world neurology · here are a few brief notes on the ... the newsletter of the world federation of...

The WFN is delighted to announce thatthe Japan Foundation for Neuroscienceand Mental Health has made a substan-tial grant to the Federation to fund theproduction and distribution of its newslet-ter, World Neurology. We are very grate-ful for this generous contribution whichwill help safeguard our prime means ofcommunication with the 24,000 or soindividual neurologists worldwide whobelong to the 90 national neurologicalsocieties that comprise our membership.By way of background on the JFNMH,here are a few brief notes on theFoundation’s eight-year history.

In 1986, the Japanese government,recognising the growing importance ofthe impact of mental, nervous systemand muscle disorders on current andfuture society, established the NationalHospital for Mental, Nervous andMuscle Disorders. It was to be anadvanced national medical centre onthe model of the National Cancer andCardiovascular Centre, which has asits mission the provision of the mostadvanced medical treatment, the pro-motion of state-of-the-art research,and specialist training.

But it was soon acknowledged that gov-ernment institutions alone could not doall that was required to make substantiveprogress in these fields; and so it wasthat in September 1995, in response torequests from researchers, patients, andpatients’ families, the Japan Foundationfor Neuroscience and Mental Health wascreated with the full support of compa-nies and institutions involved in this areaand with the aim of contributing signifi-cantly to the improvement of public

health and wel-fare.

Dr Eijiro Sato-yoshi servesthe Foundationas President,and Dr. Kiyo-hisa Takaha-shi, a psychia-trist, takes careof the officemanagementin his capacityas Chairman.B a s e d i nT o k y o , t h eF o u n d a t i o nhas the follow-ing functions and roles to support variousactivities conducted by researchers,technicians, other front line medicalworkers, and patients and their families:

1. Supporting Investigation andResearch: publicly advertised support fororiginal investigation and research car-ried out by young researchers. 2.Supporting International ScientificExchange: programmes to dispatchJapanese researchers overseas, or toinvite overseas researchers to visitJapan, etc. 3. Supporting ScientificMeetings: organizing and promotinginternational congresses and researchmeetings held in Japan 4. SponsoringMedical Education: training of youngresearchers, scientists, medical workers,etc. 5. Publicity Work: disseminatinginformation (knowledge) of mental andneurological disorders through publica-tions.

As a charitable, non profit organization,the WFN shares the same vision as theJapan Foundation for Neuroscience andMental Health and is happy to draw tothe attention of all neurologists the splen-did work done by them. The WFN wouldalso like to express the hope that our col-laboration will be ongoing and mutuallybeneficial.

Visit the WFN website at http: / /www.wfneurology.org

V O L U M E 1 8 , N U M B E R 3 , S E P T E M B E R 2 0 0 3

T h e 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

W O R L DNEUROLOGY

ALSO IN THE ISSUE:! Editorial

! President’s Column! WFN CME Programe

! WFN Publication & WebsiteCommittee Report

! WFN Junior TravellingFellowship Reports

! Regional News! Book Reviews & Calendar

WFN President Jun Kimura, Trustees, officials and Council ofDelegates at Sydney Convention Centre for the Planning Meeting, (July 5-6, 2003) of 2005 World Congress of Neurology

JAPAN FOUNDATION FOR NEUROSCIENCE SUPPORTS‘WORLD NEUROLOGY’

EDITOR-IN-CHIEFDr. Jagjit S. Chopra, # 532 Sector 33-B,Chandigarh-160 047, India. Fax: +91-172-2665532E-mail: [email protected], [email protected]

EDITORIAL ADVISORY BOARDDr. Richard Godwin-Austen, World Federationof Neurology, 12 Chandos Street, LondonW1G 9DR, UKDr. Robert B. Daroff, Case Western ReserveUniversity / University Hospitals of Cleveland,Department of Neurology, 11100 EuclidAvenue, Cleveland, OH 44106-5015, USADr. Jun Kimura, 1203 PCU, 169 Ujimyoraku,UjiShi, Kyoto 611-0021, JapanDr. Theodore Munsat, Department ofNeurology, New England Medical Center, Box314, 750 Washington Street, Boston, MA02111, USADr. Johan Aarli, Department of Neurology,University of Bergen, Haukeland Hospital, N-5021 Bergen, NorwayDr. Julien Bogousslavsky, Service deNeurologie, Centre Hosp. Univ. Vaudois(CHUV), CH-1011 Lausanne, SwitzerlandDr. William M Carroll, c/o AAN Secretariat, 145Macquarie Street, Sydney, NSW 2000,AustraliaDr. Roberto Sica, Neurology / Buenos AiresUniversity, Hospital Ramos Mejia, Urquiza609, Buenos Aires 1221, ArgentinaDr. Daniel Truong, The Parkinson’s & MovementDisorder Institute, 9940 Talbert Avenue, Suite204, Fountain Valley, CA 92708, USA

REGIONAL VICE PRESIDENTSDr. S.M. Al Deeb (Pan-Arab), Saudi ArabiaProf. Dr. Leontino Battistin (Pan-European), ItalyDr. Pedro Chana (Pan-American), ChileUruguay Prof. Najoua Miladi (Pan-African),Tunisia Dr. Jin Soo Kim (Asian-Oceanian),South Korea

ASSISTANT EDITOR Dr. I.M.S. Sawhney, Department of Neurology,Morriston Hospital, Swansea SA6 6NL, UK;e-mail: [email protected]

WFN ADMINISTRATOR Keith Newton, World Federation of Neurology,12 Chandos Street, London W1G 9DR, UK

PUBLISHING STAFFPublisher:

Peter F. Bakker ([email protected])Marketing Manager:

Joyce HobbelinkDesign and Layout:

Desh Deepak KhannaOperational Support:

Annemieke van Es

ADVERTISINGPlease send inquiries about advertising in WorldNeurology to the Advertising Department, ElsevierScience Ltd., The Boulevard, Langford Lane,Kidlington, Oxford OX5 1GB, UK. Phone: +44-1865-843 258; Fax: +44-1865-843 976; email:[email protected]

MANUSCRIPTSThe Editor is happy to receive unsolicitedmanuscripts or photographs for considera-tion, but cannot accept responsibility for anyloss or damage to such material. Manuscriptsshould be submitted in English, typed on

white paper using double spacing with mar-gins of at least 3 cm. Authors should submitmaterial on computer disk (Microsoft® Word®or plain ASCII format) whenever possible.Tables and figures should be separated fromthe text and should clearly indicate theauthor’s name. Colour photographs and illus-trations are encouraged.

EDITORIAL STATEMENTAlthough great care is taken to ensure accura-cy, the WFN and Elsevier Science BV cannotbe held liable for any errors or inaccuracies inthis publication. Opinions expressed are thoseof the authors. Elsevier Science BV, the Editor,the WFN or the Grantor cannot be heldresponsible for the validity of clinical treat-ments, dosage regimens or other medicalstatements made. Any dosage referred toshould be checked against the relevant datasheet for the product.

World Neurology, ISSN 0899-9465, is pub-lished by Elsevier Science BV, Molenwerf 1,1014 AG Amsterdam, the Netherlands; phone:+31 (20) 485 3358, fax: +31 (20) 485 3237; e-mail: [email protected]

REPRINTSReprint requests and all correspondenceregarding the journal should be addressed tothe Editor. However, back issues of WorldNeurology can be obtained from the publisher.

CHANGE OF ADDRESSNotice of change of address should be sent to:World Neurology, Editorial Secretariat, 12 ChandosStreet, London W1G 9DR, UK. Fax: +44 20 73234012; e-mail:[email protected]

Printed by Kay Dee Associates at ChandikaPress Pvt Ltd, 126 Industrial Phase-1,Chandigarh- 160002, India. Fax: +91-172-2657783. Ph: +91-172-3203401

World Neurology2

W O R L D N E U R O L O G Y , V O L U M E 1 8 , N U M B E R 3 , S E P T E M B E R 2 0 0 3


PUBLISHING INFORMATION

T h e 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

W O R L D

NEUROLOGYV O L U M E 1 8 , N U M B E R 3 , S E P T E M B E R 2 0 0 3

CONTENTS

Japan Foundation for Neuroscience supports ‘World Neurology’ 1

Editorial . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

President’s Column . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4

WFN CME Program . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6

WFN Publications and Website Committee Report . . . . . . . . . . . . 8

WFN Junior Travelling Fellowship Reports . . . . . . . . . . . . . . . . . . 9

Neurofilament Light Mutations and the Pathogenesis ofCharcot-Marie-Tooth disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . .10

Regional News . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .12

Book Reviews . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .13

Calendar . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .16

COPYRIGHT © 2002 World Federation of Neurology. All rights reserved.Published by Elsevier Science BV, Amsterdam, the NetherlandManuscripts accepted for publication become the copyright of the WorldFederation of Neurology (WFN). Before publication a copyright form will be supplied by the Publisher, which must be completed by all authors.

World Neurology is the voice of the WorldFederation of Neurology, helping to pro-vide its membership with updated infor-mation; but the printing and mailing of aquarterly newsletter has been a majorarea of expenditure for the Federation.The magnificent grant from the JapanFoundation for Neuroscience towardsthe production and distribution costs of‘World Neurology’ will be a great help inthis respect. The Japan Foundation hasrecognized a need to make commoncause with the WFN by supporting thispublication for the ultimate aim of reliev-ing the neurological burden in society,which is one of the WFN’s major goals,particularly in the developing countries.The training of physicians from thesecountries too is an ongoing priority for theFederation and the funds saved throughthe Japan Foundation’s generosity cannow be diverted towards the training andeducation of physicians/neurologists inthese underprivileged parts of the world,and for research purposes. It is hopedthat this relationship between the WFNand the Japan Foundation will be a longlasting affair and credit for this wonderfulassociation between the two goes toPresident Jun Kimura, Dr. EijiroSatoyoshi and Dr. Kiyohisa Takahashi,who deserve our appreciation and grati-tude.

The Planning Meeting for the XVIIIthWorld Congress of Neurology in 2005and the Council of Delegates meetingtook place in Sydney in July 2003. Theywere very important sessions. The WorldCongress is expected to be a great suc-cess in this beautiful city. The arrange-ments being made by the local organiz-ers of this Congress are first rate and thescientific programme promises to besuperb. An added attraction is the prox-imity to the city centre of the SydneyConvention Centre, which has excellentfacilities.

Jun Kimura has given brief accounts ofwhat transpired at Sydney in hisPresident’s Column. Apart from theCouncil of Delegates meeting and acombined meeting with local organizers,there were also some other importantmeetings. A separate report, on themeeting of the Publications and WebsiteCommittee, appears in this issue. Theother important meeting held in Sydneywas of the Structure and FunctionsCommittee chaired by JulienBogousslavsky. This Committee is con-sidering in detail the future management

of WFN affairs—whetherby a continuation of theLondon Office at 12Chandos Street orthrough the engagementof a professional associ-ation management com-pany with the prospect ofbetter management andproper fundraising—andthe pros and cons werefully discussed. A num-ber of proposals havebeen received from pro-fessional organizationsand have been scruti-nized and analyzed, par-ticularly with regard to thefinancial implications and possible bene-fits. It will take some further discussionbefore the final proposal is handed overto the WFN Trustees.

The six-monthly report on the WFN’sCME programme makes exciting read-ing. This programme is proving fruitfuland, as one of the most successful WFNprogrammes, is being expanded. Manycountries are reaping the benefits andthe comments from the participatingcountries are self-explanatory. Thosewho are managing the programme underthe leadership of Ted Munsat deserve agreat deal of praise for their efforts to pro-vide educational materials to those inneed.

The short manuscript in this issue on thepathogenesis of Charcot-Marie-Toothdisease shows the latest developmentsin our understanding of this disease. Weare grateful to the authors of this articlefor accepting our invitation to submit thiscontribution.

The Seattle-based Allen Institute for BrainSciences is a leader in the efforts to pin-point the genes responsible for buildingand operating the human brain. Thegenome of the common mouse is akin tothe human genome, which containsabout 30,000 genes. Strange when welook at it but finding genes in mice whichcontrol the development and functions ofthe brain may help researchers to pursuestudies on the treatment of neurologicaldiseases by experimenting with drugs onmice. Paul G. Allen has remarked that, “ifwe can understand gene expression inthe brain, we can learn so much aboutneurological diseases and the geneticcomponent of human behaviour, likeemotions”. The team is trying to identifythe genes which are active in differentregions of the brain, a very difficult under-taking since an estimated two thirds of

mouse and human genomes play somefunctions in brain activity. It is hoped thatthe efforts of this team of researchers willsucceed in the gene expression map ofthe brain. It will help scientists to detectneuronal functions which become affect-ed in neurological and mental disorderssuch as Alzheimer’s disease and schizo-phrenia. The Brain Atlas expected fromthe Allen Institute will be different fromothers. The latter are based on brainscans and not on the gene expressionmaps being charted by Allen Instituteworkers.

Is the long term use of mobile phones ahealth hazard, particularly for the brain?This question is being asked in this sci-entific world in which technical advanceshave become an essential part of life. It ispredicted that 20-60 percent of the ener-gy emitted from a mobile phone isabsorbed by the user’s head, heating upthe brain’s structures. It has been shownthat the rate of deaths from brain canceramong hand-held phone users is higherthan those who use non-hand-heldphones. Brain tumours develop more onthe side of the head to which the personheld his phone, with most tumours beingacoustic neuromas. Mobile phone radia-tion can also destroy brain cells, whichmay lead to early Alzheimer’s disease. Itis said that pre-adolescent children arepotentially more at risk. Perhaps a longterm scientific study is necessary toprove or disprove these observations ofthe health hazards from mobile phones,but in the meantime the increasing use ofmobile phones amongst schoolchildrenneeds to be curtailed before we know thefinal outcome of this study.

Jagjit S. Chopra, FRCP. PhDEditor-in-Chief

EDITORIAL 3



EDITORIAL

WFN Structure and Functions Committee Meeting atSydney. Left to right: Roberto E. Sica (Argentina), Frank M.Yatsu (USA), Michael Donaghy (UK), Jagjit S. Chopra(India), and Chairman Julien Bogousslavsky (Switzerland)

PRESIDENT’S COLUMN4



We held the Annual General Meeting(AGM) on July 6th in conjunction with theplanning meeting for the World Congressof Neurology (WCN) 2005, which washosted by the local organizers in Sydney.

The planning meeting went as well asexpected thanks to the meticulouspreparation by William Carroll, CongressPresident, Geoffrey Donnan, CongressChair, Samuel Berkovic, Chair ofScientific Program, Stephen M. Davis,Chair of Education, John Morris, Chair ofLocal Organizing, and other dedicatedmembers of the local OrganizingCommittee. Roger Rosenberg, Chair ofthe WFN Research Committee, andChris Kennard, Secretary-General ofWCN 2001 held in London, coordinatedthe joint effort representing the WFNmembers of the Organizing Committee.In this report, I will not dwell on theprogress we have made in planning theWCN 2005, except to say that I wasimpressed by the effort and determina-tion to develop a highest caliber scientif-ic program and the most interestingsocial events, which should attract alarge number of participants to Sydneyon November 5-11, 2005.

Keith Newton, our Administrator, with thehelp of Susan Bilger, spent an enormousamount of time in organizing the AGM,which is now required under theCompanies Acts. We were so con-cerned that the SARS scare and the fearof global violence might prevent interna-tional travels that Keith had a contin-gency plan to meet at Helsinki if Sydneyfailed. Thus, I am particularly pleased toreport that the AGM also went well to thesatisfaction of all national delegates whoparticipated. We were able to achieve aquorum for the Council of Delegates(COD) meeting, which was attended by18 national delegates carrying 15 proxiesgiven to the participants of their choice. Iwould like to highlight the main topics ofdiscussion, and describe a perspectivefrom my vantage point. A full report willshortly appear in the Web if you are inter-ested in a more detailed account.

According to the Articles of Association,which dictate our procedural principles,one of the three Elected Trustees mustretire at each AGM, the longest in officeretiring first. An Elected Trustee so retir-ing may be reelected. As all threeTrustees were elected simultaneously inLondon during the WCN 2001, the

choice was made by drawing lots for Dr.William Carroll from Australia to retire thisyear. In the election conducted at thebeginning of COD, he was re-elected asa Trustee out of five equally deservingcandidates. I am happy to have him onboard to maintain a close tie with theAustralian Neurological Association inour joint project to organize the mostsuccessful WCN 2005 in Sydney.

Dr. Michael Donaghy, on behalf of theAssociation of British Neurologists, madetwo separate but related motions regard-ing the role of the Nominating Committeeand the election process: 1) All candi-dates proposed as officers and trusteesshould be directly elected by the dele-gates on the basis of single transferablevoting, and 2) Two of the three electedtrustees should be nominated from thecurrent body of national delegates.

Dr. Frank Yatsu, former chair of theNominating Committee, attended theAGM to represent the current chair, Dr.Thomas Brandt, who was unable to par-ticipate. He explained to the COD thereasons why the Nominating Committeewas formed and how it was operated.Both motions were then discussed atlength before a call for questions, whichresulted in an overwhelming number ofvotes against the motions. The spirit ofthe proposals, however, was well takenand supported by the Trustees, who willmake concerted efforts to make theprocess more transparent, publicizingthe rules of the deliberations and theselection criteria for all those concerned.

A question was raised on how the mem-bers of the Nominating Committee were

selected. Like any other committeeappointments, the president solicitsnominations from different sources toprepare a geographically balanced slatebased on the candidate’s prior experi-ence and interest as well as past recordof excellence. All appointments areeventually approved by the Officers andTrustees as well as the Chairmen of therespective committees. Unlike the ordi-nary committees often chaired by one ofthe Trustees for close liaison with themanaging directors, the NominatingCommittee, once appointed, functionsindependently with no input from eitherthe Officers or Trustees to avoid any realor perceived conflict of interest. The pro-ceedings and decisions are held in pri-vate to avoid any outside pressures orinfluences.

The person in charge of the WFNaccounts departed our auditors withoutproperly notifying his successor of thedate of the AGM and the need to pro-duce the figures in good time.Consequently we experienced a majorproblem with timely preparation of theaccounts of the Federation for the lastfiscal year covering January 1, 2002through December 31, 2002. They man-aged to complete the work in a draftform, which Richard Godwin-Austen,Secretary-Treasurer General, presentedto the COD, detailing various insufficien-cies in the statements. The delegatesgave the draft a tentative approval withthe condition that the final audit be circu-lated amongst the national member soci-eties as soon as the work is completed.Given the unsatisfactory state of affairswith the financial report, the appointmentof auditors for the current year has still to

PRESIDENT’S COLUMN

WFN President, Trustees and officials at Council of Delegates meeting at Sydney—July6, 2003. Left to Right: Marianne de Visser, Julien Bogousslavsky, Johan Aarli, Jun Kimura(President), Richard Godwin-Austen, William Carroll, Keith Newton, Susan Bilger.

be finally confirmed.

Dr. Roberto Sica, as chair of theMembership Committee, recommendedthat the COD accept the MongolianAssociation for Neurology as a newmember of WFN. The motion was car-ried unanimously. I wish to be the first towelcome Professor D. Baasanjav and 40neurologists from Mongolia as the 90thmember society, and look forward todeveloping a mutually beneficial affilia-tion.

I should mention in passing that theMembership Committee has also beennegotiating with the ChineseNeurological Society that wishes to beaffiliated with the WFN. There was acause for optimism at the time of theVancouver AGM last year with the antici-pation that a formal application could bemade in time for this year’s COD. Wehave tentatively agreed to honorTaiwan’s existing membership and toadditionally recognize the ChineseNeurological Society and Hong KongNeurological Society as the national andregional representation respectively. Wehave discussed the possibility of giving asingle vote to each society consideringthat Hong Kong is regarded as theSpecial Administrative Region as it waswhen it belonged to the UK.

In September 2002, I visited with Dr.Chen, the President of the ChineseNeurological Society, to discuss thedetails of their constitution and remainingconcerns regarding the TaiwanNeurological Society and Hong KongNeurological Society. We also exploredvarious options to resolve another majorissue relating to the amount of dues that

the Chinese Society would be asked topay. While I was contemplating a sec-ond visit to Beijing earlier this year tofinalize the agreement, I was saddenedto learn that Professor Chen had passedaway. With her demise, we were unableto proceed with the anticipated arrange-ment to formally seek China’s entry to theWFN this year. The MembershipCommittee, now chaired by Dr. MariannedeVisser after the retirement of Dr.Roberto Sica, will renew the negotiation,which has been pending because of theSARS crisis. We certainly hope to bringthe Chinese Neurological Society into theFederation, although we firmly adhere tothe traditional position to remainabsolutely free of international politics.

Dr. Johan Aarli, the First Vice Presidentand Chair of the Public Relations andWHO Committee, led the discussionregarding the role of the regional vicepresidents (RVP), which is poorly definedat the moment. Of the five RVPs electedin 2001, Drs. Carlos Chouza (Pan-American), Dr. Leontino Battistin(European), Dr. Jin Soo Kim (AsianOceanian) participated in the discussion.Dr. Najoua Miladi (Pan-African), and Dr.Saleh Al Deeb (Pan-Arab) were unable toattend the meeting. Except for the roleon the Editorial Advisory Board of WorldNeurology under the editorship of JagjitChopra, the RVPs have no formal func-tions within the WFN. A consensusemerged that the RVPs should have amuch stronger basis to truly representthe respective regions. To establish thispractice, we need to have a more formal-ized relationship with each of the fiveregional groups. The RVPs should alsoserve as the contact for the regionalWHO offices to identify the needs unique

to the region for prevention of neurologi-cal disorders and for dissemination ofpertinent information. The fundamentalaim was to make the WFN integrated andclosely knit. We cannot radically alter thefunction of RVPs half way in their term,but, after further deliberation among theTrustees, we hope to present newlydefined functions of RVPs for approval ofthe COD at the AGM in 2004 so thatthose elected or appointed in 2005 willhave a more clearly defined role.

Dr. Richard Godwin-Austen discussedthe new concept of regional congressesthat would take place in the interveningyears between the World Congress ofNeurology at four-year intervals. Inessence, the Federation offers its sup-port to a regional organization or nation-al society to hold joint regional congress-es on the basis of combined planningand sharing of a profit realized. Thisdoes not apply to the regional organiza-tions or national societies who arealready arranging their own independentmeeting successfully. It is aimed to helpless affluent regions that might have agreat difficulty in hosting scientific meet-ings on their own. These conferencesshould develop scientific programs high-lighting the topics of special concern tothat particular area. Bids are now beinginvited from regional or national soci-eties. Any local organizers interested inthis arrangement should initially discussthe plan with the respective RVP beforecontacting the London office for furtherinstruction.

Mainly for logistic reasons, only a fewcommittees met in Sydney. Theseinclude Publications and WebsiteCommittee, chaired by Francois Boller,and Structure and Function Committeeunder the direction of JulienBogousslavsky. In the interest of time,we compressed the oral presentationfrom various committees at the CODmeeting. To complement the report, anexcerpt from World Neurology was circu-lated to detail the activities of each com-mittee. I will not reiterate the report,which is made available on the websitefor your perusal. All in all, I am happywith the progress each committee hasmade during the past two years. Majoradvances have been achieved but wehave the potential to move even further.

After the Sydney conference, I attendedthe 25th annual meeting of theColombian Association of Neurologyunder the able direction of Professor

PRESIDENT’S COLUMN 5



Meeting of Trustees, Officials, Regional Vice Presidents and Editor World Neurology atSydney—July 5, 2003

Gustavo Pradilla, on August 7-9, 2003.This most successful congress dealt withan appropriate mixture of various neuro-logical disciplines covered by a number

of local and international faculties. Allsessions were conducted in Spanish withsimultaneous translation for foreignspeakers. I was happy to see many old

friends and get acquainted with newones in Bucaramanga. The quadrennialWCN, now scheduled for November 5-11, 2005 in Sydney, serves as a mosteffective venue for presenting scientificachievements and interacting with dele-gates of varied backgrounds. We wouldlike to have a large number of partici-pants from different corners of the world.Unfortunately, however, not everyonecan attend such an international meetingbecause of fiscal and logistic constraints.Thus, the WFN is committed to workclosely with regional and national groupsin all parts of the world to improve com-munication with member societies. Assuch, local meetings, like the one organ-ized in Colombia, play an equally impor-tant role for the mission of the WFN inadvancing neuroscience and clinicalpractice, which may be unique to thearea.

Jun Kimura, MDPresident, WFN

WFN CME PROGRAM6



Six-monthly report:February 2003 - August2003The fifth mailing of Continuum of thetitles “Acute Stroke Management”,“Emergency Neurology”, and“Headache Update”, took place on 17July 2003 to 28 countries. As usualdemand for the journals continues to out-strip supply. Members are looking for-ward to studying the new titles, acknowl-edging their shipments with commentssuch as “truly excellent” (Uruguay) and“fantastic” (Cuba).

1. Participants—Twenty eight countriesare involved in the program at present.Eastern European countries continue toparticipate very enthusiastically and returnregular feedback on courses. Bulgariaand Romania submitted their first evalua-tion forms early in the year, and receivedalmost 80 certificates of participationbetween them. Turkey, Serbia &Montenegro (Yugoslavia), Slovenia andLithuania are also regular contributors.However, comments have also beenreceived from countries that have not pre-viously returned feedback includingGuatemala and Syria. If certificates of

participation are taken as a measure ofmembers’ increased activity, the programcan clearly be seen to be expanding. Todate, nearly 600 certificates have beenawarded, which exceeds the number forthe whole of 2002.

1.i. New members—Since the lastreport Argentina, Bangladesh andMexico have joined the program underthe coordination of Dr Federico Pelli, DrShariful Islam Sohel and Dr Lilia NunezOrozco respectively. All have shownenthusiasm in organising the program intheir countries. Bangladesh joined theprogram just prior to the July mailing.Innovatively for the CME program, thecoordinator, Dr Sohel, and theBangladesh Society of Neurology, held apress conference introducing the pro-gram to the medical community on 18August 2003. They have also distributedcopies of Continuum, and printed evalu-ation forms and discussion group guide-lines. With two national societies,Guatemala is now viewed as two sepa-rate countries for purposes of the pro-gram. Dr Luis Salguero remains coordi-nator for the Asociacion Guatemaltecande Neurologia (Guatemala 1), while DrHenry Stokes is coordinator for theAsociacion Guatemaltecan de CienciasNeurologicas (Guatemala 2).

1.ii Russia—In addition to coordinating

the program in Russia, Professor AllaGuekht, has also agreed to organise theprogram in countries of the former SovietUnion, Estonia and Azerbaijan for exam-ple. She will canvass these countries atthe All-Russian Society of Neurologists inMoscow during September. This adds toher already heavy workload, and ofcourse also places extra demand uponsupplies of Continuum. Russia has avery active program, and extracts from areport by Professor Guekht are givenbelow with her permission. The reportdemonstrates the excellent use beingmade of Continuum, its positive recep-tion in Russia, and the success of theprogram with a coordinator of the enthu-siasm and organisational skill ofProfessor Guekht.

1. iii Request for Increase in Dona-tion of Continuum—It is unusual forcountries’ requirements for Continuum tobe met from the current donation of 200copies of each Continuum title at thetwice-yearly mailings. Demand increas-es for what is considered by recipients tobe a very valuable learning resource.This, coupled with the fact that ProfessorGuekht is assuming the coordination offormer states of the Soviet Union, led usto make a request to the AAN to extendtheir already generous donation of 200copies per title, to 300 copies. Thisrequest was made via Andrea Weiss in

WFN CMEPROGRAM

Opening Ceremony (left to right) Gustavo Pradilla—President Organizing Committee VIColombian Congress of Neurology, Alvaro Beltran—President Universidad Industrial deSantander, Manuel Uribe—President Neurological Association, Jun Kimura—PresidentWorld Federation of Neurology, Carlos S. Uribe—Ex-President Colombian NeurologicalAssociation.

June for consideration by the EditorialBoard.

1. iv Non-participating countries—Although each country’s activity withinthe program is routinely monitored, adetailed review of each country’s activityis undertaken every six months. The lat-est review in May resulted in Poland,South Africa and Morocco being sus-pended from the program for the timebeing. We had received very littleresponse to our communications, andthey had not returned evaluation formson the courses they had been sent for avery long time, if at all. However, asusual, should they provide feedback at alater date and wish to re-join the pro-gram, they will be welcomed.

2. Feedback—The system of circulatingfeedback to members of Continuum fac-ulties continues, with comment fromevaluation forms being forwarded toAndrea Weiss for circulation to the facul-ties concerned.

Comments on Continuum continue to bevery positive and individuals constantlyemphasise the relevance of the informa-tion for their neurological practice. Allcourses are much appreciated, and arange of comments from the evaluationforms on various courses, follows foryour information and interest.

PARTICIPANTS’COMMENTSCourse: Tumours of the Brain andSpinal Cord—“This course was excel-

lent in adding new information about thetherapeutic approach for brain and spinalcord tumours ... and broadened our mindin genetics as a tool for early diagnosisand management.” (Egypt). “The coursewas very beneficial in adding much aboutthe diagnostic outlines on the geneticbasis, and also therapeutic outlines “ itadded much to our management experi-ence of tumours of the brain and spinalcord.” (Egypt). “I like the case reportsand the neuro-imaging in the text. Newinformation for me was the chapter onmolecular biology.” (Czech Republic).“These study group programmes shouldbe continued. They are very useful forme.” (Turkey). “The content is veryinstructive and appropriate for our prac-tice.” (Tunisia)

Course: Learning Disabilities—“Thestudy is very interesting, full of informa-tion that improves my knowledge aboutlearning disabilities. I liked it verymuch...” (Romania). “The content wasvery well systematised and very easy toapply in our everyday practice, and ofgreat interest to us as there are tests wedidn’t use very frequently til now. Wefound as very interesting the casedescriptions. May be a video/CD-ROMpresenting some cases and the appro-priate tests could be very useful for us.”(Romania). “The course was interesting...the material was far from our knowledgeand really we have not enough experi-ence with dealing with such items, so thecourse was so fruitful and we thank youfor sharing us your knowledge.” (Syria).

Course: Tropical Neurology—“Both

the material in the book as well asendeavour of participants is of extremelyhigh quality. This course helped us inenhancing the knowledge in this impor-tant, but sometimes neglected segmentof neurology. Overall, rating of thiscourse is extraordinary.” (Yugoslavia). “Iread each chapter of Tropical Neurologywith great interest. I was especially inter-ested in the chapter “Strokes in theTropics”. Along with reading this materi-al during the summer, which was very hotin my town in Serbia (40ºC), I treated thepatient who developed postapartalvenous thrombosis. Thus, this materialsignificantly helped me in establishingthe diagnosis.” (Yugoslavia)

Course: Disorders of CognitiveFunction—“Probably the bestContinuum course. Very picturesque,inspiring and interesting. I also liked thepractical approach.” (Slovenia). “Thebook is very well written and full of usefulexamples and illustrations. The accom-panying video is very informative andshows additional aspects which are noteasily presented in a text format.”(Slovenia). “We were all impressed withexcellent case studies and we agreedthat it is the best way for illustration ofcognitive and behavioural impairment.”(Yugoslavia). “The functional anatomy isdifficult to understand, but good, basicinformation The mental evaluation isexcellent and it will be translated for clini-cal use. The patient management prob-lems and appendices were very informa-tive.” (Guatemala 1). “The concept is dis-cussed in a concise and practical format.The information is useful in everyday neu-rological practice.” (Guatemala 2)

Course: Infectious Diseases—“Thereis a lot of useful information for clinicalpractice, some to refresh pre-existingknowledge and some to learn about it,The authors of Continuum courses pro-duced an excellent and comprehensivecoverage of nervous system infectionsthat is easy to read and understand.”(Yugoslavia)

Course: Iatrogenic Neurology—“Ilearnt a lot of things in iatrogenic neurol-ogy of which I had limited knowledge. Iparticipated in giving lectures on the top-ics. It was a great pleasure.” (Sri Lanka).“Very good overall rating. Excellent cov-erage on drug induced movement disor-ders, complication of anti-epilepticdrugs, and complications of chemother-apy/radiotherapy.” (Sri Lanka). “Content,excellent. Fulfilled a vacuum of knowl-

WFN CME PROGRAM 7



Destination Australia—Convention Centre for XVIIIth World Congress of Neurology,Sydney, 2005

edge.” (Guatemala 1).

Course: Sleep Disorders—“Case pre-sentations, very helpful. Approach todiagnosis and treatment, very useful.Insomnia description, too short.Emphasis on paediatric sleep disorders,excellent. Appendices, excellent!!”(Guatemala 1). “The content was excel-lent and useful for me as a clinical neu-rologist” the depth of the topics coveredis neurologically very educational. Whengoing through the references and bibli-ography, we feel it is up to date.”

(Guatemala 2)

Course: Neuroimmunology—“Patho-physiological aspects of diseases werethoroughly done with vast essentialknowledge being given. A little more clin-ical orientation might make this ventureperfect. Thank you.” (Sri Lanka).

NB: Although these are all positive com-ments, they are nevertheless truly repre-sentative of those received.

LIST OF PARTICIPATINGCOUNTRIES

Argentina, Bangladesh, Bulgaria, Cuba,Croatia, Czech Republic, Egypt,Guatemala(1), Guatemala(2), Honduras,Hungary, India, Jordan, Lebanon,Lithuania, Mexico, Peru, Philippines,Romania, Russia, Slovenia, Sri Lanka,Syria, Tunisia, Turkey, Uganda, Uruguayand Yugoslavia (Serbia).

Monica BroughWFN CME Program Manager

A meeting of the Publications andWebsite Committee was held July 5,2003 at the Regent (now Four Seasons)Hotel in Sydney in the context of a meet-ing held in preparation of the 2005 WCN.

The attendees included F Boller, JunKimura, Bill Carroll, Johan Aarli and JagjitChopra, as well as Keith Newton andSusan Bilger.

Two main topics were discussed: theweb site and the Journals.

Once again we must congratulate BentoGarcia de Sousa on the nice job done forthe upgrading and maintenance of theweb. It was proposed that an actual con-tract be written and signed concerninghis precise role. This will be prepared byFB with help from KN. Statistics are avail-able for each segment of the website,but they should be more accessible thanthey are now. The proposal of having aseparate (linked) page for each researchgroup was approved. The question of aforum was resolved in favor of “publicwith restrictions”. A considerable amountof discussion revolved around the relat-ed issue of obtaining e-mail addressesfor WFN members. As of now theLondon office has around 1500 address-es. Two strategies will be pursued inorder to get more. 1) All national dele-gates will be asked to send the mailinglist of their national societies. 2) DrKimura will approach the new AANPresident to see if WFN can get accessto the AAN list. After this is done, theLondon office will coordinate with theExecutive Director of AAN. 3) Thereshould be a “guest book” for all thosewho consult the website with the possi-bility of having one’s address not record-ed. Since the web is now open, a brief

questionnaire should be included in theguest book.

The following letter has been preparedand addressed to all WFN Delegates.

One of the recommendations thatemerged from the recent WorldCongress Planning Meeting, held inSydney, was that the WFN Central Officeshould collect the e-mail addresses of asmany neurologists as possible world-wide so that we may improve both theeffectiveness and rapidity of our commu-nications with our members. I am there-fore writing to ask you, as NationalDelegate, to please forward to theLondon Office all the addresses in yourpossession. Needless to say, they will be

treated with the utmost confidentialityand be used only to distribute material ofacademic interest or information relatedto WFN business. It would also be mosthelpful if you could ask your colleaguesand other contacts to do the same.

JournalsJNS—Again the issue of the revenuesgenerated by the Journal was discussed.It was decided that there should be ameeting that included Elsevier, FB andthe Secretary of the Finance Committee,Mark Hallett. One question to be clarifiedis the actual ownership of the Journal.The existing contract goes back to 1988(under Dr Masland’s Chairmanship) andthe question of ownership is not specifi-cally mentioned.

WFN PUBLICATIONS & WEBSITE COMMITTEE8



WFN PUBLICATIONS AND WEBSITE COMMITTEEA Report

WFN Publications and Website Committee Meeting—Left to right: (Sitting) Johan Aarli, JunKimura, Bill Carroll, (Standing) Jagjit S. Chopra, Keith Newton and Committee ChairmanFrançois Boller

World Neurology (WN).A major Japanese Foundation hasexpressed interest in supporting thisWFN project and WN has been proposedfor this purpose. One way to increaserevenues is to ask the organizers offuture meetings to include an announce-ment accompanied by a donation. This

applies to bidders for future WCNs, butalso to regional meetings which seeksponsorship from the WFN.

The Editorships of JNS and WN areup for renewal with the coming of a newAdministration in 2005. A suitable searchsubcommittee will be put in place. The

current Editors are eligible for renewal.Nothing is new concerning otherJournals. In particular, no word has beenreceived from Lippincott about ADAD.

François Boller, MD, PhDChairman Publications & WebsiteCommittee, Paris, France

WFN JUNIOR TRAVELLING FELLOWSHIP REPRTS 9



I was nominated for 2002GlaxoSmithKline Junior TravellingFellowships to attend the 6th Congressof the European Federation ofNeurological Societies in Vienna , Austria(October 26th-29th, 2002).

I am very sorry for such a long delay ofmy report. I am very grateful for the finan-cial support given by the WorldFederation of Neurology, which made itpossible for me to participate the con-gress. As my special field of interest con-cerns brain tumors, I participated in thework of neuro-oncology section. On thebasis of my abstract ‘Survival and prog-nostic factors of patients with brainglioma in Estonia’ the OrganizingCommittee proposed me to have a shortoral overview on this topic. This was myfirst presentation in such big and honor-able congress so I tried to do my best.The presentation gave me a good expe-rience and encouraged to continue my

work in this field. Besides above-men-tioned section I also participated in thelectures on cerebrovascular diseases,stroke treatment, multiple sclerosis,glioma treatment headache and others.Poster sessions gave me a goodoverview about several different field ofneurology and were the best place fordiscussions and meetings with col-leagues.

Especially, I liked the video-session ofmovement disorders where interestingcases were discussed. I enjoyed my stayin Vienna very much.

Aive Liigant, Dept. of Neurology & NeurosurgeryUniversity of Tartu 2 L. Punsepa St.51014 Tartu, Estonia.

I attended the 7th Conference of theEuropean Federation of NeurologicalSocieties- at Helsinki from 30th Aug. to

2nd Sept. 2003. I am very much thankfulto World Federation of Neurology togrant me the award of junior travelling fel-lowship in order to attend this great inter-national conference I ever attended. Ialso presented a paper on epidemiologi-cal survey of epilepsy in a defined geo-graphical area inside India and I got anaward of poster prize.There were someinteresting sessions like tournament ofjunior neurologist, quiz contests andteaching session of high academic qual-ity. The satellite symposium organised bydifferent pharmaceutical companieswere also highly informative.More than3200 delegates from the European coun-tries and rest of the world attended thisgreat congress and it was said that EFNSfor the first time saw this massive gather-ing. The 8th congress of EFNS was dec-lared to be held at Paris in Sept. 2004.

GP Burman,Hissar, India

WFN JUNIOR TRAVELLING FELLOWSHIP REPORTS

WINNERS OF THE WFN JUNIOR TRAVELLINGFELLOWSHIPS 2003Name Country MeetingTomislav Breitenfeld Croatia 128th Annual Meeting of the American

Neurological Association, San Francisco, USAOctober 19-22, 2003

Mercedes Bello Sanchez Cuba AAEM & IFCN Joint MeetingSan Francisco, September 16-20, 2003

Ana Margarita Chong Medina Cuba AAEM & IFCN, Joint Meeting,San Francisco, September 16-20, 2003

Alexander Rousseaux Lafargue Cuba AAEM & IFCN, Joint Meeting,San Francisco, September 16-20, 2003

G.P. Burman India 7th EFNS Congress, Helsinki, Finland,August 30-September 2, 2003

Achal K Srivastava India 25th International Epilepsy CongressLisbon, Portugal, October 12-16, 2003

Lodi Gogovska Macedonia 7th EFNS Congress, Helsinki, Finland,August 30-September 2, 2003

Irena Dujmovic Serbia & 7th EFNS Congress, Helsinki, Finland,Montenegro August 30-September 2, 2003

Ziad Adwan Syria 56th AAN Meeting,, San Francisco,April 24-May 1, 2004

NEUROFILAMENT LIGHT MUTATIONS10



Charcot-Marie-Tooth (CMT) disease isthe most common hereditary neuropa-thy, affecting approximately 1 in 1,000 ofthe general population. A number ofgenes have been identified as harboringmutations in CMT patients, althoughadditional chromosomal loci for which nogenes have yet been identified have alsobeen found. CMT is characterized by het-erogeneity at both the clinical and thegenetic levels. Mutations in differentgenes result in the same clinical pheno-type, and different mutations in the samegene can also result in different pheno-types.

Recently, mutations in the neurofilamentlight gene (NFL) have been identified inCMT patients. Neurofilaments (VFs) arethe main intermediate filament (IF) ofpost-mitotic neurons. There are three dif-ferent neurofilament subunits, heavy(NFH), medium (NFM) and light (NFL)according to their molecular weight. BothNFL and NFM are very closely linked onchromosome 8p21, and their expressionis coordinately regulated during develop-ment. NFH expression occurs later dur-ing development, after expression of NFLand NFM. Both in vitro biochemical data,as well as transient transfection experi-

ments, have demonstrated that only NFLcan self-assemble, while NFM need toform the core of the filament, with NFMand NFH responsible for the radialgrowth of the filaments. NFs have a tri-partite structure, with a central a-helicalcoil-coiled rod domain and globular headand tail domains. The rod domain is crit-ical for assembly of NFs, which startswith a dimerization step, followed by theformation of tetramers and subsequenthigher order structures. In neurons, NFsubunits are synthesized in the cyto-plasm and transported to the axons.During their axonal transport NFs arephosphorylated, in the tail domains ofNFH and NFM. Alterations in NFs havebeen identified in a number of neurologi-cal and neurodegenerative diseases. NFaggregation, mislocalization and aber-rant axonal transport, have beendescribed in Alzheimer’s disease,Parkinson’s disease, amyotrophic lateralsclerosis, diabetic neuropathy, and toxicneuropathies, among others.

The first NFL mutation identified in CMTwas a missense point mutation resultingin a Gln333Pro amino acid change. TheGln333 amino acid is located in the high-ly conserved region of the rod domain.

Substitution for the Pro residue intro-duces a Pro-kink that destabilizes the a-helical coiled-coil. The second NFLmutation was a double missense point-mutation resulting in a Pro8Arg aminoacid substitution in the head domain. Thehead domain of NFL has also beenshown to be important for filamentassembly, but the relative importance ofindividual amino acid residues is notknown. The assembly of NFs has beenshown to be regulated in part by phos-phorylation of the head domain.

Recently published data has identifiednew mutations of the NFL gene in otherCMT patients. The mutations are locatedin all three domains of the NFL gene. Inthe head domain of NFL new mutationsidentified are Glu7Lys, Pro8Gln, Pro8Leu,Pro22Ser, Pro22Thr, and Glu89Lys. Itappears that the Pro8 and Pro22 aminoacid residues may be mutational hot-spots. Newly identified mutations in therod domain of NFL are Asn97Ser andAla148Val. Two additional mutationshave been identified in the tail domain ofNFL, Asp469Asn and deletion of Glu528(delGlu528). The Asp469Asn amino acidchange had been previously identified ina study searching for NF mutations in

Neurofilament Light Mutations and the Pathogenesis ofCharcot-Marie-Tooth Disease

THE BRUCE S. SCHOENBERG INTERNATIONAL AWARD INNEUROEPIDEMIOLOGYIn tribute to Bruce S. Schoenberg, MD’s career in training neurologists internationally in epidemio-logic methods, this award salutes a young investigator selected from a developing country orEastern Europe.Endowed by GlaxoSmithKlinePPrreesseennttaattiioonn: Recipient is expected to give a 20-minute presentation based on the selectedabstract during a neuroepidemiology scientific session at the AAN 56th Annual Meeting in SanFrancisco, California.RReecciippiieenntt wwiillll rreecceeiivvee:: ! Certificate of recognition ! Complimentary registration for 56th AnnualMeeting, which will be held April 24-May 1, 2004 ! Reimbursement for 56th Annual Meeting traveland lodging expenses ! Recognition at 2004 Awards Luncheon at 56th Annual Meeting.EElliiggiibbiilliittyy:: ! Must be an investigator under the age of 45 at the time of submission ! Must be a per-manent resident of a developing country or Eastern Europe ! Must have played an important rolein epidemiologic research on a neurological disease.AApppplliiccaattiioonn PPrroocceedduurree:: Applicants should submit ONE complete set of the following materials.Nomination of a candidate is allowed but not required. ! Completed application form ([email protected] for copy of form) ! Current curriculum vitae including date of birth, training,and bibliography ! Maximum 500-word abstract in which applicant played an important role in epi-demiologic Program) ! Deadline: December 1, 2003.For more information, please contact Cecelia Adams at [email protected] or (651) 695-2788.

NEUROFILAMENT LIGHT MUTATIONS 11



ALS patients, but the amino acid changewas identified in both ALS patients andcontrols, thus suggesting that it could bea polymorphism and not a real mutation.It is interesting to note that so far most ofthe single nucleotide polymorphisms(SNPs) identified in the NF genes arefound in the non-helical tail domain of theNF subunits.

We have initially characterized the effectsof the different NFL mutations on assem-bly of NFs using a heterologous transfec-tion system in vitro. Both Pro8Arg andGln333Pro mutant NFL resulted in theloss of the self-assembly capability ofwild type human NFL [hNFL(wt)]. As itwould be expected in the case of a dis-ease with an autosomal dominant patternof inheritance, both mutations also dis-rupted wild-type NFL filaments. The twomutants had different effects on theassembly of heteropolymeric filamentscomposed of NFL and NFM. TheGln333Pro NFL mutant disrupted theassembly of NFL-NFM heterofilaments.Expression of this Gln333Pro mutantresulted in the formation of aggregates,in which NFM was found to co-localize. Incontrast, co-expression of the Pro8Argmutant with wild-type NFM resulted in theformation of filaments in most cells.However, these filaments were abnormaland had a high tendency to be very thickand bundled. The Asp469Asn hNFL vari-ant behaved in all cases in a similar fash-ion as wild-type hNFL. Alterations in the

NF network caused by NFL mutationscould disrupt axonal transport. Thismechanism is similar to that recentlydescribed for mutation of the kinesinmotor protein KIF1Bb, a mutation linkedwith CMT2A. Disturbances in kinesin-driven anterograde transport, especiallyof synaptic vesicle precursors, arethought to be responsible for the devel-opment of neuropathy in CMT2Apatients. Interestingly, mutations inanother kinesin motor have beendescribed in a family with hereditaryspastic paraplegia.

In summary, different upstream alter-ations, such as mutations in motor-pro-teins or structural neuronal proteins,could lead to altered axonal transport,which could explain the similaritiesobserved regarding the pathogenesis ofthe different types of CMT. Moreover, dis-ruption of axonal transport is a good can-didate as a common pathogenic mecha-nism in a number of neurological andneurodegenerative diseases.

R.P-O. was supported by a post-doctoralfellowship from the Charcot-Marie-ToothAssociation (CMTA,2002),and is theCarolyn Redell CMTA Fellow (2003).Research in Dr. Liem’s laboratory is sup-ported by the National Institutes ofHealth.

References 1. Brownlees J., Ackerley S., Grierson A.J.,

Jacobsen N.J., Shea K., Anderton B.H.,

Leigh P.N., Shaw C.E., and Miller C.C..(2002). Charcot-Marie-Tooth disease neu-rofilament mutations disrupt neurofilamentassembly and axonal transport. Hum MolGenet. 11, 2837-44.

2. De Jonghe, P., Mersivanova, I., Nelis, E., delFavero, J., Martin, J. J., van Broeckhoven,C., Evgrafov, O. and Timmerman, V.(2001). Further evidence that neurofilamentlight chain gene mutations can causeCharcot-Marie-Tooth disease type 2E. Ann.Neurol. 49, 245-249.

3. Georgiou, D.M., Zidar, J., Korosec, M.,Moddleton, L.T., Kyriakides, T., andChristodoulou, K. (2002). A novel NF-Lmutation Pro22Ser is associated withCMT2 in a large Slovenian family.Neurogenetics 4, 93-6.

4. Jordanova, A., De Jonghe, P., Boerkoel,C.F., Takashima, H., De Vriendt, E.,Ceuterick, C., Martin, J.-J., Butler, I.J.,Mancias, P., Papasozomenos, S.Ch.,Terespolsky, D., Potocki, L., Brown, C.W.,Shy, M., Rita, D.A., Tournev,I., Kremensky,I., Lupski, J.R., and Timmerman, V. (2003).Mutations in the neurofilament light chaingene (NEFL) cause early onset severeCharcot-Marie-Tooth disease. Brain 126,590-7.

5. Mersiyanova, I. V., Perepelov, A. V.,Polyakov, A. V., Sitnikov, V. F., Dadali, E. L.,Oparin, R. B., Petrin, A. N. and Evgrafov, O.V. (2000). A new variant of Charcot-Marie-Tooth disease type 2 is probably the resultof a mutation in the neurofilament-lightgene. Am. J. Hum. Genet. 67, 37-46.

6. Perez-Olle, R, Leung, C.L., and Liem, R.K.(2002). Effects of Charcot-Marie-Tooth-Linked mutations of the neurofilament lightsubunit on intermediate filament formation.J Cell Sci. 115, 4937-46.

Yoshihara, T., Yamamoto, M., Hattori,N., Misu, K., Mori, K., Koike, H.,.

Raul Perez-Olle and Ronald K.H. Liem.Department of Pathology, Columbia

University College of Physicians &Surgeons. New York, USA

NOTICEDear WFN member,

You are invited to participate in a web survey on the perceived quality ofjournals in clinical neurology. With the support of the World Federation ofNeurology, we would like to target WFN members as our survey sample.We believe that your valuable opinion will represent the voice of neurolo-gists worldwide. Your support will assist in the overall development of bet-ter programs to evaluate research performance in medicine and especiallyin clinical neurology.

We will be most appreciative if you could take a few moments to followthis link http://www.sistm.unsw.edu.au/people/wpyue/ and complete theshort survey.

If you wish, the result of the survey will be sent to you later via email uponcompletion of the study. Please note that all information provided will bekept strictly confidential.

Bibliometric and Informetric Research Group (BIRG,htttp://birg.web.unsw.edu.au/)

University of New South Wales, Sydney 220522, Australia

New Regional Vice-President

Dr. Pedro Chana (Chile), New RegionalVice President, Pan American Region, toserve two years. This position was heldfor the previous two years by Dr. CarlosChouza of Uruguay.

7th Congress of theEuropean Federation ofNeurological Societies

The 7th Congress of the EuropeanFederation of Neurological Societies washeld in Helsinki from August 31 toSeptember 2, 2003 under the patron-age of Mrs. Tarja Halonen, President ofthe Republic of Finland, and the hostwas the Finnish NeurologicalAssociation. It was co-sponsored also bythe World Federation of Neurology, rep-resented by the First Vice President, Prof.Johan Aarli, and by the Vice President forEurope, Prof. Leontino Battistin. TheCongress was chaired by the Presidentof the EFNS, Prof. W.D. Heiss, and thelocal committee was chaired by Prof. J.Palo. On the first day there were 11Teaching Courses on various aspects ofthe neurological diseases, attended bymany young people, coming from allover Europe. The 3 days of the Congresswere divided into Main Topics in themorning and Focused Workshops in theafternoon. On the first day the three MainTopics were: Primary vs. SecondaryPrevention of Stroke; Mechanisms ofChronic Axonal Injury in MultipleSclerosis; and Drug Development andSafety. On the second day the threemain topics were: New Frontiers inMovement Disorders; InterventionalTreatment of Refractory Epilepsy; andGenetics applied to NeurologicalDiseases. On the third day the threemain topics were: Treatment ofDementia; Emerging Treatment in non-glial tumours; and Autonomic and sexualdysfunction.

In the afternoon there were fourteenFocused Workshops, with the followingtitles: Treatment of metabolic disorders,Assessment of Parkinson’s disease inclinical practice, New imaging tech-niques to monitor brain tumour therapy,Practical approach to evaluation ofpatients with dementia, Molecular mech-anisms of demyelination in hereditaryneuropathies, Acute stroke treatment—Elements of care, Viral infections of theNervous System, Benign MultipleSclerosis, Clinical diagnosis of mito-chondrial diseases, Neuro-NuclearMedicine—New aspects in imaging,New neurological channelopathies,Systemic reviews in evidence-basedneurology, White matter diseases:assessing the significance with neuroim-

maging, and Controversies in the treat-ment of stroke.

There were also some Special sessions;on Sunday, one was dedicated toNeuroethics, another one was a meetingof the European Association of YoungNeurologists and Trainees. On the sec-ond day there was a special session onMusic and Neurology, organized by aSpecial Interested Group, another wasorganized by the European StrokeInitiative and finally one was organizedby the European Society for ClinicalNeuropharmacology devoted to CNSdrug action and gene expression. Therewere also other special sessions, onededicated to the basal ganglia group,another one dedicated to the History ofNeurology (Clifford Rose Lecture) andfinally one dedicated to the Hyperbaricoxygenation program.

During the lunch time and also in theevening there were various SatelliteSymposia dedicated to various aspectsof therapy in CNS diseases and support-ed by educational grants from pharma-ceutical companies.

The Congress was attended by almost3500 neurologists coming from all overEurope but also from elsewhere and allthe sessions were very well attended.There was also CME accreditation. Ofparticular relevance was the OpeningCeremony, held on Sunday August 31, inthe late afternoon and opened byProfessor W.D. Heiss with a memory ofDr. Friederike Tschabitscher, the execu-tive director of the EFNS, who had diedjust one month before the Congress. Allthe audience stood for a minute’ssilence in memory of Mrs. Tschabitscher,known as Uschi. Prof. Dieter Heiss out-lined the great relevance of the workdone by Dr. Tschabitscher, for EFNS andproposed the institution in her memory ofbursaries for young neurologists in thefuture. The ceremony was closed by amusical program, performed by theHelsinki Streams and also by a lecture byProf. Jorma Palo of Helsinki, who gave avery nice satiric view of Finland in thepast and in the present.

The Congress was very successfulin all scientific sessions that wereattended by many people, with out-standing presentations, and also in thesocial part, also quite nice, and verywell organized by the local committee.The closing ceremony was excellentwith an appointment in September

2004 in Paris for the 8th Congress ofthe European Federation ofNeurological Societies.

Prof. Leontino BattistinWFN Vice President for Europe

National StrokeSymposiumNational Stroke Symposium was organ-ized on February 2-3, 2002 by Nizam’sInstitute of Medical Sciences,Hyderabad, India. This was the firstregional stroke meeting organized inIndia for the year 2002 and wasendorsed by the International StrokeSociety. Eminent faculty from India andabroad (Singapore, USA, Italy), partici-pated. The International Stroke Societywas represented by Prof G. Lenzi andProf PM Dalal. The response to the meet-ing was overwhelming, with about 300delegates actively participating from allparts of India, most of whom expressedtheir interest in becoming members ofthe International Stroke Society. Topicsdiscussed included, epidemiology,imaging, thrombolytic therapy, strokeunits, strategies for primary and second-ary prevention and role of surgical inter-vention in stroke. There was an opensession, in which stroke issues specifi-cally relevant in the Indian context werediscussed. The need for having anIndian stroke association was felt. Theongoing ICASS (Indian Collaborativeacute stroke study) project, its strengthsand limitations were discussed at length.All in all, it was a highly successful meet-ing which highlighted the interest instroke among the neurologists of India.The scientific proceedings of this meet-ing appeared in the form of a specialstroke supplement of the December2002, vol. 50 issue of Neurology India, official journal of NeurologicalSociety of India, under the editorship of Dr Subhash Kaul, convener ofNational Stroke Symposium. The entirecontents can be viewed at http: www.neurologyindia.com.

REGIONAL NEWS12



REGIONAL NEWS

BOOK REVIEWS 13



The Indian Stroke Association wasformed in May 2002 and is still in itsinfancy with a membership of 40 neurol-ogists. There are over two-dozen strokeunits in the country with excellent facili-ties for care of acute stroke patients.

Dr. G. Arjundas (Chennai)President, Indian Stroke Association

NINDS at 50-50 years ofBrain Research

Editor: Lewis P. Roland, M.DISBN : 1 888799 71 4 No. of Pages: 360Price: $44.95Publication Date: 2003Publishers : Demos Medical

Publishing —N.Y

An excellent monograph on the 50 yearsof history of National Institute ofNeurological Disorders and Stroke. TheEditor has explicitly described its birthand development, to become one of themost powerful Institutes in modern neu-rosciences with, to its credit, six Nobellaureates and five Lasker Award winners.The book is well written with numeroushistorical photographs to enrich its con-tents. There are short biographies offamous neuroscientists who wereDirectors of the Institute, includingRichard L. Masland who was President ofthe World Federation of Neurology for 8years. This volume should be a part ofevery Neuroinstitute’s Library to inspireyounger neuroscientists, not only tomatch but surpass the achievements ofthose who built this Institute.

Editor-in-Chief

Managing theSymptoms of MultipleSclerosisEditor: Randall T. Schapiro M.D ISBN : 1 888799 78 1 (Pbk)

No. of Pages: 198Price: $19.95Publication Date: 2003Publishers : Demos

A very helpful book for those who treatmultiple sclerosis patients. There is indepth discussion of the disease which iswidespread throughout the world. Theauthor has meticulously planned the fourmajor parts in this book which are mostappropriate and which give the total viewof this ailment starting from Disease andits management, managing the variousMS symptoms including movement andwalking, care of bladder and bowel,speech and swallowing, sensory distur-bances, and diet and nutrition in additionto medical therapy. The author is thedirector of a multiple sclerosis centre andhis own observations, experience andclinical handling of such patients add firsthand knowledge of this disease. Thebook is recommended for neurologists,general physicians, physiotherapists andpostgraduate students.

Prof. S. PrabhakarPostgraduate Institute of Medical Education &Research, Chandigarh - India

Fragments ofNeurological History Editor: John M.S. Pearce ISBN: 1 8609 43381 P274 H No. of Pages: 633Price: £46.00Publication Date: April 2003Publishers: Imperial College Press

This book highlights the neurological his-tory of almost all diseases of the nervous

system: the first anatomical and neuro-physiological phenomena recorded; thefirst scientific human cadaveric dissec-tion by Herophilis and Erasistratus in325-255 B.C.; and the revolutionaryadvances made by Vesalius (1514-1564). The ‘coils’ of the brain were firstnoted by Praxagoras (300 BC) andErasistratus 260 BC who compared themwith the coils of the intestine. Vesaliusrecognised focal epilepsy—precedingHughlings Jackson—and described it asfollows: ‘arisen from an obstruction of thebrain extending to those processes atthe origin of nerves, a certain aura orvapour is always felt to be carried fromthe leg through the hip, then scapula,upward to head and then left leg’. Thebook gives a vivid description of thelocalization of functions, early neu-roanatomy and neurophysiology. Thereare early descriptions of convulsions ofthe brain, fissure of Rolando and Sylvius,recognition of Erb, Westphal and tendonreflexes, Clark’s column, Schwann cells,Wallerian degeneration and myelin,aphasia or aphenia, Paul Broca’sdescription of aphasia etc. There are toohistorical descriptions of dementias,headache, epilepsy, CSF, stroke andvascular disease, ocular and cranial dis-orders. This volume also gives shortbiographies of those famous neuroscien-tists who recorded early symptoms andsigns of neurological diseases in ancienttimes. It is a very interesting publicationwith lots of illustrations, photographs andquotations from early works. Students ofneurology and neurophysicians will bethrilled to read it.

Editor-in-Chief

BOOK REVIEWS

WFN TIEOne of the many success stories of theLondon World Congress was thelaunch of the new WFN silk tie, a rever-sal (blue ‘Circle of Willis’ on a redbackground) of the traditional tie wornby WFN members for a number ofyears now. For those who missed theopportunity to acquire this souvenir of

this memorable Congress when in London or who could not attend WCN2001, ties are available from the London Secretariat Office at the same priceas the blue version £20.00 (U.S. $30.00); Ladies Scarves (blue only, 31x31inches) are also available for £30.00 (US$50). Orders with payment to: WFNSecretariat, 12 Chandos Street, London W1G 9DR, UK.

ADVERTISEMENT14



Special Product Highlight

The Brain’s Alpha Rhythms

A review of classical andmodern studies of the alpharhythm component of theelectoencephalogram withcommentaries onassociated neuroscienceand neuropsychology

Edited by: J.C. Shaw

To the author’s knowledge thisbook is the first to review the wholefield of the Alpha rhythmcomponent of the electroence-phalogram (EEG). It reviews theclassical studies from the 1930sthrough the 1980s when EEG research became dominated by event-related potential studies. Renewed interest in the alpha rhythmdeveloped in the 1990s when neuronal oscillations became a majorfocus of interest in the neurophysiology of brain function. Many of thelater studies of alpha activity that resulted from this development are fullydocumented in the book.

ISBN 0-444-51397-3, 380 pages

USD 135/Euro 135, hardbound

Pub date: June 2003

For a complete list of contents please visit the book’s homepage onhttp://www.elsevier.com/locate/isbn/0444513973/

Come and meet usElsevier Science attends manyscientific meetings each year.Below please find a list of meet-ings at which we will have one ormore booths in 2003. Pleasecome and visit us to browse thelatest releases in our book pro-gram, see online demonstrationsof our electronic products andservices, and to get free samplecopies of our journals.

Society for Neuroscience (SFN)8-12 November 2003, New Orleans, USA

American Epilepsy Society5-10 December 2003, Boston, USA

We look forward to seeing you!

Neurology in Clinical Practice4th Edition

Walter G Bradley MD, FRCP,Robert B Daroff MD, Gerald MFenichel MD and Joseph JankovicMD

With over 150 international contributors.

‘The principal neurology text whichdefines the state of neurological diag-nosis and neurological disorders wellinto the 21st century‘—Neurology*

NEUROLOGY IN CLINICAL PRACTICE is widely acknowledged as theworld’s definitive encyclopaedic neurology reference.

The new Fourth Edition delivers state-of-the-art explanations of generalneurologic principles and relevant neuroscience, as well as authoritativeapproaches to the diagnosis and management of the full spectrum ofneurologic disorders.

Master the very latest advances in...

! movement disorders

! mitochondriopathies

! channelopathies

! neurologic complications of paediatric HIV and AIDS

! neuromuscular disorders

! neuro-oncology

This multimedia exponentially boosts your referencepower!

Neurology in Clinical Practice now comes with a free website, includedin the fantastic introductory price of £270.00! As well as the full text,online and fully searchable, you also get:

! ongoing content updates

! video clips and additional full-colour photos

! links to related resources

! relevant material from other neurology texts

! and much more!

Easily find out what’s what... with help from a “who’s who” in yourfield!

NEUROLOGY IN CLINICAL PRACTICE 4th edition features contri-butions from over 150 eminent neurologists—40% of them new to thisedition. They present you with an optimal blend of traditional clinicalpractices and innovative new perspectives.

Nowhere else will you find so much detailed, clinically focused coverageof your specialty...in such a versatile, multimedia format. Order yourcopy today!

0750674695 " November 2003 " Hardback " 2,512 pages / 650 illus "Elsevier Butterworth Heinemann " £270.00

For further details, sample chapters and to order, visit www.into.else-vierhealth.com/neurology, or order by phone: +44 (0) 1865 474010 orfax: +44 (0) 1865 474011.

ADVERTISEMENT 15



Journal of theNeurologicalSciences

Science DirectBackfiles

Now that the completecollection of articles everpublished in the Journal ofthe Neurological Scienceshas become available, youcan read and download full-text articles published as early as 1964 if your institute has aScienceDirect subscription.

Here you can find the first abstract of the first article published inJanuary-February 1964 (p 21- 2).

`Lightning eye movements’ (ocular myoclonus) by A. Atkin andMorris B. Bender, Department of Neurology, Mount SinaiHospital, New York, N. Y., U.S.A.

Abstract

1. (1) Three patients showing rapid bursts of horizontal to-and-fro eye movements were studied.

2. (2) The burst pattern consisted of small conjugate saccades.

3. (3) Bursts most often occurred following horizontal gazemovements toward the more paretic or ataxic side of thepatient’s body, and the first movement of each burst was mostoften toward the less paretic side.

4. (4) All three of the patients showed some signs of dysfunctionin the pontine paramedian zone; 2 of the 3 patients also showedevidence of pretectal lesions, and in both these patients theburst eye movements appeared to be closely associated witheye movements characteristic of pretectal dysfunction.

5. (5) Several other peculiarities of ocular movements, whichmay in some way be related to the `lightning eye movements’,were noted in both patients with predominant pretectal involve-ment.

Please visit http://www.info.sciencedirect.com/backfiles/formore details on the ScienceDirect Backfiles.

THE LANCET Neurology ! Clinical research ! Reviewed ! In Context

The September issue of thisexciting review journal fromTHE LANCET PublishingGroup is now available.

Reviews covered in thisissue are:

! Compulsive use of dopamine replacement therapy in Parkinson’s disease: reward systems goneawry?

Andrew D Lawrence, Andrew H Evans, Andrew J Lees

! CSF markers for incipient Alzheimer’s disease

Kaj Blennow, Harald Hampel

! Triple-H therapy in the management of aneurysmal subarachnoid haemorrhage

Jon Sen, Antonio Belli, Helen Albon, Laleh Morgan, Axel Petzold, Neil Kitchen

! Synaptic plasticity in the ischaemic brain

Paolo Calabresi, Diego Centonze, Antonio Pisani, LetiziaM Cupini, Giorgio Bernardi

Forum

! Physician-assisted death: dying with dignity?

Peter Reagan, Rachel Hurst, Lisa Cook, Zbigniew Zylicz, Margaret Otlowski, Jan H Veldink, Leonard H van den Berg, John HJ Wokke

THE LANCET Neurology E-Toc alerts now available! Sign-uptoday at http://neurology.thelancet.com/profile/etoc

For more details on how to subscribe, please visit http://neurol-ogy.thelancet.com today or contact THE LANCET Neurology, 32Jamestown Road, London, NW1 7BY, UK, Tel +44 (0) 20 76114102, or email [email protected].

CALENDAR16



2003

7th Congress of the WorldFederation of Interventional andTherapeutic Neuroradiology 2-5 November, 2003

Recife, BrazilContact: Congress Secretariat, RuaGaleno de Almeida, 319-319-05410-030 - Sao Paulo-SP— BrazilTel: 55 11 3891-0295, Fax: 55 113891-1780, Website:www.wfitn2003.org.br

4th Neurobiology of AgeingConference 6-7 November, 2003

New Orleans, LA, USAContact: Conference Secretariat, AprilWilliams, Elsevier, The Boulevard,Langford Lane, Kidlington, OxfordOX5 1GB, UKTel: +44 (0) 1865 843089, Fax: +44 (0) 1865 843958, e-mail: [email protected],Website: www.nba-interactivemeet-ing.com

33rd Annual Meeting of the Societyfor Neuroscience 8-12 November, 2003

New Orleans, LA, USAContact: Society for Neuroscience, 11Dupont Circle, NW, Suite 500,Washington DC 20036, USATel: (202) 462-6688, Fax: (202) 462-9740, e-mail: [email protected]: web.sfn.org/ACSplash.cfm

4th Asian Sleep Research SocietyCongress20-23 November, 2003

Zhuhai, ChinaContact: Congress Secretariat,Shanghai Inst. for Biol. Sci., ChineseAcademy of Sci. Bd. 31-111, YueYangRoad 319 Shanghai, 20031, China Tel: 86-21-64336650/64335358, Fax:86-21-64375762 e-mail: [email protected],Website: www.shisa-sleep.com/

11th Asian-Australasian Congress ofNeurological Surgery22-26 November, 2003

SingaporeContact: 11th AACNS Secretariat, 302Orchard Road, #16-04 Tong Building,Singapore 238862Tel: (65) 6738 1871, Fax: (65) 67387691, e-mail: [email protected]: www.aasns.com/aacns

An International Conference onNeuropsychology: Recent Advancesin the East and West 9-12 December, 2003

Hong Kong, ChinaContact: Rm 309, Sino Building,Chung Chi College, the ChineseUniversity of Hong Kong, Shatin - Tel:852-2609-6648 , Fax: 852-2603-5019, e-mail:[email protected],Website:www.psy.cuhk.edu.hk/~aschan/lab.htm

European Charcot FoundationSymposium 200311-13 December 2003

Lisbon, PortugalContact: Ms. M. Friedrichs-Bosmans,European Charcot Foundation,Heiweg 97, 6533 PA Nijmegen,NetherlandsTel: +31-24-3561954, Fax: +31-24-3540920e-mail: [email protected],Website: www.charcot-ms.org

2004

3rd Asian & Oceanian MyologyCentre Annual Scientific Meeting 8-9 January, 2004

SingaporeContact: The NNI Meeting Secretariat,National Neuroscience Institute, 11Jalan Tan Tock Seng, Singapore308433, Republic of SingaporeTel: +65-6357-7151/7608, Fax: +65-6256-4755e-mail: [email protected],Website: www.nni.com.sg

II Conferencia CientÌfica de CIREN 24-27 February, 2004

Habana, CubaContact: Centro Internacional deRestauraciÛn NeurolÛgica (CIREN) Evento RestauraciÛn NeurolÛgica2004 Ave. 25 # 15805, 11300 Playa Ciudad de La Habana, Cuba,Website: www.ciren.ws/rt2004

4th Conference of NeurologySantiago De Cuba, Cuba2-4 March, 2004

Contact: Dr. Francisco Ruiz Miyares,President of the OrganizingCommitteeCalle 8 Nro.260 entre 7 y 9 VistaAlegre. Santiago de Cuba 90400,

CubaTel: 53-226 644288 / 53 226 642760E-mail: [email protected]

IV International Symposium onComa and Death9-12 March, 2004

Habana, CubaContact: Dr. Calixto Machado, MD,Ph.D., President, Instituto deNeurologÌa y NeurocirugÌa, 29 y D,Vedado, Apartado Postal 4268,Ciudad de La Habana 10400Tel.: 537-553022 Ext 218 , Fax: 537-228382 e-mail: [email protected]: Ms. Migdalia LunaCisneros,Organizadora del Simposio,Palacio de Convenciones de LaHabanaTel: (537) 22-8382/21-9496, e-mail:[email protected]: www.sld.cu/eventos/comay-muerte/index.htm

2nd Mediterranean Congress ofNeurology2-4 April 2004,

Nicosia - Cyprus Website: www.medneuro2004.com

5th International Prader-WilliSyndrome Scientific Workshop andConference9-12 April, 2004

Christchurch, New ZealandContact: PWS Conference Secretariat,The Conference Office, Centre forContinuing Education, University ofCanterbury, Private Bag 4800,Christchurch, New Zealand Tel: +64.3.364.2915, Fax:+64.3.364.2507 e-mail:[email protected]: www.pwsa-nz.co.nz/web-site_2004conf

VI International Congress onHeadache in Children andAdolescents 21-25 April, 2004

Vienna, AustriaContact: INTERPLAN, Congress,Meeting & Event Management AGAlbert-Rosshaupter-Strasse 65, D-81369 M¸nchen, GermanyTel: +49 / 89 / 54 82 34 15 , Fax: +49/ 89 / 54 82 34 43e-mail:[email protected], Website:www.i-plan.de/ichca

CALENDAR

world neurology · here are a few brief notes on the ... the newsletter of the world federation of...

Documents