ©D can apply for the department-to-depart- ment exchange program and participate in the EFNS Academy of Young Neurol- ogists. COM Starting in Madrid, Aug. 23-26, 2008, .

and at every subsequent congress, a spe- OTOLIA /F

cial Mediterranean session will be orga- CH _ nized with European and North African MACKY

participants speaking on a topic of com- © mon interest. EFNS scientists also have been invited as guest speakers to the congresses of the Pan Arabic Union of Neurological Soci- eties (PAUNS). At the 2007 PAUNS meet- ing held on Oct. 25-27 in Yasmine Ham- mamet, Tunisia, attendees organized a common session on peripheral neu- ropathies. At the 9th Cairo International Neurol- ogy Conference, held by the Egyptian Neurological Society on Feb. 13-15, 2008, a common PAUNS-EFNS session was on the program. The EFNS has joined WFN President Jo- han Aarli in his efforts to help develop de- partments of neurology at universities throughout Africa. Because the EFNS can only sponsor educational programs, it joined with the WFN and IBRO to organize the first African Regional Teaching Course (RTC) in Dakar, Senegal on June 26-28, 2008, for Senegalese neurologists and those from the surrounding French-speaking coun- ALLETT H tries. ARK

The local organizer was Gallo Diop, .M R Ph.D. The topics, selected by the African D colleagues, were peripheral neuropathies and neurodegenerative diseases.