B Y D I A N A M A H O N E Y

Else vier Global Medical Ne ws

B O S T O N — Patients withtreatment-resistant epilepsy cansignificantly reduce their fre-quency of seizures with the useof an implantable device thatdetects preseizure electrical ac-tivity and preemptively abortsseizures.

In a multicenter randomized

controlled trial, 191 patientswith medically intractable par-tial onset seizures were im-planted with the neurostimula-tor, and their seizures declinedby a mean of 29% during activestimulation with the device,compared with a 14% reduc-tion during sham activation, Dr.Martha J. Morrell reported atthe annual meeting of theAmerican Epilepsy Society.

In the later, open-label phaseof the study in which all of thepatients received the activestimulation, nearly half of the171 patients for whom 12weeks of data were availableexperienced at least a 50% re-duction in seizure frequencyrelative to baseline, said Dr.Morrell, clinical professor ofneurology at Stanford (Calif.)University and chief medical

officer of NeuroPace, develop-er of the Responsive Neu-rostimulator System (RNS).

The cranially implanted RNSdevice differs from convention-al, “open loop” brain stimula-tion technologies that involvethe scheduled delivery of elec-trical stimulation to specificbrain regions independent ofbrain activity.

“The RNS delivers stimula-

tion in response to a detectedevent,” said Dr. Morrell, notingthat the treatment is “individu-alized and dynamic” in that ituses computer technology torecognize and respond to pat-terns of brain activity specificto individual patients’ seizurepatterns.

The responsive neurostimu-

B Y V L A D I M I R

H A C H I N S K I , M . D.

President, WFN

Life is brain. It enables qual-ity of life and humanprogress. Without a healthy

brain, little else matters. TheWorld Federation of Neurology(WFN) aims to integrate, prior-itize, and help apply advances inbrain diseases and the promo-tion of brain health worldwide.The best way to ensure thisWFN role and future is to helpshape it. Continuity withoutquestioning risks obsolescence,and aimless change produces“big thunder, little rain,” ac-cording to a Chinese proverb.The best time to plan change isduring stable evolution.

The history of the WFN has been one ofsteady progress, particularly in the past 4years under the leadership of Johan Aarli.Upon meeting him, he strikes one as “a gen-tleman and a scholar.” He is dignified with-out being pompous, and informal withoutbeing familiar. His learning in the arts, lit-erature, and different cultures is impressive,and his manner engaging. His presidencyhas been enhanced and complemented by

his gracious wife Gullborg, who makeseveryone feel welcome and at ease.

When Johan became President of theWFN, I discovered other qualities—his abil-ity for careful planning and decisive action.I was impressed by how thoroughly heplanned his tenure, how he identified somedifficult problems, and how quickly and de-cisively he moved to resolve them. Membersof his team were encouraged to propose anddiscuss issues, but there was never any doubt

as to who made the final deci-sions. He has great moral au-thority through the silent elo-quence of his example. Hefostered a wonderful team spiritwhich, in part, explains why thepast 4 years were so remarkable.

Among his major accomplish-ments have been the Africa Ini-tiative; welcoming as a memberthe Chinese Neurological Soci-ety, the world’s largest, and sev-eral others to take membershipup to a record 110 societies; andmoving to a 2-year cycle for theworld congresses to allow us totake neurology to those who can-not afford intercontinental travel.Johan, a heartfelt thank you!

Some positions have changed,but most of the team remains in

place and will build on what has beenachieved. The pace of progress needs to beaccelerated further if we are to help stem thegrowing burden of neurological disorders.Already, they are the leading cause of dis-ability adjusted years in the world and theyare projected to rise.

Our agenda is huge, and our resources aremodest, so we need to integrate, focus, and

NeurologyWorld

VOL. 25 • NO. 1 • FEBRUARY 2010

Implant Short-Circuits Some Epileptic Seizures

A Blueprint for Continuity and ChangeT H E O F F I C I A L N E W S L E T T E R O F T H E W O R L D F E D E R A T I O N O F N E U R O L O G Y

See Seizures • page 8

I N S I D E

See Contiunity • page 4

Place your classified

advertisement today!WORLD NEUROLOGY now offers job advertisements to an international print readership of over

25,000 neurologists and to a much larger on-line readership through the Publicationssection of the World Federation of Neurology Web site at www.wfneurology.org.

For Europe, write primaryeurope-ads@elsevier.com or fax us on +44 (0)207 4244433

For U.S., contact Robert Zwick at r.zwick@elsevier.com or call 973-290-8226

SudanNeurologists hold thefirst formal day-longclinical skills course,aiming in future to makeit longer and to attractcandidates from theneighboring countries.PA G E 3

IndiaAt the 2nd EuropeanNeurological SocietySymposium in Chennai,speakers presented aseries of lectures and casediscussions on stroke,epilepsy, and neuopathies. PA G E 4

SpainTwo researchers outline recent discoveries in thegenetics of dementia andsuggest that investigatorsand clinicians prepare forthe ensuing technologicaland ethical challenges.PA G E 1 2

Dr. Vladimir Hachinski, the new President of the WFN, is shownhere in front of a flowchart depicting his organizational plan.

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2 • WORLD NEUROLOGY WWW.WFNEUROLOGY.ORG • FEBRUARY 2010

WORLD FEDERATION OF NEUROLOGYEditor in Chief Dr. Mark Hallett (U.S.A.)

EDITORIAL ADVISORY BOARDDr. Pierre Bill (South Africa); Dr. William M. Carroll (Australia); Dr. Jagjit S. Chopra (India); Dr. Michael Finkel (U.S.A.); Dr. Osvaldo Fustinoni (Argentina); Dr. Francesc Graus (Spain);Dr. Alla Guekht (Russia); Dr. Theodore Munsat (U.S.A.); Dr. Daniel Truong (U.S.A.); Dr. Alexandros Tselis (U.S.A.)

WFN OFFICERSPresident: Dr. Vladimir Hachinski (Canada)First Vice-President: Prof. Werner Hacke (Germany)Secretary-Treasurer General: Dr. Raad Shakir (United Kingdom)

ELECTED TRUSTEESDr. Gustavo Roman (U.S.A.); Dr. Ryuji Kaji (Japan); Dr WolfgangGrisold (Austria)

CO-OPTED TRUSTEESDr. Roger Rosenberg (U.S.A.); Dr. Niphon Poungvarin (Thailand)

REGIONAL DIRECTORSDr. Alfred K. Njamnshi (Pan Africa); Dr. Jacques De Reuck(Europe); Prof. Riadh Gouider (Pan Arab); Dr. Amado San Luis(Asian-Oceania); Dr. Robert Griggs (North America); Dr. AnaMercedes Robles de Hernandez (Latin America)

EXECUTIVE DIRECTORKeith NewtonWorld Federation of NeurologyHill House, Heron SquareRichmond, Surrey, TW9 1EP, UKTel: +44 (0) 208 439 9556/9557 Fax: +44 (0) 208 439 9499info@wfneurology.org

EDITOR OF THE JOURNAL OF THE NEUROLOGICAL SCIENCESDr. Robert Lisak (U.S.A.)

WORLDNEUROLO GY

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WORLD NEUROLOGY, an official publication of the World Federationof Neurology, provides reports from the leadership of the WFN, itsmember societies, neurologists around the globe, and news fromthe cutting edge of clinical neurology. Content for WORLD NEUROLOGY

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Disclaimer: The ideas and opinions expressed in WORLD NEUROLOGY

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©Copyright 2010, by the World Federation of Neurology

EDITOR IN CHIEF’S COLUMN

An Opportunity to JoinThe Global Community In this issue, the WFN

Junior Travelling Fel-lowship program for

2010 is announced. Wealso have reports from anumber of fellows whotook advantage of this

program in 2009 (see p. 10). This is clearly a fineprogram, and young neurologists from low orlower-middle income countries should be en-couraged to apply.

The first impression after reading over the fel-low reports is the sense of enjoyment. Goingto these large international meetings gives thefellows a sense of being part of a large enter-prise. They see persons giving lectures that theyhave only read about, they can learn about newtechniques for diagnosis and treatment. They

seem keen to employ their new knowledge intheir home environment. Many of them arealso participating in research activities and havean opportunity to present their findings and dis-cuss their work with other interested partici-pants and experts. This is also a critical experi-ence in keeping them motivated and increasingthe quality of their work.

It is also a great opportunity, of course, totravel and to experience a new city and coun-try. There is a big difference and a great distancebetween Addis Ababa and Florence. Interac-tions with neurologists from many places areimportant too. All this likely helps to increaseunderstanding and friendship in a world whereopportunities to connect are improving butrisks still abound because of a residual lack oftrust and cooperation between people. ■

BY MARK HALLETT, M.D.

2010 Junior Travelling Fellowships

This year, the World Feder-ation of Neurology is

again offering Junior Travel-ling Fellowships for 20 youngneurologists from countriesthat have been classified bythe World Bank as low- orlower-middle income to at-tend approved internationalmeetings.

Applicants should hold a postthat is not above that of associ-ate professor and they shouldbe no older than 42 years.

They are asked to send:� The name and dates of themeeting for which they wishto register;� A CV and bibliography;� A letter of recommenda-tion from the head of their de-partment; and� An estimate of expenses, toa maximum £1,000.

Those who are planning topresent a paper or poster atthe meeting they plan to at-tend should also include an

abstract with their application.The applications should be:

� sent to World Federation ofNeurology, Hill House, HeronSquare, Richmond, Surrey,TW9 1EP, United Kingdom;� e-mailed to info@wfneu-rology.org; or� faxed to +44 (0) 208-439-9499.

They should be received atthe WFN office by Friday,March 19, 2010. The fellow-ship awards will be announcedsoon thereafter. ■

Research Group Holds PDCourse at Bali Meeting

B Y D A N I E L T R U O N G, M . D.

Together with the Indone-sian Neurological Associa-tion, the World Federation

of Neurology Research Groupon Parkinsonism and RelatedDisorders organized a course inParkinsonism and movementdisorders in Bali, Indonesia, inNovember.

It ran concurrent with the an-nual meeting of the Indonesianassociation and was an initiativeto reach out to developing coun-tries spearheaded by me at therequest of Erik Wolters (theNetherlands), the chair of theResearch Group.

The faculty included expertssuch as Roongroj Bhidayasiri(Thailand), Mark Hallett, KatieKompoliti, Irene Litvan, DavidRiley, and myself, all of us from

the U.S.A., and Erik Wolters (theNetherlands). Topics includedParkinson disease and its man-agement, dystonia, tremor, pro-gressive supra- nuclear palsy, mul-tiple system atrophy, myoclonus,and chorea. As part of the pro-gram, 250 textbooks on move-ment disorders were distributed.

The faculty was impressedwith participants’ enthusiasm asreflected in the vibrant discus-sions. Faculty members werealso able to meet local neurolo-gists as well as visit the Univer-sity Hospital of Denpasar. ■

DR. TRUONG is head of theParkinson and MovementDisorder Institute at OrangeCoast Memorial Hospital inFountain Valley, Calif., U.S.A.,which he founded and where hepractices as a neurologist.

Nominations for WFN Medals

The WFN medals—for serviceto international neurology

and scientific achievement inneurology—will next be pre-sented at the 20th World Con-gress of Neurology in Mar-rakesh, Morocco, in 2011.

Nominations for these presti-gious awards are now invitedfrom WFN members, secondedby at least five neurologists, threeof whom should be from otherWFN member societies. Thenominee should have given hisor her consent to the nomina-

tion, and a citation of no morethan 300 words in support of thenomination should accompanythe proposal. Each award alsocarries an honorarium of $5,000.

Nominations must be markedfor the attention of the MedalCommittee, and sent c/o theWFN, Hill House, HeronSquare, Richmond, Surrey, TW91EP, United Kingdom; e-mailedto info@wfneurology. org; orfaxed, +44 (0) 208-439-9499. Theyshould arrive at the WFN Lon-don Office by April 16, 2010. ■

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FEBRUARY 2010 • WWW.WFNEUROLOGY.ORG WORLD NEUROLOGY • 3

FAREWELL NOTE

Facing the Challenges of a New DecadeAs the newly elected Presi-

dent of the World Feder-ation of Neurology,

Vladimir Hachinski is leading usinto a new decade; there is aworldwide and increasing needfor more and better neurologi-cal services. The new leader-ship is exceptionally well posi-tioned to meet these challenges.

Stroke is one of the many global health problems inwhich the voice of the WFN will be heard. One of thefirst research projects in the WFN was the compilationof a geographical pathology of cerebral vascular diseasein 1957. And stroke has never been more important thannow. The main mission of the WFN today is to reducethe global burden associated with all neurological dis-orders—a burden borne by people everywhere and of allages and every segment of society. It is the disproportionbetween the global burden associated with neurologicaldisorders and the available resources to deal with this bur-den that present a challenge for the WFN.

A Solid LeadershipLet me take this opportunity to thank Dr. Hachinski, Dr.Raad Shakir, the Secretary-Treasurer General of theWFN, and Prof. Werner Hacke, Dr. Ryuji Kaji, Dr.Niphon Poungvarin, Dr. Gustavo Roman, Dr. RogerRosenberg, and Dr. Marianne de Visser for their effortsand collaboration during the last 4 years. The WFN lead-ership is solid in its unanimous support to establish high-quality neurological services worldwide. My warmthanks also to the London-based WFN administration,led by Mr. Keith Newton, for its institutional memory,enduring loyalty, and hard work.

Global LinksThe WFN has a close collaboration with regional neu-rological associations. We are deeply indebted to theAmerican Academy of Neurology for its central role ininternational neurology training in the form of theAAN–CME (continuous medical education) program,which is given for free to developing countries. We havealso had the pleasure to be joined by the European Fed-eration of Neurological Societies (EFNS) in executingthe Africa Initiative, which we started in 2006. TheEFNS has established an important training programin neurology for sub-Saharan Africa, alternating be-tween French- and English-speaking countries

Two neurologists are especially important for theAfrica Initiative—Alfred K. Njamnshi from Cameroon,the Pan-African Regional Director, and Amadou Gal-lo Diop from Senegal, who coined the phrase, “WithAfrica, For Africa.” This has become the theme for the20th World Congress of Neurology in Marrakesh, Mo-rocco, in 2011, which will be the first WCN on theAfrican continent. The Pan Arab Union of Neuro-logical Societies (PAUNS) has offered training pro-grams in neurology for candidates from sub-SaharanAfrica, and the constructive support Dr. RiadhGouider, the president of PAUNS, is important for oureducational activities.

The Asian-Oceanian region has the greatest part ofthe world’s population and has become an importantpart of the world’s neurological research and practice.To acknowledge this strategic position, we organized theWFN Council of Delegates for 2008 in New Delhi, dur-ing the 12th Asian Oceanian Congress of Neurology.The WFN is also grateful to the Thai organizers of the19th World Congress of Neurology, which took placein Bangkok in October and was highly successful.

During my presidency, I had the pleasure of at-tending the 12th Pan-American Congress of Neurol-ogy in Santo Domingo, Dominican Republic, and Iwas struck by the extent and depth of the neurologi-cal activities presented. The experiences from someCentral American countries, presented by Dr. TedMunsat, professor emeritus in neurology at the TuftsUniversity School of Medicine in Boston, and Dr. Mar-co Medina, director of the neurology training pro-gram at the National Autonomous University of Hon-duras, Tegucigalpa, have been helpful in formulatingthe Africa Initiative.

Continuing ProgressIt is also time to thank the administration at the WorldHealth Organization’s Department of Mental Healthfor its close collaboration and insight and understand-ing of neurological aspects of public health, which weregard as essential for the development of neurology.

The nucleus of the WFN is composed of the nationaldelegates, and I thank you all for your loyalty to the or-ganization, your engagement, and your collaboration.Our committees—Constitution & Bye-Laws, Africa, Ed-ucation, Finance, Fundraising, Membership, Nominat-ing, Public Relations, Publications & Website, Re-search, Stroke Affairs and Liaison, and WCNLiaison—consist of our members and are crucial for theFederation’s work and progress.

The new leadership will start the new decade with newcommittees. Let me thank the committee chairs andmembers for their engagement in WFN activities and fortheir hard work, and I give my best wishes to those whowill now pick up the torch and carry on the work forworld neurology: Quod bonum felix faustumque sit! (Ci-cero)—May it be good, fortunate, and prosperous! ■

BY JOHAN A. AARLI, M.D.

Sudan is Africa’s largest country, ex-tending from the great African Sa-

hara in the north to the equatorial rainforests in the south. Most of its 40 mil-lion inhabitants live in the rural areaswhere farming and animal raising are themain livelihoods. The multi-ethnic, mul-ticultural, and multireligious state is ad-ministratively divided into 25 states, witha federal central government based in thecapital Khartoum.

Modern medicine was introduced tothe country by the British, who ruled thecountry from 1898 until the indepen-dence on Jan. 1, 1956. The KitchenerSchool of Medicine, which was estab-lished in 1923 and incorporated in theUniversity of Khartoum in 1951, hasgraduated thousands of highly traineddoctors. However, neurology has andmostly still is taught to medical studentsby general physicians. There is no formalpostgraduate neurology training pro-gram available in Sudan and all of thecountry’s neurologists have been trainedabroad. A recent major expansion in thenumber of medical schools in the coun-try—there are now about 24—has re-sulted in an unprecedented number ofunder- and postgraduate students whoneed training in all the medical speciali-ties, particularly neurology.

With the encouragement and support

of my colleagues in Sudan as well asabroad, I was able to arrange and carry outthe first formal clinical skills neurologycourse in Sudan last May at Soba Univer-sity Hospital, Khartoum, the country’s

biggest tertiary care university hospital.Registration for the full-day course

was limited to 25 candidates, and it wasfully booked before we could put a pub-lic advertisement. In the end, there were28 candidates because we also invited 3senior nurses.

The course began with structuredseminars on neurological history tak-ing, clinical examination, localization,and differential diagnosis in neurology.We covered basic neurophysiology andneuropharmacology, held an open dis-cussion forum on epilepsy, and a sessionon multiple sclerosis supplemented withvideo material, which the candidatesfound most useful.

Finally, there was a 2-hour hands-onneurological examination in the Practi-cal Assessment of Clinical ExaminationSkills format covering a variety of clini-cal scenarios and common neurological

problems in the Sudan. Tutorsguided the candidates though ex-aminations of patients with var-ious types of stroke, cerebellarataxias, peripheral neuropathy,nontraumatic spastic paraplegia,and neuromuscular disorders.

The day ended with a chal-lenging open clinical quiz duringwhich candidates were able torevise the course contents and re-

hearse some of the procedures. It also in-cluded information about conditions thathad not been covered in the seminars andclinical examinations.

The course was well received andpraised by both candidates and academ-ic officials. Prof. Ammar El Tahir, thedean medicine at Soba University Hos-pital, Suliman Hussein, the director gen-eral of Soba, and Dr. Alaa HassanAhmed, head of the department of med-icine at the University of Khartoum, at-tended some of the sessions and left uswith very encouraging comments. Feed-back from the candidates was also verypositive, and they suggested extendingthe course to 2 days.

We hope we will be able to extend thecourse to 2 days and that we will be ableto attract candidates from the neighbor-ing countries of Kenya, Uganda, Malawi,Tanzania, and Ethiopia.

However, such a course would need alot of logistic support. The World Fed-eration of Neurology has offered somehelp, but formal adoption by the WFN orone of its branches would secure its con-tinuity. We would also like to see world-class experts volunteering to teach in up-coming courses. A visit from arepresentative of the WFN or other in-ternational bodies such as the EuropeanFederation of Neurological Societies, theAmerican Academy of Neurology, or theAssociation of British Neurologists, andother African or Arabic neurological so-cieties, would be a major enhancement inhelping boost the profile of the course.

In addition to logistic support, we alsoneed tools for neurological examinations,ranging from patellar hammers, oph-thalmoscopes, tuning forks, visual acuityand colour vision charts, neuroanatomydemonstration models, to mannequinsfor training in lumbar puncture.

We also need assistance in establishinga neurology unit at Soba University Hos-pital as well as devising a program totrain 12 epilepsy specialist nurses in theSudan. ■

Eager Response to Sudan’s First Clinical Neurology Course

BY OSHEIK ABU’ASHA SEIDI,M.B.B.S.

Dr. Seidi is associate profes-sor of medicine and consul-tant neurologist in the facul-ty of medicine and SobaUniversity Hospital, Univer-sity of Khartoum, Sudan.

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4 • WORLD NEUROLOGY WWW.WFNEUROLOGY.ORG • FEBRUARY 2010

leverage improvements in neurologicaldisease and promotion of brain healthworldwide. Many opportunities exist forsynergy. The Greek root “syn” (with) im-plies working with others, both individu-als and organizations. This includes re-gional, national, international, and sub-specialty groups; the World Health Orga-nization (WHO); and other initiatives withwhich we can generate synergy, such asthe global alliance against chronic dis-eases. Synergy also implies that the com-bination is more than the sum of its parts,that is, it creates added value. Value, eval-uation, and viability must be our bywords.

ValueIf we are to make a difference, we mustfocus on modest investment, high-yield ac-tivities. This usually occurs when there isa great need, a solution, and a big gap be-tween what is known and what is applied.That statement has broad application inneurology. If the WFN concentrates onidentifying the minority of actions thatyield the majority of results and syner-gizes with other organizations, then ourimpact can be many times greater thanthey would be if we worked alone. Theeconomist Vilfredo Pareto noted in theearly 20th century that 20% of peopleowned 80% of the land in Italy. Years lat-er, the Pareto Principle was reformulatedas the 80:20 rule, that is, about 80% of re-sults come from about 20% of the efforts.

Value can also mean “added value.” Ifsomething is already being done, whatelse can be done for additional value atlow cost? For example, if the WFN

cosponsors the teaching of an outstand-ing individual in a developing country,could the sessions be professionally video-taped, produced, and used elsewhere andon our Web site as part of a growing cat-alogue of online courses? Or could theproduction of a short documentary aboutpeople from a particular country be usedfor raising funds and awareness of theirneeds? Another dimension of value is“comparative value”—what is the valueof one activity compared with another?

The American Heart Association, theWFN, the World Stroke Organization,the European Stroke Organization, theEuropean Stroke Conference, the Cana-dian Stroke Network, the National Insti-tute of Nervous Disease and Stroke, andLippincott Williams & Wilkins are sup-porting a synergium (a forum for work-ing together) to develop and commit to aworld stroke agenda, identifying in rankorder what steps would yield the greatestresults. If successful, this process could bea model for developing prioritized agen-das for major neurological diseases in co-operation with all relevant organizations.

Evaluation and ViabilityA key component of any activity is eval-uation. Unless we have objective baselinemeasures, repeated after implementations,we cannot be sure of our impact. In theera of evidence-based medicine, we needevidence-based actions and evaluations.

Wherever possible, we should lookfor interconnections and synergies.There are many more worthy projectsthan we could possibly afford. Thus it be-

comes critical that we focus our effortson viability. Before beginning a project,we must ask how it will continue afterour involvement ceases. We cannot be afunding agency, so we must harness ourtrue wealth: the prestige, expertise, andcommitment of our members.

The WFN is sound academically, ad-ministratively, and financially, making thisthe perfect time to plan change for best re-sults. We can afford “to make haste slow-ly,” festina lente, in the words of Augustus.The more we consider, consult, and reflectat the outset, the more decisively and ef-fectively we can move forward.

We will have continuity: Johan hasagreed to represent us at the WHO, withwhom he published the influential “Atlas:Country Resources for Neurological Dis-orders” (2004) and “Neurological Disor-ders: Public Health Priorities” (2006),and to serve as an adviser to the Presidentand the Africa Initiative, his proudestachievement. Secretary-Treasurer Gen-eral Raad Shakir will continue, with ex-panded administrative responsibilitiesand in his influential role as Chair of theExpert Neurology Committee advisingon the revision of the International Clas-sification of Disease (ICD-10).

Werner Hacke, now WFN Vice-Presi-dent, will have the important portfolio ofCongresses and will help with our effortsto form alliances with other brain-relat-ed organizations. Ryuji Kaji will contin-ue in a new and ambitious portfolio.

We also will have change. Roger Rosen-berg, Chair of the Research Committee,ended his tenure after a highly successfulscientific and educational program of the19th World Congress of Neurology inBangkok, Thailand, organized by NiphonPoungvarin and his colleagues with re-

sounding success. Roger and Niphon,you and your colleagues have set a highstandard. Thank you very much!

New BloodWe welcome two new elected trustees,Gustavo Roman and Wolfgang Grisold,both with long-standing involvementwith the WFN, Gustavo in neuroepide-miology and Wolfgang in education.

Several other individuals will join theExecutive, some with new portfolios.All of the appointments will be for 2years, renewable, including individualmemberships in committees and taskforces. This allows for greater numbersof individuals to become actively in-volved and for flexibility to participate inseveral activities. Each of the commit-tees will have task forces with specificgoals, plans, and timetables.

An important first step is to survey allrelevant activities in a given area. For ex-ample, a number of different organiza-tions offer a variety of educational activ-ities. Could something be gained bybetter coordination and a systematic eval-uation of quality and results? It wouldhelp to know where we are, before we de-cide where we are going. Many more willbe involved and much more will need tobe done. Please advise us on ongoing ac-tivities and volunteer for specific tasks bywriting to info@wfneurology.org.

As Johan has indicated in his farewellmessage, all Chairs and Committee mem-berships ceased as of Dec. 31, 2009. Thenew Chairs are being appointed and withtheir help, the new committee and taskforce members. We will aim at an optimalbalance between continuity and change aswe ask you to join us in broadening thehorizons of world neurology. ■

Synergy Key to Plan’s SuccessContiunity • from page 1

MEETING REPORT

ENS Holds Symposium in India The 2nd European Neurological Society Symposium

in India took place in Chennai in July last year. A pan-el of four ENS speakers and four Indian neurologistspresented a series of lectures and discussed cases witha large audience of more than 400 neurologists fromseveral neurological centers in India.

As president of the ENS, I thanked the organizers forthe opportunity to lecture in India and to interact andexchange ideas and experiences with our Indian col-leagues. Here is a brief summary of each of the pre-sentations that were made during the 2-day gathering: � On the first day, I presented a comprehensive re-view of cerebral venous thrombosis, focusing on sev-eral trials and registries that would get underway inthe next few years. I urged attendees to consider theparticipation of Indian neurological centers in thestudies.

The next day, I described interventions for increas-ing the effectiveness of rTPA. I presented a critical re-view of pharmacological and endovascular interven-tions—either available or under development—thatmight overcome the limitations of the 0- to 4.5-hourIV rTPA treatment.� Dr. Deepak Arjundas, of the department of neurol-ogy at the Vijaya Health Centre in Chennai, spokeabout stroke in India. He described Vijaya’s well-equipped and busy Stroke Centre, though he noted thatthe high cost of rTPA in India was a barrier for ex-panding acute stroke treatment.

� Prof. Martin J. Brodie, director of the epilepsy unitat the Western Infirmary in Glasgow, Scotland, re-viewed antiepileptic drugs, focusing on their mecha-nism of action, side effects, and therapeutic spectrum.

He gave a second presentation on the managementof epilepsy, using mono- and polytherapy. After his lec-ture, two Indian neurologists presented two complex

clinical cases of epilepsy, which were opened to dis-cussion by the panel and audience members.� Dr. Roop Gursahani, an epilepsy expert from Mum-bai, outlined the clinical effectiveness of leveracetamand reviewed its effectiveness as an add-on therapy, afirst-line therapy, and on refractory status epilepticus.� Prof. Giuseppe Lauria, of the National Neurologi-cal Institute and head of the Skin Biopsy, PeripheralNerve, and Neuropathic Pain Centre in Milan, spoke onpainful neuropathy in the context of its pathophysiol-ogy, comorbidities, nosological classification, the tech-

niques for diagnosing and quantifying the condition,and available treatments.� Dr. U.K. Misra, of the department of neurology atthe Sanjay Gandhi PGIMS in Lucknow, India, illustrat-ed his talk on peripheral neuropathies with the pre-sentation of several interesting clinical cases, some ofthem rarely seen in Europe.� Prof. Alexis Arzimanoglou, of the Institute for Chil-dren and Adolescents with Epilepsy at the UniversityHospitals of Lyon, France, gave a thoughtful presen-tation of the challenges, advantages, and disadvan-tages of the classification of epileptic seizures and syn-dromes. He also delivered the final lecture of thesymposium in which he addressed the topic of epilep-sy surgery in which he stressed the importance of ear-ly surgery in drug-resistant epilepsy and reviewed thepresurgical evaluation strategies.� Dr. V. Jayakumar from Madurai presented a very in-teresting series of video recordings of different typesof seizures in children and adolescents.

At the end of the meeting, we held the final roundof the Young Neurologist Excellence Award tourna-ment. The top two competitors won a trip to attend the2010 ENS meeting in Berlin.

The interest and active participation of Indian neu-rologists was enormous, and all of the lectures were al-ways followed by numerous and pertinent questionsfrom the audience. In turn, the ENS faculty enjoyed theinteraction with their Indian colleagues. ■

BY JOSÉ M. FERRO, M.D., PH.D.

Dr. Ferro is director of the depart-ment of neurosciences at the SantaMaria Hospital in Lisbon and chair-man of the faculty of medicine atthe University of Lisbon. He is pres-ident of the European NeurologicalSociety.

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6 • WORLD NEUROLOGY WWW.WFNEUROLOGY.ORG • FEBRUARY 2010

220011003rd International Conference onHypertension, Lipids, Diabetes & StrokePreventionMarch 4-6Berlinwww.kenes.com/strokeprevention

8th World Congress on Brain InjuryMarch 10-14Washington, D.C.www.internationalbrain.org

6th World Congress forNeuroRehabilitationMarch 21-25Viennawww.wcnr2010.org

2nd International Conference “Advancesin Clinical Neuroimmunology”May 31-June 1Gdañsk, Polandwww.bokiz.pl/neuroim2010

15th Annual Meeting of the InternationalSociety for the History of theNeurosciencesJune 15-19Pariswww.ishn.org

20th Meeting of the EuropeanNeurological SocietyJune 19-23Berlinwww.congrex.ch/ens2010

14th Congress of the EuropeanFederation of Neurological SocietiesSept. 25-28Genevawww2.kenes.com/efns2010/Pages/home.aspx

23rd Scientific Meeting of theInternational Society of HypertensionSept. 26-30Vancouver, Canadawww.VancouverHypertension2010. com

2nd European Headache and MigraineTrust International CongressOct. 28-31Nice, Francewww2.kenes.com/ehmtic/Pages/Home.aspx

14th World Pain Clinic Congress & the1st Asian Congress on PainOct. 28-31Beijingwww2.kenes.com/wspc/Pages/Home.aspx

2200111110th International Conference onAlzheimer’s & Parkinson’s DiseasesMar. 9-13Barcelonawww.kenes.com/adpd

20th World Congress of NeurologyNov. 12-18Marrakesh, Morocco

Calendar of International Events

WFN 2009 ANNUAL REPORT

A Sound Foundation for 2010 B Y J O H A N A . A A R L I , M . D.

For the World Federation of Neu-rology, the most important eventin 2009 was the 19th World Con-

gress of Neurology, which took placein Bangkok, Thailand, Oct. 24-30.

Despite the international financialcrisis and political unrest, the Thaineurologists prepared for Congress ina mature, open, and well-balancedway and made it an extremely suc-cessful and memorable conference.

The world congresses in neurologyare crucial for the visibility of theWFN and remain the most effectivevenue for presenting scientific achieve-ments and interaction between dele-gates of varied backgrounds and per-spectives. It is therefore time to extendour sincere thanks to the Thai orga-nizers, and to the WFN officers whowere involved in the arrangementsfor the Bangkok congress.

Dr. Roger Rosenberg and Dr. Nara-porn Prayoonwiwat organized thescientific program, Dr. SiwapornChankrachang was responsible forthe teaching tourses, and Dr. RaadShakir, with his experience from theworld congresses in London (2001)and in Sydney (2005), organized the3rd Tournament of the Minds. To-gether with them was an army ofhard-working colleagues, each ofwhom deserves our warm thanks.

Dr. Niphon Poungvarin, the Con-gress President, and his staff collabo-rated with the professional meetingorganizer, Congrex, to coordinate thelectures, symposia, courses, postersessions, and exhibitions, as well asthe transport between the hotels andcongress center. It all was executed ina convincingly effective manner, even

in the small details, which gives theBangkok congress a well-deservedplace in the WFN history.

Unique to the WCN 2009 was thepresence of H.R.H. Princess MahaChakri Sirindhorn, who presidedover the opening ceremony. HerRoyal Highness’s presence was agreat honor for the Federation, andit demonstrated the Thai Royal Fam-ily’s broad engagement in scienceand medicine and in promoting thesocial and economic development ofthe Thai nation.

In 2008, the WFN decided to es-tablish two WFN Medals—one forscientific achievement in neurologyand the other for service to interna-tional neurology. The medals werepresented for the first time at theBangkok world congress. Theawardees were Dr. Noshir Wadia ofIndia for his work for service to in-ternational neurology, and Dr. RogerRosenberg of the United States for hisscientific achievement in neurology.

At the Council of Delegates, Dr.Vladimir Hachinski was elected Pres-ident of the WFN; Dr. Werner Hacke,the First Vice-President; and Wolf-gang Grisold, a new Trustee. The neu-rological societies of Albania, Arme-nia, and Kazakhstan were welcomedas new members of the Federation,which now numbers 110 societies.The Moroccan Neurological Society isalready well underway with the prepa-rations for the 20th World Congress ofNeurology, which will take place inMarrakesh in November 2011.

An important part of the WorldHealth Organization’s internationalresponsibilities is the InternationalClassification of Diseases (ICD). Mostcountries use the ICD-10, which will

now be revised. The WHO Depart-ment of Mental Health has appoint-ed a WHO International AdvisoryGroup for the revision of the ICD-10Chapter VI on Diseases of the Ner-vous System. The Advisory Groupwas set up in collaboration with theWFN and had its first meeting inGeneva in June last year. The ICD-11is scheduled to be completed in 2012.

The Africa Initiative is well estab-lished, with Alfred K. Njamnshi(Cameroon) as the Regional Director.Amadou Gallo Diop (Senegal) has putthe number of neurologists in sub-Sa-haran Africa in 2009 at 267. The last es-timate, in 1996, counted 121. Evenwith the inherent uncertainties, thenumber of neurologists in Africa is in-creasing, and it is encouraging thatnew neurologists have now beentrained in South Africa for Angola andNamibia. The first Ethiopian neurol-ogy residents have completed theirtraining at Addis Ababa University,bringing that country’s number ofneurologist up to 14—for a populationof about 80 million people. The Ital-ian Neurological Society is active intraining Ethiopian neurologists.

In 2009, Osheik Seidi organized aneuroscience course in Khartoum, Su-dan, and will run another this year. TheFrench Neurological Society has beenvery active in training African neurol-ogists. Several other national societiesare working in various developingcountries. National neurological asso-ciations can also “adopt” candidatesby covering expenses for travel, school,and accommodation for training incandidates from developing countries,provided they return to their owncountry after they have qualified. Suchdiscussions have already started. ■

Last year marked the 10th anniver-sary of People to People, a U.S.-

based nonprofit organization that isdedicated to improving the health ofAfrica’s poor, especially those inEthiopia, and combat HIV/AIDS onthe continent.

The group is the brainchild of Dr.Enawgaw Mehari, an Ethiopian-bornneurologist who completed his med-ical training in Czechoslavakia and afellowship in neurology the UnitedStates. After visiting his home coun-try in 1999, Dr. Mehari was moved tomotivate those in the diaspora to con-tribute funds and use their skills inhelping make a difference in the livesof those their compatriots.

With funds from donors and the ac-tive participation of intellectual, eco-nomic, and government leaders inEthiopia, the organization went fromstrength to strength in its first decade.

It has contributed to the developmentof a neurology training program, ayouth reproductive health program; aknowledge-sharing project in collab-

oration with the University of AddisAbaba and the World Bank; an inter-national discussion forum on healthmatters; HIV/AIDS education andawareness programs for high schoolstudents; and a boarding school forgirls. Other educational initiatives in-clude vocational training for AIDSorphans and a postgraduate program

in social work at the University of Ad-dis Ababa.

People to People also publishes theHorn of Africa Journal of AIDS, a bian-nual publication for medical schoolsin Ethiopia and East Africa and runsa project that posts articles on pre-ventative medicine online for theEthiopian public. In 2005, it negoti-ated with Pfizer Foundation for afree supply of the drug fluconazolefor AIDS patients with systemic fun-gal infections.

Looking ahead, the organization re-mains committed to fostering home-based hospice care in Ethiopia withguidance from U.S.-based hospice pro-fessionals and mitigating the impact ofthe brain-drain among Ethiopian med-ical doctors and nurses. ■

This article is based on informationfrom People to People.

People to People Marks Its First Decade

THE FOCUS WILL CONTINUETO BE ON MITIGATING THE

IMPACT OF THE BRAIN-DRAININ THE ETHIOPIAN MEDICAL

COMMUNITY.

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8 • WORLD NEUROLOGY WWW.WFNEUROLOGY.ORG • FEBRUARY 2010

lation system comprises elec-trodes that are surgically im-planted in epileptic regions ofthe brain and connected to thecomputerized, battery-poweredneurostimulator, which is em-bedded in the patient’s skull.The device, which continuous-ly monitors the electrical activ-ity of the patient’s brain, is pro-grammed by a neurologist todetect and disrupt significantelectrical events.

“The programming is donewirelessly by the physician via alaptop computer,” Dr. Morrellsaid. “It’s highly modifiable inthat the physician can view thepatient’s electrocorticographicactivity in real time and changethe [signal-detection] criteria atany time based on individualpatient characteristics.”

Up to two leads, each con-taining four electrodes, can beconnected to the neurostimu-lator, so the system can moni-tor and deliver responsive stim-ulation to two distinct epi-leptogenic zones independent-ly, she noted.

Because the neurostimula-tion occurs in response to aber-rant electrical activity in thepatient’s brain, fewer electricalimpulses are being delivered tothe brain than would occurwith continuous stimulation.This in turn diminishes the pos-sibility of treatment-related ad-verse events, Dr. Morrell ex-plained.

In an initial feasibility study of65 patients, the responsive neu-rostimulation system demon-strated excellent safety, tolera-

bility, and preliminary evidenceof efficacy, Dr. Morrell said.“There were no serious device-related adverse events, and stim-ulation-related symptoms expe-rienced by several subjects wereaddressed by adjusting the stim-ulation settings.”

The preliminary efficacy evi-dence from that study showedthat a minimum 50% reductionin seizure frequency was expe-rienced by 43% of the patientswith complex partial seizuresand 35% of those with total dis-abling seizures (Neurothera-peutics 2008;5:68-74).

In the double-blind pivotaltrial, the 191 patients were ran-domized to active or sham ther-apy. All of the patients werebetween 18 and 70 years of age(median age 35 years), and allhad partial onset epilepsy lo-calized to one or two foci andhad failed at least two anti-epileptic medications.

The patients were taking anaverage of three antiepilepticmedications to attempt seizurecontrol, and approximately 34%of the patients had been treatedpreviously with vagus nervestimulation, 33% had prior sur-gical resection, and 16% hadbeen treated with both.

“These patients tended to bevery ill. Most of them hadepilepsy for more than 20 years,and many were having at leastthree seizures per 28-day peri-od—often many more thanthat,” Dr. Morrell said.

Of the 191 patients implant-ed with the responsive neu-rostimulator device, 50% hadmesial temporal seizure onset,42% had neocortical seizure on-set, and 8% had both, Dr. Mor-rell said in a press briefing at themeeting.

The trial consisted of an ini-tial, 12-week period prior tosystem implantation duringwhich baseline seizure activitywas collected, followed by a 12-week blinded period when par-ticipants were randomly as-signed to have the responsivestimulation activated or left in-active, she said.

At each of the 31 trial sites,the patients and one neurolo-gist were blinded to the stimu-lation status, while a separateneurologist programmed thedevices in order to maintain thestudy blinding. The responsivestimulation was optimized inthe treatment over the next 4weeks, followed by 84 days ofdata collection. At the end ofthe blinded efficacy period,stimulation was activated for allof the study participants for 2years post implantation, Dr.Morrell said.

In addition to the statistical-ly significant reduction inseizure frequency in the activetherapy group relative to thosein the sham therapy condition,there were no serious, unantic-ipated device-related adverseevents during the trial, nor wasthere a difference between thetwo groups with respect to therate of adverse events, includ-ing depression, memory im-pairment, and anxiety, Dr. Mor-rell reported.

The findings suggest that re-sponsive neurostimulation maybe a promising treatment op-tion for individuals withseizures that are resistant toconventional antiepileptic ther-apy. It is important to note thatthe apparent increase in thenumber of patients experienc-ing at least a 50% reduction inseizure frequency relative tobaseline during the open-labelphase of the study suggeststhat the system might becomemore effective over time, Dr.Morrell noted.

The responsive neurostimu-lation system has not yet re-ceived approval from the U.S.Food and Drug Administration,but NeuroPace plans to submita premarket approval applica-tion to the agency in early 2010,Dr. Morrell said. ■

Treatment IndividualizedSeizures • from page 1

Major Finding: Seizures declined by a mean of 29% duringactive stimulation with a neurostimulator over the first 12weeks, compared with a 14% drop during sham activation.

Data Source: Multicenter, randomized, sham-controlledclinical trial of 191 patients with medically intractable par-tial onset seizures.

Disclosures: Dr. Morrell is chief medical officer of Neuro-Pace, which developed the system and funded the trial.V

ITA

LS

Using neurostimulation forseizure control is not

new. In the early 1970s, Dr.Irving S. Cooperand his colleaguesdemonstrated thatcerebellar stimula-tion was capable ofreducing seizurefrequency in halfof treated patients.

However, thiscurrent trial is a cus-tomized treatment,where each patient receives themost appropriate type of stim-ulation possibly designed withour current technology. Thestudy findings will contributeto our understanding of treat-ment resistance in epilepsy. Isresistance associated with the

structure where the seizuresarise? Could resistance be as-sociated with specific electrical

patterns that are not“aborted” by a safestimulation? Or is itpossible that alltypes of seizures canbe equally con-trolled in a numberof patients receivingneurostimulation,and other factors(such as individual

genetic profile) play an impor-tant role in resistance to treat-ment? Further analysis of theresults should help clarify theseissues.

Some patients may not betoo excited about a 40% re-duction in seizure frequency.

However, in patients with re-fractory epilepsy, we rarelyobserve a pharmacologicallyinduced improvement of thismagnitude that is sustainedpast the “honeymoon” periodwith a new medication. Inthe few patients who achievesuch an improvement, theside effects of the drugs arefrequently intolerable.

Therefore, despite thesmall risks associated withthe surgical device implanta-tion, neurostimulation offersthe possibility of a signifi-cant improvement in seizureswithout the common side ef-fects of drugs.

DR. DANIELLE M. ANDRADE

is the director of theTransitional Epilepsy Programat the University of Toronto.C

OM

ME

NT

S

Risks Offset Improvement

B Y M I C H E L E G. S U L L I VA N

Else vier Global Medical Ne ws

B A N G K O K , T H A I L A N D — Serumlevels of N-acetyl aspartate are signifi-cantly higher in patients with relapsing-remitting multiple sclerosis and clinicalsyndromes suggestive of MS than theyare in patients with neuromyelitis opti-ca and they might be a valid biomarkerto help distinguish the disorders.

In a study of 176 subjects, Dr. CarlaTortorella found that serum N-acetyl as-partate (NAA) levels were about 14 timeshigher in those with MS or a clinicallyisolated syndrome suggestive of MS(CIS) than they were in those with neu-romyelitis optica (NMO). In fact, levelsin NMO patients were the same as theywere in age-matched healthy controls.

NAA is normally synthesized in neural

mitochondria and leaves the cell by sev-eral methods: passing from neurons tooligodendrocytes where it is catabolized;passing through the astrocytes into the ex-tracellular space and thus into the blood-stream; and passing into the cerebrospinalfluid, Dr. Tortorella said at the WorldCongress of Neurology. This process isabnormal in patients with MS, leading toincreased serum NAA levels, but no stud-ies have compared these levels in patientswith MS and those with NMO.

Dr. Tortorella examined serum andCSF levels of NAA in 48 patients withrelapsing-remitting MS, 20 with CIS,and 32 with NMO, and included 76 age-matched healthy controls for compari-son. At baseline, those with NMO wereolder (median 43 years) than those withCIS (28 years) or MS (38 years). Diseaseduration was also different: CIS, 6

months; MS, 6 years; NMO, 5 years.The Expanded Disability Status Scale

(EDSS) score was 1.5 in the CIS group,2 in the MS group, and 4.6 in the NMOgroup. None of the MS or CIS patientswere taking disease-modifying drugs,whereas 10 of the NMO patients weretaking immunosuppressants.

All of the patients submitted serumNAA samples. The levels were similarlyhigh in those with CIS and MS (1.7mM/L in each group). These were sig-nificantly higher than the levels found inthose with NMO and in healthy controls(0.12 mM/L each).

All of the MS and CIS patients hadCSF levels available for testing, where-as only eight of the NMO patients did,and there were no CSF samples fromhealthy controls. “Nevertheless, the CSFNAA levels were markedly and consis-

tently higher in the CIS and MS patients[0.68 and 0.76 mM/L] than they were inthe NMO patients [0.05 mM/L],” Dr.Tortorella, of the University of Bari,Italy, said.

She found no significant associationbetween NAA levels and age, disease du-ration, or disease activity. However, inthose with MS, she found a significantcorrelation between increasing NAA lev-els and worsening EDSS scores.

Because the correlation betweenserum NAA and MS is so much strongerthan it is with NMO, Dr. Tortorella sug-gested that NAA might be a useful waynot only to help distinguish between thedisorders but to measure the progressionof MS, particularly in the early phase ofthe disease.

Dr. Tortorella did not have any con-flicts of interest to declare. ■

NAA Levels Discern MS From Neuromyelitis Optica

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www.clinicalneurologynews.com

— We Write Medicine’s First Draft —Clinical Neurology News

THE LEADER

IN NEWS

AND

MEETING

COVERAGE

Before the drugis approved...

Before the guidelineis issued...

Before the researchis published...

eli

arch

You read it first in

EADER

ine

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10 • WORLD NEUROLOGY WWW.WFNEUROLOGY.ORG • FEBRUARY 2010

2009 JUNIOR TRAVELLING FELLOWSHIP ROUNDUP

A Chance to Connect, Share, and Learn9th Congress of the FrenchNeuroscience Society, Bordeaux,France (May 26-29)

BY MOUNIR OUZIRMaking Worthwile Contacts. This 3-day gathering was cochaired by Prof.Abdelhamid Benazzouz and Prof.Stéphane Oliet, both of the University ofBordeaux.

The program comprised 10 plenarylectures, among them the Paul BrocaLecture given by Pierre Magistretti,

Ph.D., of Switzer-land, titled “Neu-ron-Glia MetabolicCoupling and Plas-ticity; and the Al-fred Fessard Lec-ture, delivered byChristine Petit,Ph.D., of France,on linking deaf-

ness genes to auditory physiology.I presented a poster on the impact of

lead on the circadian rhythm of loco-motor activity and the prophylactic ef-fect of melatonin and 5-metoxytryp-tophol in rats. The circadian rhythm oflocomotor activity and its synchroniza-tion by light is an essential behavioralparameter for assessing the function ofsupply-chain integration of photic in-formation. It is currently known thatlead interacts with the glutamatergictransmission, especially by blocking N-methyl-D-aspartate receptors.

My colleagues and I studied the im-pact of lead on the rhythm of circadianactivity, knowing that the suprachias-matic nucleus is the gateway to photicinformation mobilizing a glutamatergicsynapse. Our results showed a decreasein locomotor activity and disturbance ofthe rhythm of circadian activity in thelead-treated rats, suggesting the deteri-oration of the transmission of photic in-formation toward the suprachiasmaticnucleus.

Moreover, we found that melatoninand 5-methoxytryptophol seemed to beprotective against these effects.

I appreciate the opportunity affordedme by the WFN to attend this Congress.I encountered leading neuroscientistsand learned about new developments intechnology and research. In addition, Imade contact with Prof. Benazzouz, andthis year, I am going to be working withhim on Parkinson’s disease. ■

MR. OUZIR is a doctoral student at theLaboratory of Clinical Neuroscience andMental Health at the University Hassan IIin Casablanca, Morocco.

BY SAMIRA CHAIBEncouraging Scientific Cooperation.The Bordeaux neuroscience congresswas attended by researchers, lecturers,and students working in neuroscience inthe public and private sectors.

It was a great opportunity to share re-

cent advances in research that is shapingthe understanding of brain mechanismsand the treatment of neurological andpsychiatric diseases. I firmly believe thatit is such sharing that encourages scien-tific cooperation.

I presented a poster based on my re-search work in which I am evaluating theantinociceptive and anti-inflammatory

effects of theethanolic extractof the root of theMoroccan medici-nal plant, Rubiaperegrina.

Many of the ses-sions I attendedwere useful and in-teresting, such as

those addressing ethical considerations inthe neurosciences, depression in OCDand related disorders, and new conceptsin the pathophysiology of pain.

My thanks to the WFN for awardingme the travelling fellowship. It allowedme to attend this inspiring meeting andto discover the charms of Bordeaux,which is classified by UNESCO as aWorld Heritage Site. ■

MS. CHAIB is a doctoral student in theLaboratory of Pharmacology,Neurobiology, and Behaviour in the Facultyof Sciences at Cadi Ayyad University inMarrakesh, Morocco.

13th Congress of the EuropeanFederation of NeurologicalSocieties, Florence, Italy(Sept. 12-15)

BY YOHANNES WOUBISHETWOLDEAMANUEL, M.D.

Sharing New Skills. Earlier last year, Imet the presidents of the WFN andEFNS in Addis Ababa and became awareof the WFN’s mission to develop neu-rology in Africa, and in sub-SaharanAfrica in particular. I was motivated towork toward that goal, which was also

echoed at the con-gress in Florence.

I learned muchwhile I was thereand look forwardto other opportu-nities of learningnew skills and be-ing able to applythem in the clinical

setting and share them with my Ethiopi-an colleagues.

Numerous talks made an impressionon me. Dr. Anna Poggesi of Italy spokeabout using the white matter hyperin-tensities load as a predictor of subcorti-cal vascular encephalopathy, based onpreliminary data from the LADIS[Leukoaraiosis and Disability] study. Sheand her colleagues found that severe age-related white matter changes were asso-ciated with the presence of specific neu-rological signs, independently of infarcts.

In a session on small-vessel diseases itwas suggested that an early feature ofvascular dementia might be both dysex-ecutive symptoms and memory decline,rather than one or the other. Poster pre-sentations on neurobrucellosis and onamyotrophic lateral sclerosis in HIV pa-tients, and an analysis on AIDS andleukoaraiosis were all thought-provoking.

Finally, I was fortunate to attend a lec-ture on mirror neurons and their im-portance in neurological and psychiatricdisorders by Prof. Giacomo Rizzolatti ofthe University of Parma (Italy), lead in-vestigator of the team that discoveredmirror neurons in the macaque monkey.

To the WFN, I say thank you the Ital-ian way: “Grazie mille!” ■

DR. WOLDEAMANUEL is in the departmentof neurology at Addis Ababa University,Ethiopia.

BY MOHAMMED EL-SHERIF, M.D.Opportunities Abound. Given this vol-ume and range of experts at the EFNSmeeting, I learned many new things,made new contacts, and discovered newopportunities.

All of the scientific sessions I attend-ed were interesting, especially one thatwas titled “The Good Life: Patient Self-Management Artsand Education,”that showed howpeople with neu-rological illnesscan achieve a bet-ter quality of lifethrough the artsand education.

A series ofhands-on teaching courses were partic-ularly informative, especially those thatwere on electromyography, nerve con-duction, and transcranial magnetic stim-ulation. I already work in electrophysi-ology but I welcomed the practicalreviews and exposure to new informa-tion.

I presented a poster on an evaluationof the central nervous system affection inpatients with diabetes mellitus that wasfollowed with a lively discussion that pro-vided helpful comments on the work.

Since the meeting, I am more proac-tive in dealing with my patients, more fo-cused on my teaching and research andon ensuring that I keep up with the lat-est developments. My deep appreciationgoes to the WFN for awarding me thefellowship. ■

DR. EL-SHERIF is an assistant lecturer inthe department of neurology at MansouraUniversity (Egypt).

19th World Congress of Neurology,Bangkok, Thailand (Oct. 24-30)

BY ANITA ARSOVSKA, M.D.Exposure to Innovation. The congresstheme “Innovation in Neurology,” was

echoed in each of the presentations inwhich participants learned about the lat-est advancements in stroke, epilepsy,multiple sclerosis,movement disor-ders, headache,and pain.

I gave a posterpresentation basedon a case report onSneddon’s syn-drome, epilepticseizures, and re-current stroke, which was followed by auseful discussion with my colleagues. Ihad many similar opportunities to speakto experts in neurology, to share experi-ences, and exchange ideas.

I was also exposed to a tremendousamount of new information about re-cent advances in neurology, for example,developments in the new antiepilepticdrugs as well as advanced imaging ofepilepsies, new treatment options for is-chemic stroke, new diagnostic and ther-apeutic possibilities of neurovascular ul-trasound, and the potential therapeuticrole of stem cells in neurological dis-eases. It was the kind of informationthat I can pass on to my colleagues andstudents and apply when I treat and di-agnose my patients.

I am very thankful to the WFN for thisunique opportunity and the generoussupport that to allowed me to attend theBangkok congress. ■

DR. ARSOVSKA is a specialist-neurologistand teacher at the University Clinic ofNeurology, Vodnjanska, Skopje,Macedonia.

BY AHMED ASHAFUDDOULA, M.D.Meeting the Experts. It was my greatfortune to be able to attend the WCN,and I thank the WFN for the experienceof a lifetime.

Being in the presence of renownedneurologists and many other expertswas an enrichingexperience. Theirlectures, the pre-sentations of theirresearch works,and the workshopsand teaching ses-sions were invalu-able, exposing theparticipants to thelatest advances and developments acrossthe spectrum in neurology and neuro-logical science.

One of the highlights for me was a lec-ture on carotid endarterectomy by Prof.Hans-Christoph Diener, the pioneer ofstroke units in Germany.

I enjoyed sharing my views andknowledge with others during the de-bate sessions for the poster presenta-tions. In fact, it has encouraged me topossibly present a poster at the nextmeeting I attend.

Continued on following page

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PROGRESSIVE

MYOCLONUS EPILEPSIES:

PME’s IN THE NEW MILLENNIUM

From Marseille to Venice

VENICE, San Servolo - 28 April - 1 May 2010

In partnership with

Fondazione Mariani CARE OnlusCura Assistenza Riabilitazione Età Evolutiva

Scientific Committee

Eva Andermann (Chair) - Montreal Neurological Institute and Hospital, Montreal

Giuliano Avanzini - Fondazione IRCCS “C.Besta”, Milan

Pierre Genton - Hôpital Henri Gastaut - Centre Saint Paul, Marseille

Berge Minassian - The Hospital for Sick Children, Toronto

INFORMATION, PROGRAM and REGISTRATION are online at

www.fondazione-mariani.org

POSTER submission deadline: March 15, 2010

VIDEO entries are also accepted

For further questions: email pme2010@fondazione-mariani.org

Scientific Secretariat: Anna Jansen, email anna.jansen@uzbrussel.be

Wednesday, April 28 (evening)

Introductory session

Thursday, April 29

Unverricht-Lundborg disease

Lafora body disease

Round table: Mechanisms of Lafora disease

Video session

Friday, April 30

The action myoclonus-renal failure syndrome (AMRF)

The neuronal ceroid lipofuscinoses (NCL)

PME’s in lysosomal disorders

Other PME’s

Saturday, May 1

Neurophysiology and neuroimaging in PME’s

Common physiopathological mechanisms

Differential diagnosis

Diagnostic guidelines

Round table: Guidelines for the diagnostic work-up

Treatment guidelines

Round table: New perspectives in therapy

In cooperation withMontreal Neurological Institute and Hospital, Montreal

The Hospital for Sick Children, Toronto

Fondazione IRCCS Istituto Nazionale Neurologico “C.Besta”, Milan

International School of Neurological Sciences, Venice

Fondazione

Pierfranco e Luisa Mariani

neurologia infantile

Viale Bianca Maria 28

20129 Milan, Italy

tel. +39 02 795458

fax +39 02 7600.9582

www.fondazione-mariani.org

FEBRUARY 2010 • WWW.WFNEUROLOGY.ORG WORLD NEUROLOGY • 11

The education program on acutestroke therapy and stroke preventionwas most useful and informative from aclinical practice perspective. I hope soonto be able to attempt thrombolysis inacute stroke in my country. We use bot-ulinum toxin to treat hemifacial spasmand blepharospasm but not in post-stroke spasticity.

Other sessions on infections of thenervous system, electrophysiology,movement disorders, and slide prepara-tion, were equally informative. ■

DR. ASHAFUDDOULA is assistant professorof neurology at the SZR Medical Collegein Bogra, Bangladesh.

BY RIZALDY PINZON, M.D.Constructive Participation. The ex-tensive program offered at the WCN of-fered many opportunities for learningand exchange among the participants.

Within the first 2 days, I had alreadyparticipated in four workshops. Oneworkshop, on botulinum toxin, wasvery interesting, and I am eager to useit in my patients in my practice in In-donesia. Another, on testing autonom-ic disorder, made me realize that auto-nomic complaints are commonlyunderdiagnosed and undertreated byneurologists.

It was a unique experience for me tobe able to attend so many lectures byleaders in the specialty. One such ad-

dress was present-ed by Dr. VladimirHachinski, thenewly electedpresident of theWFN. He spokeabout stroke andemphasized thatthe increase in thenumber of strokes

in many parts of the world is the resultof urbanization and Westernization.That is most definitely the case in In-donesia, where I practice.

I also found the scientific session onpain and headache interesting and in-formative. This is a common complaintin our in outpatient clinics, and I wasonce again intrigued by a report aboutusing botulinum toxin injection for treat-ing migraine.

After the workshops on Sunday, Oct.25, we attended the opening ceremonywhere we were entertained with tradi-tional Thai dancing and performancearts and a grand banquet.

This congress was an important andenriching experience for me and I thankthe WFN for the fellowship award. ■

DR. PINZON is in the neurologydepartment at Bethesda Hospital,Yogyakarta, Indonesia.

BY TRINH THI NGOC TRINH, M.D.Invaluable, Enriching Experience. Mytrip to the WCN in Bangkok marked thefirst time I had traveled abroad to an in-ternational scientific meeting. Therewas such a wide range of useful and ex-cellent sessions that I had to carefully se-lect which I attended.

I attended a session on acute cerebral is-chemic stroke that dealt with selection ofpatients to receive tPA based on penum-bral criteria usingdiffusion- and per-fusion-weightedimaging mismatchand transcranialdoppler ultrasono-graphy. Anothersession on strokeprovided useful in-formation abouttrends in antithrombotic therapy.

In particular, I enjoyed the many visual

presentations during discussions aboutneuroimaging and electroencephalog-raphy, and especially a series of sleepEEGs and video monitoring that demon-strated differentiation between epilepticand nonepileptic paroxysmal events in di-agnosing epilepsy.

Prof. David Zee, of Johns HopkinsUniversity, Baltimore, U.S.A., conducteda course in nystagmus, in which I learntabout pathophysiology, investigations,new treatments and how to approach topatients with nystagmus.

I was able to update my knowledge ofdifferential diagnosis and pathogenesis

of restless leg syndrome and the role ofiron in RLS, and learned a number ofnew techniques relating to the diagnosis,treatment and management of nar-colepsy and cataplexy. I also benefitedfrom other sessions on movement disor-ders, multiple sclerosis, and neuropathy.

The talks, seminars, symposia andcontributions of medical knowledge inthis Congress were invaluable to me. Myspecial thanks to the WFN for makingmy attendance possible. ■

DR. TRINH practices as a neurologist inHo Chi Minh City, Vietnam.

Continued from previous page

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12 • WORLD NEUROLOGY WWW.WFNEUROLOGY.ORG • FEBRUARY 2010

The prevalence of genetic dementiais about 1% in patients sufferingfrom neurodegenerative dementia,

varying from less than 1% in Alzheimer’sdisease to 10%-15% in frontotemporal lo-bar degeneration orprion diseases.

Genetic AD is usu-ally associated withautosomal dominantearly-onset AD. It re-sembles early-onsetsporadic AD, thoughsome mutations maypresent particularphenotypes. Causalmutations have been described in threedifferent genes: amyloid precursor pro-tein (APP), presenilin 1 (PSEN1), andpresenilin 2 (PSEN2). Mutations inPSEN1, in chromosome 14, are the mostfrequent and to date, 177 missense mu-tations have been published (http://www.molgen.ua.ac.be/ADMutations/).APP is located in chromosome 21, and 32pathogenic missense mutations plus du-plications of the entire gene can causegenetic AD. PSEN2 is located in chro-mosome 1, and 14 pathogenic mutationshave been described in this gene.

Genetic frontotemporal lobar degen-eration (FTLD) is even more heteroge-neous. The first mutation causing FTLDwas identified in 1998 in the microtubule-associated protein tau (MAPT) gene, inchromosome 17. Since then, it has beenshown that more than 44 missense mu-tations plus partial deletions of the genecause the disease. Genetic defects in pro-granulin (PGRN), also in chromosome

17, were shown to cause FTLD in 2006,but since then, up to 67 point mutationsand the complete deletion of the genehave been described in familial FTLD.

MAPT mutations cause autosomal

dominant early-onset FTLD with almostcomplete penetrance by age 65 years. Thephenotype of PGRN mutations is moreheterogeneous, resembling frontotempo-ral dementia, progressive aphasia, corti-cobasal degeneration, or even AD. The dis-ease usually presents as familial dementia,but may also appear as sporadic or late-onset dementia. Less frequently, muta-tions in chromatin-modifying protein 2Bgene (CHMP2 in chromosome 3), valosin-containing protein (VCP in chromosome9), or TAR DNA binding protein gene (inchromosome 1) cause genetic FTLD.

Genetic prion diseases are associatedwith missense mutations or insertions inthe prion protein gene. Surprisingly, 40%of genetic prion diseases lack familial his-tory of disease. They usually present asrapidly progressive dementia, but maymimic early-onset AD or FTLD. Despitethe number of possible genetic defects, nogenetic alteration is found in 11% of au-tosomal dominant early-onset AD, one-

third of autosomal dominant FTLD, andin an unknown number of familial poorclassified dementia, suggesting new genescausing dementia are yet to be discovered.

However, given the number of analy-ses that need to beperformed to estab-lish if a patient suffersfrom genetic demen-tia as well as the lowprevalence of geneticcases, genetic studiesare too time-consum-ing, expensive, and in-efficient to be doneroutinely in patients

with dementia. Testing is usually restrict-ed to most common genetic defects inthose cases with an early-onset autosomaldominant pattern of inheritance. How-ever, different circumstances, such as in-complete penetrance, de novo mutations,or lack of familial information cause ge-netic dementia to present sometimeswithout familial history of disease.”

Therefore, strict criteria for genetic test-ing selection could lead to the under-diagnosis of genetic cases. In this sense, re-search is now focused on markers thatcould screen more accurately which cas-es should be genetically tested and whichgenes should be studied in each case.

To date, levels of the protein progran-ulin in plasma, serum, or CSF are theonly biomarker that is useful in this sense.Pathogenic mutations in PGRN causedisease through loss of function (hap-loinsufficiency). Some researchers haveshown that carriers of PGRN mutationspresent reduced levels of progranulin inplasma, serum, or CSF, compared withcontrols or familial noncarriers, with nooverlap between values.

In addition, reduced levels of pro-granulin are seen in all phases of the dis-ease, including presymptomatic individ-uals. These levels can be measured by anenzyme-linked immunosorbent assay,which is cheaper and easier than directsequencing of the complete gene. Ofcourse, a low level of progranulin doesnot predict which genetic defect the pa-tient has in PGRN, but it does avoid hav-ing to spend time and money studyingcases of PGRN negative familial FTLD,and it allows for the screening of PGRNdefects in broader series of dementia toavoid clinical misclassification.

Apart from deciding “when” and“which” genetic testing should be done fordementia, there are two other questions:“what for?” and “hows?” should the test-ing be done. No different treatments orprognostic clues can be offered to geneticcases. In addition, detecting a genetic ab-normality that causes dementia in a pa-tient puts all of his/her direct family mem-bers at 50% risk for dementia without anypossibility of avoiding or even delaying thedisease onset. However, many patientsand their relatives request the genetic as-sessment because they want to establishthe cause of the disease for either familyor financial planning or simply to knowwhat the future might hold for them. Inthis sense, the results of genetic counsel-ing programs for dementia show that ge-

netic testing of pathogenic genetic de-fects is safe even for asymptomatic at-risksubjects when performed by a multidisci-plinary trained team with pre- and post-test assessment sessions (Arch. Neurol.2001;58:1828-31; Am. J. Alzheimers Dis.Other Demen. 2005;20:233-8).

Finally, most cases of dementia are notcaused by a concrete genetic defect. Eventhough, heritability—the extent to whichgenetic factors explain a phenotype—iscalculated to be as high as 60%-80% insporadic AD. After hundreds of geneticassociation studies, only the presence ofthe allele E4 of APOE has been unequiv-ocally established as a genetic risk factorfor AD, and it accounts for 50% of the ge-netic susceptibility for AD in whites.

New technologies, with genomic-wideassociation studies of thousands of sub-jects will probably identify new risk fac-tors, such as the very recently describedclusterin, PICALM, or CR1 (Nat. Genet.

2009;41:1088-93 [Erratum in: Nat Genet.2009;41:1156]; Nat. Genet. 2009;41:1094-9). Epidemiological studies of geneticrisk factors provide valuable informa-tion about the molecular basis of disease.Nevertheless, the management of infor-mation about genetic risk factors at theindividual level is complex. At the symp-tomatic level, detection of the APOE E4or another genetic risk factor may in-crease the positive predictive value of aparticular diagnosis. Even so, the bene-fit is low and dementia guidelines suchas those of the European Federation ofNeurological Sciences or the British Na-tional Institute for Health and ClinicalExcellence do not recommend routinegenotyping for clinical purposes.

At the asymptomatic level, known andto-know genetic risk factors could traceprofiles of risk and even calculate the rel-ative risk for the future development ofdisease. The REVEAL study showed thatthe disclosure of APOE status in adultchildren of AD patients is safe (N. Engl.J. Med. 2009;361:245-54). However, if thegeneral population pretest risk for beingdemented is 1% at age 65 and about 13%at age 85, it is of doubtful clinical inter-est to know through genetic testing if therisk is higher or lower, when complete ornull risk cannot be assured and no pre-vention strategies could be offered.

Social requests are often different frommedical recommendations, and it is notscience fiction to predict that genetic riskscanning for cardiovascular diseases, can-cer, or dementia will be soon offered to thegeneral population. Technical and ethicalissues about management and interpreta-tion of results also will arise in parallel toexciting discoveries in the genetics of de-mentia, and researchers and cliniciansshould prepare for such challenges. ■

Testing for Genetic Dementia Presents Ethical Nuances

BY RAQUEL SÁNCHEZ-VALLE, M.D., PH.D., AND ALBERT LLADÓ, M.D., PH.D.

Dr. Sánchez-Valle and Dr. Llado arein the Alzheimer’s Disease andOther Cognitive Disorders Unit inthe department of neurology atHospital Clínic in Barcelona.

A Valuable Update on TrendsNeuroscience in Medicine: Third Edition

Edited by P. Michael Conn (New York: Humana Press, 2008)

The third edition of this book pro-vides a comprehensive overview of

the basics in anatomy, physiology, andmedicine and their implications for thepractice of neurology.

The 55 authors, all experts in their re-spective areas of research, have pro-duced an up-to-date, user-friendly vol-ume of work for medical andpostgraduate students, nurses, neurol-ogists, and general practitioners whohave an interest in neurology and wantto review the basic science underlyingclinical issues. Among the topics cov-ered are cytology and organization ofcell types, anatomy of the spinal cordand brain, the physiology of ion chan-nels, transporters and electrical signal-ing, synaptic transmission, and pre-

synaptic and postsynaptic receptors.The subjects of neuroembryology

and neurogenesis are presented withgreat clarity, and details of vasculatureof human brain and clinical correla-

tions of stroke are a good ex-ample of how the book pre-sents its information, startingwith the basics and progress-ing to clinical applications.

Also emphasized are thefunctions of the cerebellum,brain stem, and cranialnerves; the trigeminal andlimbic systems; basal ganglia;and the thalamus. The basic

aspects of pain, visual system, and au-dition are particularly well explained,as are disorders of language and im-munology. The authors also discussthe intricacies of the biology of drugaddiction and neuropathology of dis-ease, again from the basics through toclinical application.

The detailed chapter summaries andmeticulous referencing, in addition tothe depth of content, make this workan important source on current trendsin neuroscience and their implicationsfor the clinical setting. ■

BY JAGJIT S. CHOPRA,F.R.C.P.E., PH.D., F.A.M.S.,F.I.A.N.

Dr. Chopra is a formerEditor in Chief ofWORLD NEUROLOGY andcurrently serves on itsEditorial Advisory Board.

RESEARCH IS FOCUSED ONMARKERS THAT COULD MOREACCURATELY SCREEN WHICH

CASES SHOULD BE TESTED ANDWHICH GENES ARE STUDIED.

09_13wfn10_1.qxp 1/25/2010 2:30 PM Page 12

Leading resources in clinical neurology!

Volume 9 Issue 2 January 2008 ISSN 1389-9457

Editor-in-Chief:SudhansuChokroverty

Field EditorsR.P. AllenC. GuilleminaultP. LevyL. Ferini-StrambiO. Bruni

Associate EditorsR.P. AllenC. BassettiA. CulebrasR.A. FerberR. GrunsteinC. GuilleminaultJ. HednerW. HeningS. KatayamaP. LevyM. MahowaldJ. MontplaisirM. SandersM. ThorpyT. Young

Official Journal of the World Association of Sleep Medicineand International Pediatric Sleep Association

Official Journal of the European Paediatric Neurology Society

Volume 10, Number 1January 2006

SEIZURE

Volume 15, Number 8, December 2006 ISSN 1059-1311

The Official Journal of Epilepsy Action

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Vol. 131, Nos. 1–2 30 January 2007 ISSN 1566-0702

Clinical Neurologyand Neurosurgery

Volume 108, issue 7, October 2006 ISSN 0303-8467108(7) 621–720

ReviewNeuromuscular disorders in critical illness – L. Pandit, A. Agrawal (Mangalore, India) 621

Original articlesLaterality does not influence early mortality in MCA ischemic stroke – I. Mateo, A. Pinedo, I. Escalza, J.C. Garcia-Monco (Vizcaya, Spain) 628Vascular cognitive impairment in patients with late-onset seizures after an ischemic stroke – J. De Reuck, M. De Clerck, G. Van Maele

(Ghent, Belgium) 632Cardiovascular 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) 638The 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) 644Medically 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) 648Alleviation of intracranial air using carbon dioxide gas during intraventricular tumor resection – T. Beppu, K. Ogasawara, A. Ogawa

(Morioka, Japan) 655Clinico-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 reportsRespiratory failure in a patient with antecedent poliomyelitis: Amyotrophic lateral sclerosis or post-polio syndrome? – S.-i. Terao, N. Miura,

A. Noda (Aichi, Japan), M. Yoshida, Y. Hashizume, H. Ikeda, G. Sobue (Japan) 670Bilateral C5 motor paralysis following anterior cervical surgery—a case report – K.S. David, R.D. Rao (Milwaukee, WI, USA) 675Correlation of magnetic resonance images with neuropathology 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) 682Subacute aseptic meningitis as neurological manifestation of primary SjÖgren’s syndrome – R. Rossi, M. Valeria Saddi (Nuoro, Italy) 688Thin corpus callosum and amyotrophy in spastic paraplegia—Case report and review of literature – B. Winner, C. Gross, G. Uyanik,

W. Schulte-Mattler, R. Lürding, J. Marienhagen, U. Bogdahn (Regensburg, Germany), C. Windpassinger (Graz, Austria), U. Hehr, J. Winkler (Regensburg, Germany) 692

Camptocormia or Pisa syndrome in multiple system atrophy – J. S awek (Gdansk, Poland), M. Derejko (Warszawa, Poland), P. Lass, M. Dubaniewicz (Gdansk, Poland) 699

“Frontal variant Alzheimer’s disease”: A reappraisal – A.J. Larner (Liverpool, UK) 705Transient 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) 709Adie’s pupils in paraneoplastic ganglionopathy with ANNA-1 – J.V. Campellone, A. Hageboutros (Camden, NJ, USA) 712

Book reviewsHead injury: Pathophysiology and Management – D. Van Dam (Wilrijk, Belgium) 715

(Contents continued on OBC)

For a complete list of neurology products, detailed information on the titles above and online access to the journal articles, visit

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Acute Pain

Alzheimer’s and Dementia

Autonomic Neuroscience: Basic and Clinical

Brain & Development

Clinical Neurology and Neurosurgery

Clinical Neurology News

Clinical Neurophysiology

Epilepsy & Behavior

Epilepsy Research

European Journal of Paediatric Neurology

European Journal of Pain

Experimental Neurology

Journal of Clinical Neuroscience

Journal of Neuroimmunology

Journal of the Neurological Sciences

Journal of Pain

Journal of Pain and Symptom Management

The Lancet Neurology

Neurobiology of Aging

Neuromuscular Disorders

Neuroscience & Biobehavioral Reviews

Neurotherapeutics (NeuroRX)

Pain

Parkinsonism and Related Disorders

Pediatric Neurology

Regional Anesthesia and Pain Medicine

Seizure: European Journal of Epilepsy

Sleep Medicine

Sleep Medicine Reviews

Surgical Neurology

The offi cial journal of the World Federation of Neurology THE journal for the prompt publication of studies on the interface between clinical neurology and the basic sciences.And did you know.. Journal of the Neurological Sciences is your ultimate resource for the latest developments and research on Vascular Dementia, Stroke and Multiple Sclerosis!

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09_13wfn10_1.qxp 1/25/2010 2:31 PM Page 13

14 • WORLD NEUROLOGY WWW.WFNEUROLOGY.ORG • FEBRUARY 2010

HIGHLIGHTS FROM THE JOURNAL OF THE NEUROLOGICAL SCIENCES

Atopic Myelitis Seen in Korean PatientsSpinal cord

disease, ormye lopa -

thy, can be a di-agnostic andtherapeutic chal-lenge. The spec-trum of causes ofmyelopathy isbroad and in-

cludes trauma, nutritional deficiency(such as vitamin B12 and copper defi-ciency), viral infections (HIV and humanT-lymphotropics, herpes and West Nileviruses), bacterial infections (Treponemapallidum), parasitic infections (schistoso-miasis), metabolic diseases (hepaticmyelopathy), autoimmune diseases(multiple sclerosis, systemic lupus ery-thematosus, Sjogren’s syndrome, vas-culitis), and other inflammatory process-es (sarcoidosis). Some of these are quitecommon; others quite rare. For a sub-stantial proportion, no cause is ever de-termined.

Myelitis, or inflammatory myelitis,often has an autoimmune or dysim-

mune basis, although the precise triggeris not usually known. The difficulty iscompounded by the fact that mostforms of myelitis are clinically very sim-ilar and there are few easy clues to thecause. An exception would be the coin-cidence of weakness and numbness oc-curring simultaneously with a clear-cutzosteriform rash; the diagnosis is theneasily made.

A novel form of myelitis was first de-scribed by researchers in Japan in 1997,in which four patients with atopic der-matitis developed a sensory myelopathy,with an increased IgE level in the blood,as well as abnormal signal in the cervi-cal cord ( J. Neurol. Sci. 1997;148:199-203).

The IgG index was normal, and therewere no oligoclonal bands in the cere-brospinal fluid. The IgE antibodies weredirected to mite antigens, and the pa-tients developed this disease at a time ofyear when the mite population increasedin Japan. More Japanese patients were re-ported in the next few years, a minorityof whom had motor manifestations as

well. The question arises: Is this emerg-ing myelitis (“atopic myelitis” [AM]) con-fined to Japan, or is it also present else-where in the world?

In the current paper, Dr. In Soo Jooand his colleagues at Ajou UniversityCollege of Medicine, Suwon, South Ko-rea were able to identify several cases ofatopic myelitis in Korea ( J. Neurol. Sci.2009;285:154-8).

Characteristics Echo Those in JapanThe cases were recruited from idiopath-ic myelitis cases in the Ajou MyelitisRegistry database, and of 29 idiopathiccases, 14 had AM with hyperIgEemia andmite-specific antibodies.

The clinical characteristics were verysimilar to those seen in the Japanese pa-tients, with a relatively benign clinicalcourse and no clear response to intra-venous steroids. Comparisons weremade between AM and non-AM idio-pathic myelitis. AM was primarily sen-sory, with no or mild weakness, and last-ed several months instead of severalweeks.

Dr. Joo, the corresponding author, is afull professor at the College of Medicine.He became interested in AM when hestarted seeing an increasing number ofcases similar to those appearing in Japan.He notes that atopic diseases have gen-erally become more common in Koreaand seem to be following changes in theenvironment and diet, possibly mirroringsimilar changes in atopic diseases else-where in the world.

He speculates that AM is more like theopticospinal form of multiple sclerosis(MS) or neuromyelitis optica than themore usual relapsing-remitting MS.

Dr Joo has an interest in neuromus-cular and spinal cord diseases, especiallyamyotrophic lateral sclerosis (ALS), andis involved in designing studies of genetherapy (using stem cells) in treatingthese diseases. ■

DR. TSELIS is an associate professor ofneurology at Wayne State University inDetroit, U.S.A. He is the book review editorfor the Journal of the NeurologicalSciences.

BY ALEX TSELIS, M.D.,PH.D.

Aseries of potential biomarkers ofHuntington’s disease (HD) might al-

low for much earlier diagnosis of the dis-ease, improved treatment and monitor-ing of disease progress, and more reliableend points for therapeutic trials.

Treatments for HD “[lack] sensitiveand stable markers of the changes thatoccur in subjects carrying the HD mu-tation as they pass from what is cur-rently known as the premanifest stage[when they show no obvious clinicalsigns of disease] to conventionally diag-nosed HD [which relies on clear motorsigns being visible] and its progression,”said Dr. Sarah Tabrizi, of the Institute ofNeurology, University College London.

“[We hope] to confirm the existenceof biomarkers of disease progressionfrom the very early premanifest stage;indeed, our work so far confirms thatneuronal dysfunction begins manyyears before [Huntington’s disease] isclinically diagnosed,” she said.

In their baseline report of what willbe a 3-year multinational study, the re-searchers presented data on 366 indi-viduals: 120 carriers of the HD muta-tion in the premanifest stage, dividedinto two groups based on the mediannumber of predicted years (10.8 years)to diagnosis (preHD-A or further fromdiagnosis, and preHD-B or closer to di-agnosis); 123 patients with early HD, di-vided into two groups based on func-tional capacity (stage 1 HD or HD1, andstage 2 HD or HD2); and 123 age-matched and sex-matched controls.

These different groups provided a rangeof stages of HD spanning many years(Lancet Neurol. 2009;8:791-801)

All of the participants underwent 3TMRI brain scans and a range of assess-ments, including recording the hori-zontal eye position, an isometric forcetest involving sustained tongue protru-sion, a self-pacedfinger tappingtest, gait analysis,cognitive assess-ment includingnegative facialemotion recogni-tion, a visualworking memorytest and smellidentification test,and a neuropsychiatric assessment andquality of life evaluation.

“Significant differences were seen be-tween the groups in many of thesetests, reflecting progression of the dis-ease,” said Dr. Tabrizi. “The results alsosupported the idea that neuronal dys-function or loss occurs years before aconventional clinical diagnosis is usual-ly made.”

For example, the 3T MRI scans showeda 0.8% reduction in brain volume in thepreHD-A group, compared with the con-trols, progressively rising to 8.5% in theHD2 group. White matter loss and atro-phy of the caudate and putamen was ev-ident even in the preHD groups.

Similarly, the variability of tongue forceexerted by the preHD-A patients showed

a significant deficit, compared with thecontrols, becoming progressively worsewith disease progression (adjusted meansper subgroup and confidence interval:controls 3.27 [3.18-3.36], preHD-A 3.46[3.33-3.58], preHD-B 3.68 [3.55-3.81],HD1 4.32 [4.21-4.43], HD2 4.45 [4.29-4.61]). The same trend was seen for the

smell test results,the visual memoryresults, and theoculomotor anti-saccade error rate;problems alreadypresent in the pre-HD-A patientsworsened towardthe HD2 group.

“The identifica-tion of biomarkers is likely to have a ma-jor impact on the capacity to performhigh-quality clinical trials in HD,” com-mented Anne Rosser, Ph.D., of the Uni-versity of Wales, Cardiff.

Dr Rosser added that “although thecurrent well-validated battery of semi-quantitative clinical outcome measureshave played a critical role in clinical stud-ies to date, they tend to be laborious,time consuming (and thus expensive),and subject to rater variability and con-siderable noise.

“Biomarkers that track disease pro-gression will substantially increase thepower of clinical trials in manifest dis-ease and will make possible trials inpresymptomatic patients that wouldotherwise be impractical due to the

very large numbers of subjects and longfollow-up required. It is also importantto recognize that some biomarkers mayprovide new insights into the patho-genic processes in HD, an understand-ing of which is the cornerstone of de-signing new treatments,” Dr. Rossercommented.

Roger Barker, Ph.D., of the Universi-ty of Cambridge (England), noted that“[it will] be of interest to see how muchthese markers of disease change overtime, how linear these changes are, andthe variability of them between differentindividuals.

“If the more simple markers identifiedin this study change over time in all pa-tients in a reproducible way, and truly re-flect the core pathological events in thedisease, then for the first time we havemeasures by which to look for diseasemodification with novel therapeuticagents in HD,” he continued.

If their potential is borne out by thestudy’s longitudinal results, then thesemarkers could also have an importantimpact on routine clinical testing forHuntington’s disease.

Most of the tests required are inex-pensive and easily performed by trainedpersonnel. Many more clinics mighttherefore be able to conduct them andultimately provide better care for HDsufferers.

—Adrian Burton

Mr. Burton is a freelance writer for TheLancet Neurology.

Neuronal loss ordysfunctionpossibly occursyears before aconventionalclinicaldiagnosis.

DR. TABRIZI

FROM THE PAGES OF THE LANCET NEUROLOGY

Biomarkers Could Flag HD Progression From Premanifest Stage

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FEBRUARY 2010 • WWW.WFNEUROLOGY.ORG WORLD NEUROLOGY • 15

A Dizzy Concoction of Fugu, Sake—and HaikuThe annual meeting of the Japanese Society of

Clinical Neurophysiology was held in Kokura, onKyushu island, in October 2009. The meeting, or-

ganized by the society’s presi-dent, Prof. Sadatoshi Tsuji, was agreat success, as usual, but thistime there was a special treat. Af-ter a series of superb scientificsessions, we had a welcome ban-quet as part of typical Japanesehospitality. The local food servedincluded seasonal raw fish, fugu,for which this is the season, andKyushu is the place.

Now there have been a few reports of fatality asso-ciated with eating improperly prepared fugu becauseof tetrodotoxin, a poison that the fish makes, pre-sumably to scare away its enemies. But these reportshave not frightened the Japanese, who consider this adelicacy. However, there was enough concern that thetopic of the toxin dominated the conversation. Dr.Hans Lüders, of Case Western University, Cleveland,Ohio, U.S.A., was an invited guest at the meeting, andalthough he had been a neurology resident at KyushuUniversity some years ago, he did not know muchabout the fish, probably because at the time he couldnot afford it.

Dr. Ryuji Kaji, a WFN trustee, was able to elaborateon the fugu, a funny-faced fish also known as a puffer fish.This most expensive cuisine in Japan could present a riskychallenge for a cook who does not know physiology.

According to Ryuji, a self-proclaimed gourmet, fuguis palatable for chewing but tastes bland unless it con-tains a trace amount of the tetrodotoxin, which is asodium channel blocker. After the death of a well-known performer of Kabuki (a traditional Japaneseform of theater) with this type of poisoning some 35years ago, the Japanese government tightened the reg-ulations governing the cooking of fugu and the quali-fications of the chefs who prepared it to ensure the re-moval of the liver, which contains the toxin.

A tiny bit of the toxin that escapes, however, makesit a local delicacy, causing a tingling of the tongue anda slight numbness around the mouth while chewing.He concluded that the risk was small at our party, whichwas attended by physiologists and physicians familiar

with the diagnosis and treatment (unless the toxinwould kill us all).

Dr. Hiroshi Shibasaki, the President of the Interna-tional Federation of ClinicalNeurophysiology, who ispreparing for this year’s Inter-national Congress of ClinicalNeurophysiology in Kobe,Japan, noted that the safestway to proceed is to have oneguest eat the fugu first, andthen, if he survives for half anhour, the rest of the guests caneat it with less concern. As a

matter of pride, of course, if a chef does knock off oneof the guests, he must commit suicide.

Ryuji then posed a scientific question. If tetradotoxinblocks the sodium channel, it should cause conductionblock of motor and sensory fibers, thus causing paral-ysis and loss of sensation—not positive signs like tin-gling. But, no one had a clear explanation and no fur-ther scientific exchange ensued as, by then, no one wasable to carry on meaningful discussion under the in-fluence of sake.

I had also asked at a local restaurant if it is difficultto remove the toxin for safe consumption. The chefreplied: “No, not at all; all you have to do is to get ridof the liver which contains the toxin. Anyone can dothis without difficulty.” So I asked why we need a li-censed cook to do just that. “Because a good cook keepsa piece of liver inside. Some leave one quarter, others,one third, and still others, nearly one half so that youfeel a slight tingle when you bite the fish. Trouble is, ifit tingles too much, you may not wake up next morn-ing,” he replied.

This information inspired Dr. Mark Hallett, anotherguest at the meeting (and the editor in chief of WORLD

NEUROLOGY) to write a haiku, which is a Japanesepoem of three lines, with a syllabic structure of 5-7-5:

That was good fuguMy tongue was tingling stronglyNow syounara

Analyzing these verses with his usual critical eye, Hi-roshi commented that this was not a proper haiku,

which is a serious poem that also must contain some-thing about the seasons. Instead, the poem was a sen-ryu, a more cynical one, usually with a hidden message.

Shortly after Mark took over WORLD NEUROLOGY, hecreated a new column, Neurological Story, and askedme to submit one on any topic of my choice. I said Iwould think about it, which was a big mistake. He tookit to mean “Yes,” or so he implies, as all good editorsdo. Ever since, he has made me feel like I owe him anarticle every time we meet. I have, therefore, been try-ing to avoid him. This, however, has proven difficult.With this submission, I have fulfilled my obligation,which has been bothering me for some time. Accept-ed or rejected, I no longer owe him an article. ■

Editor’s note: I continue to welcome Neurological Stories.If any one can explain the tingling with tetrodotoxin, wewould all be grateful. Delivering the answer in the form ofa haiku will gain extra credit. Jun, a former WFNpresident and outstanding scientist and educator, is verybusy as a popular lecturer all around the world, and thishas inspired another senryu:

The measure of manIs not his achievements, butFrequent flier miles.

BY JUN KIMURA, M.D.

Dr. Kimura isprofessor of neurologyat the University ofIowa, Iowa City,U.S.A., and professoremeritus, KyotoUniversity, Japan.

B Y K E R R I WA C H T E R

Else vier Global Medical Ne ws

N E W Y O R K — Carotid endarterecto-my was deemed safer than carotid arterystenting for symptomatic patients basedon results from a multicenter study of1,710 patients, although postprocedurecomplications suggest that as stent tech-nology evolves, the two approaches willneed to be revisited.

The International Carotid StentingStudy found there were twice as manystrokes (58) for carotid artery stent (CAS)patients in the per-protocol 30-day analy-sis vs. 27 in the carotid endarterectomy(CEA) patients. Furthermore, 72 CAS pa-tients had a stroke, MI, or had died at 120days of follow-up vs. 43 (5.1%) in the CEAgroup, Dr. Frans Moll said at the Veithsymposium on vascular medicine spon-sored by the U.S.-based Cleveland Clinic.

However, “the complications occurrednot so much during [stenting] but at 1-3days after the procedure,” he said in an in-terview. “This brings me to the conclusionthat [perhaps] some technical features ofthe stent are not yet as good as we wishthey were.” Perhaps “the development ofstent technology has not reached the lev-el necessary to replace traditional surgicalskills,” said Dr. Moll, a professor of vas-cular surgery at the University MedicalCenter in Utrecht, the Netherlands.

In this study, patients with sympto-matic carotid artery stenosis greater than50% were randomized to treatment withCAS (853) or CEA (857). To be included,patients had to be deemed as requiringtreatment and the stenosis had to besuitable for both stenting and surgery. Ul-trasound study of the carotid artery tobe treated was performed at or beforerandomization and at 1 month following

treatment—and will continue annually.Participating surgeons had to have per-

formed more than 50 CEA or 50 CAS pro-cedures—and more than 10 cases/stentsper year—at supervised centers in thestudy. Several stents were approved for usein this trial. All patients received best med-ical care including antiplatelet therapy oranticoagulation (as appropriate) and con-trol of medical risk factors. Aspirin plusclopidogrel were provided before stenting.

The researchers were able to analyzethe 853 CAS patients and 857 CEA pa-tients by ITT up to 120 days post ran-domization. The per-protocol analysisincluded 821 patients in the CEA groupand 828 in the CAS group. In terms ofsecondary outcomes at 120 days more pa-tients in the CAS group had any stroke(65), compared with the CEA group (34).The hazard ratio for any stroke or deathfor CAS vs. CEA was 1.91.

In an MRI substudy involving 108 CASpatients and 92 CEA patients at five cen-ters, “we see that there is a real differencebetween CAS and CEA” at up to 6 weeks’follow-up, said Dr. Moll. In terms ofnew ischemic lesions seen on diffusion-weighted MRI after the procedures, theodds ratio for CAS vs. CEA was 5.24.

“The number of serious strokes wasnot so much different—disabling strokeswere not the biggest difference—but allof these minor strokes and lesions on dif-fusion-weighted imaging were striking,”Dr. Moll said in an interview.

Notably, protection devices were rec-ommended for use during CAS but werenot mandatory. A total of 245 patientsgot CAS without a protection device, andthe remainder had protection. Therewas no significant difference in outcomesregardless of whether a protection devicewas used, Dr. Moll said. ■

Carotid Endarterectomy Deemed Safer Than Stenting

The puffer fish (fugu) is guaranteed to cause tinglesin diners—and perhaps even inspire them to poetry.

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NEUROLOGICAL STORY

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