President's Column

THE NEWSLETTER OF THE WORLD FEDERATION OF NEUROLOGY

VOLUME 16, NUMBER 2, JUNE 2001 PRESIDENT’S COLUMN

It is my hope that this column reaches the WFN membership before the London Congress. I have watched and participated in the extraordinary preparations for this major event, implemented by the Or- ganizing Committee of the Association of British Neurologists. As was said by one of Britain’s greatest - “Never have so many owed so much to so few.” All of us are deeply indebted to our English colleagues who have gone to such lengths to ensure its success, despite misfortunes, such as the global economic downturn and bovine epizootic encephalitis.

There cannot be a more appropriate time to remind the public that two Nobel prizes

Earls Court, the venue of the XVII World Congress of Neurology in London.

have been awarded to neurologists for the wanton killing of tigers for medicinal delineation of mode of transmission and purposes, of rhinoceri and elephants for identification of the agent of transmission ivory and for aphrodisiacs, or people but we do not have a treatment, other whose value to society is judged to be than preventing spread. We must apply marginal. our skills to developing an effective proph- ylaxis and therapy. Even though we neurologists are few in number, we are highly visible because we Furthermore, neurologists have a respon- are the explorers of the brain, the most im- sibility for devoting ourselves not only portant organ in the body. The brain keeps to human nervous system disorders but society moving and serves to direct it. We those of all animals, because BSE makes (cont. on page 3) us acutely aware of our global interde- pendence not only with each other but ALSO IN THIS ISSUE: with all flora and fauna. •Editorial There is great concern regarding human •WFN Annual Reports and other animal rights and, in the past, •Acute Stroke Units in Austria National Institute of Neurology and Neurosur- •Vascular Dementia gery, Mexico City. Left to right: Ignacio Ruiz, we have been callous regarding stand- Head of Postgraduate Teaching; Julio Sotelo, ards for clinical research on lower orders. •Mitochondrial Encephalo- General Director; Teresa Corona, Teaching Di- Neuroscientists must interact with society myopathies rector; James Toole, President, World Federa- to agree upon and enforce ethical stand- •Book Reviews tion of Neurology; Ricardo Colin, Head of Pre- ards for use of all species. It is no longer •Meetings Calendar graduate Teaching. tolerable for us to stand by idly and permit

Visit the WFN website at http://www.wfneurology.org 2 World Neurology

EDITOR-IN-CHIEF Dr. Jagjit S. Chopra, # 532 Sector 33-B, Chan- WORLD digarh - 160 047, India. Fax: +91-172-665532 E-mail: [email protected] [email protected] NEUROLOGY EDITORIAL ADVISORY BOARD Dr. Richard Godwin-Austen, World Federation The Newsletter of the World Federation of Neurology of Neurology, 12 Chandos Street, London W1G VOLUME 16, NUMBER 2, JUNE 2001 9DR, UK Dr. Robert B. Daroff, Case Western Reserve University/University Hospitals of Cleveland, Department of Neurology Cleveland, 11100 Eu- CONTENTS clid Avenue, OH 44106-5015, USA President’s Column ...... 1 Dr. Jun Kimura, Park City Uji Byodoin 1203, 169 Uji Myoraku, Uji Shi, Kyoto 611-0021, Japan Editorial ...... 3 Dr. Theodore Munsat, Department of Neurol- ogy, New England Medical Center, Box 314, WFN Committee Meetings at the AAN ...... 5 750 Washington Street, Boston, MA 02111, USA WFN Task Force on Neurological Services ...... 5 Dr. James F. Toole, Wake Forest University, Baptist Medical Center, Medical Center Boule- Annual Reports of WFN Research Groups Year 2000 . . . . . 5 vard, Winston-Salem, NC 27157, USA Research Group on Neuroepidemiology ...... 5 REGIONAL VICE PRESIDENTS Research Group on the History of Neurosciences ...... 6 (by virtue of Presidency of Regional Congress) Autonomic Disorders Research Group ...... 6 Dr. S.M. Al Deeb, Riyadh Armed Forces Hospi- tal, Department of Clinical Neuroscience, P.O. CSF Research Group ...... 6 Box 7897, Riyadh 11159, Saudi Arabia Neurotoxicology Research Group ...... 6 Dr. Amado M San Luis, Rm. 1006, St. Luke’s Medical Center, E. Rodriguez Ave., Quezon Acute Stroke Units in Austria ...... 7 City, Philippines Dr. Ndiaye, Centre Hospitalier Universitaire De Pan-Arab Union of Neurological Sciences ...... 7 Fann, Clinique Neurologique, BP 5035, Dakar, Senegal WFN Junior Travelling Fellowship Reports ...... 8 Dr. Jes Olesen, Professor and Chairman, De- partment of Neurology, Glostrup Hospital, Nor- First LACTRIMS Congress ...... 8 dre Ringvej, DK-2600 Glostrup, Denmark Dr. Gustavo Pradilla, Carrera 29 # 32-65, Edi- Vascular Dementia: it is time for a new approach ...... 8 ficio San Diego Of. 201, Apartado Aereo 678, Bucaramanga, Colombia Mitochondrial Encephalomyopathies ...... 11 ASSISTANT EDITOR Book Reviews ...... 13 Dr. I.M.S. Sawhney, Consultant Neurologist, Morriston Hospital, Swansea SA6 6NL, UK Meetings Calendar ...... 16 WFN ADMINISTRATOR Keith Newton, World Federation of Neurology, COPYRIGHT © 2001 World Federation of Neurology. All rights reserved 12 Chandos Street, London W1G 9DR, UK Published by Elsevier Science BV, Amsterdam, the Netherlands. Manuscripts accepted for publication become the copyright of the World Federation of PUBLISHING STAFF Neurology (WFN). Before publication a copyright form will be supplied by the Publisher, Publisher: which must be completed by all authors. Peter F. Bakker Marketing Manager: Joyce Hobbelink be submitted in English, typed on white paper World Neurology, ISSN 0899-9465, is published Design and Layout: using double spacing with margins of at least 3 by Elsevier Science BV, Molenwerf 1, 1014 AG Helmut Hummelbrunner cm. Authors should submit material on compu- Amsterdam, the Netherlands; phone: +31 (20) Operational Support: ter disk (Microsoft® Word or plain ASCII for- 485 3358, fax: +31 (20) 485 3237; e-mail: Maureen Twaig, Annemieke van Es mat) whenever possible. 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cult to recover from and this is obviously a One of the main themes at the World Con- EDITORIAL matter of great concern. Nevertheless the gress of Neurology 2001 is ‘Stroke’. This administrative team of Keith Newton and issue carries an article on “Vascular De- Large numbers of neurologists and other Susan Bilger, with their ability, zeal and en- mentia” in which Prof. Vladmir Hachinski neuroscientists are looking forward to a thusiasm, will again try to rebuild the data- puts his views that a time has come for scientific bonanza at the next World Con- base as quickly as they can. With the sup- a new approach to this ever increasing gress during the British summer in London port of national neurological societies, in- cause of dementia in the elderly and that this month. There have been great sci- dividuals and others they hope to return there is a need for it to be clearly delineat- entific advances in neurology since the to normal operations in as short a time as ed from dementia due to Alzheimer’s dis- last World Congress in Buenos Aires in possible. ease, although both may co-exist in some 1997. The mapping of the human genome patients. He has suggested that we need Annual reports for year 2000 of some of and the possibilities of gene therapies in- to overturn our current approach of gath- the WFN Research Groups are printed in crease the chances for effective treatment ering data into arbitrary, dogmatic, obso- this issue. They reflect the amount and for some of the genetically linked neuro- lete categories and begin developing a magnitude of the work that is being car- logical disorders. Let us await with interest new pragmatic, systematic, data based ried out by some of the more vibrant the important research in this area which approach. Congratulations to him, inci- groups and the plans that others are for- will be revealed at the XVII World Congress dentally, for having been appointed Ed- mulating. of Neurology in London. Around 3,000 itor-in-Chief of the prestigious journal persons are already registered with more The report of the Pan-Arab Union of Neu- ‘Stroke’. pouring in everyday; and, if one goes by rological Sciences is also featured here. It Prof. Salvatore DiMauro has with great previous Congress estimates, 20–25% of is heartening to learn that this group is fast brevity discussed the intricate problems attendees register ‘on the spot’. There- emerging and accelerating the progress and concepts of “Mitochondrial Enceph- fore, the London Congress is expected to of neurology in this part of the world. be a great success. alopathies” which at one time were con- Elsewhere on the globe, the Latin Amer- sidered as myopathic ailments in chil- Dr. James F. Toole in his President’s ican Committee for Treatment and Re- dren with mental dysfunction and renal Column has reminded all neurologists of search in Multiple Sclerosis (LACTRIMS) derangement. Prof. DiMauro deserves our their responsibilities towards humans and also deserve our applause for their 1st thanks for this explicit write up of a difficult equal care for animals. He has rightly Congress and our encouragement for the problem. called neurologists the ‘explorers of the planned 2nd Congress in 2002. It is a brain’, the most important organ in the newly formed group but the quality of their body and he is also right in reminding performance is self-evident. neuroscientists of the need both to guard Many nations need to emulate the exam- human values and protect the rights of an- ple of Austria, a nation of 8 million people. imals to exist in their natural environments. The report “Acute Stroke Units in Austria” He has touched upon some other perti- by Prof. Michael Brainin, President of the nent questions for neurologists to ponder, Austrian Society for Stroke Research, is il- concerning questions of neuroethics and luminating and reveals the masterly plan- the updating of medical ethics etc. ning that has been put into the aim of Burglars struck at the World Federation of doubling the present twenty-five function- Neurology office in the heart of London al stroke units in that country by 2005. This Jagjit S. Chopra, MD during the night of 18/19 April. The loss of will be a quite fantastic feat and they de- Editor-in-Chief, World Neurolog equipment and precious data will be diffi- serve our congratulations for their efforts.

(President’s Column – cont. from p. 1) intellectual pacesetter because neurolo- and vascular dementia, both of which will gists can relate to philosophy, ethics, and assume leading positions, depending on cannot afford to have impaired individu- religion in unique ways. the nation, as causes of disability and als in leadership positions - pilots flying death by the year 2025. We in neurology airplanes or leaders of nations making Within the past few months, I attended must make a global plan for the field of faulty decisions and, yes, impaired doc- the American Stroke Association meeting, stroke and, to that end, the International tors making decisions regarding their pa- which has grown from perhaps 50 dedi- Stroke Society has chosen to have a Stra- tients. For these reasons, I am a strong cated individuals, of which I was one, to tegic Planning Retreat in London, imme- advocate for the World Federation of Neu- nearly two thousand attendees. Although diately preceding the World Congress of rology assuming global leadership in the this multi-disciplinary group involves pri- Neurology, so that plans for a parallel rela- field of neuroethics and inviting members marily neurologists, I am struck with the tionship with the World Federation of Neu- of other disciplines to participate with us, reality that the World Federation of Neu- rology can become a reality. through the World Health Organization, in rology has no group dealing with stroke. updating the codes of medical ethics, in- Our research group, founded by Helmut In February, I had the good fortune to be cluding the right to self determination re- Lechner and John S. Meyer, has been dis- invited to address the National Institute garding the end of life and the protection solved. It is my strong belief that neurolo- of Neurology and Neurosurgery in Mexico of impaired individuals from the preda- gists must be the primary care givers for City, on the occasion of its conclusion tors who exist in every society. The WFN stroke victims but that we have abdicat- of the academic year. Director General should embrace its global mandate be- ed our responsibility. We must resurrect Julio Sotelo presided at a most remark- cause it now has an unparalleled commu- our presence and restructure ourselves able convocation (see photograph on nications network and could become the into the premier group dealing with stroke (cont. on page 4)

WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org 4 PRESIDENT’S COLUMN

(President’s Column – cont. from p. 3) frontpage). Founder Emeritus Professor Velasco Suarez proudly informed me that he was the first neurosurgeon in Mexico. His efforts, and those of Dr. Francisco Ru- bio-Donnadieu, are now being taken up by Director General Julio Sotelo and his associates, Alfredo Gomez Avina, Misael Uribe, in collaboration with child neurologist Jose Eduardo San Esteban, the Deputy Minister of Health for Mexico. All of these individuals are extremely enthusi- astic about the opportunities for equitable distribution of health care in Mexico. They have a very inclusive outlook for clinical neuroscience, which includes embracing their colleagues in psychiatry and neurosurgery, as well as other allied disciplines. One has the impression of a very vibrant organization, full of enthusiasm, under the direction of Dr. Teresa Corona, Teaching Director. Dr. M. Zuheir Al-Kawi, Dr. Ashraf Kurdi, Dr. A. Kareem, Dr. Basim Yaqub In April, my wife and I attended the Third Jordanian Neurosciences Society Confer- Cooperation Neurology Workshop, with beacon shining to the East, encouraging ence, in collaboration with the Pan Arab the purpose of bringing individuals from young individuals from countries that do Union of Neurological Societies, with en- countries that do not ordinarily send not have the opportunities that the devel- dorsement by the World Federation of delegates to World Congresses. It was oped countries have to learn the newest Neurology, in Amman, Jordan (see photo- most impressive to be with the young advances in the field of neurology and graph above). Professor Ashraf Kurdi and neurologists from Albania, Belarus, Bul- to become real participants in the neuro- Professor Abdel Karim Al-Qudah were the garia, Croatia, Czech Republic, Estonia, sciences. These meetings are underwrit- main organizers. Working with them in a Georgia, Greece, Hungary, Moldova, Italy, ten by the EFNS and serve as a model very close relationship was the Organizing Poland, Romania, Russia, Slovak Repub- for other regional groups to emulate with Committee, Scientific Program Commit- lic, Slovenia, Ukraine, and Yugoslavia. the financial and intellectual support of tee, Social Committee, and the Finance This, in my opinion, is one of the stellar ac- the World Federation of Neurology. The Committee. All did enormous work in pre- complishments of the ten year old EFNS, EFNS is truly a regional model within neu- paring a very exciting and successful pro- the guiding lights of which have been Pro- romedical organizations and the WFN is gram. fessor Franz Gerstenbrand and his wife, proud to have encouraged this new devel- Friederike Tschabitscher, and now under opment during the administration of Lord Thence, my wife and I went to Trest, Czech the able leadership of Dr. Jes Olesen (see John Walton, Frank Clifford Rose, and Republic, for the second EFNS European photograph below). They have been a Klaus Poeck. Another example of collab- orative leadership also includes the com- bined efforts of the Association of British Neurologists and the EFNS with the World Federation of Neurology during this administration to effect a global meeting at the World Congress in London in June, which I hope the vast majority of neurologists in the world will attend, so that I can personally greet as many as possible. Hopefully, the EFNS will reach an accord with the ENS in the future, so that the scant resources available to neurology will not be duplicated unnecessarily.

We look forward to the election of new officers who will carry us into the future with the selection of the site of the next World Congress of Neurology, during the time of this upcoming Congress. There will be much excitement and many decisions in London. I hope that all who possibly can will attend.

European Federation of Neurological Societies Cooperation Workshop, Trest, Czech Republic - Pro- James F. Toole, MD fessor Franz Gerstenbrand and Dr. Friederike Tschabitscher. President, World Federation of Neurology

WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org WFN COMMITTEE MEETINGS 5

WFN COMMITTEE MEETINGS AT THE ADDITIONAL AAN CANDIDATES Dr Theodore Munsat chaired meetings Munsat’s position as a trustee of the WFN FOR WFN of the Research Committee and Continu- continues to the end of 2001 but he holds ing Education Committee in Philadelphia it in a personal capacity. To assist in the ELECTIONS during the recent annual meeting of the process of selecting the next Research American Academy of Neurology. Attend- Committee Chair, suggestions were invit- The recommendations of the Nomi- ance was not expected to be large, given ed from those present and further con- nating Committee for candidates for that meetings of both committees will be sideration will be given to the matter in WFN positions were published in held at the World Congress in London. London. The Continuing Education Com- World Neurology, Volume 15 Number Nevertheless, some useful issues were mittee focused its attention on how best 4, December 2000. Under Article 6.3 aired. After the London meeting of the Re- to use its limited resources and there of the WFN Articles of Association, search Committee it is Dr Munsat’s in- was general support for directing efforts any further nominations supported by tention to distribute to Research Groups at neurology training programs. Lissette 5 or more authorised Delegates and a list of funding organizations interested Jimenez from Puerto Rico undertook to received by the Secretary-Treasurer in support of international health groups. work on a draft for a protocol to provide General at least thirty days prior to Under the new ‘constitution’ of the WFN modest financial support for training pro- the Annual General Meeting (Council (its so-called Articles of Association), the grams in developing countries. This will of Delegates) are to be added to the Research Committee will enjoy no special contain methods of making the program list put forward for consideration. status as it did in the past and will become known, reviewing requests and making The following two candidates will be a Standing Committee like any other. Dr awards. added to the lists for the positions in- dicated: WFN TASK FORCE ON NEUROLOGICAL Dr Antonio Culebras (USA) SERVICES First Vice President Professor Leontino Battistin (Italy) The Task Force on Neurological Services orders; stroke; brain and spinal injuries European Regional Vice President met in Philadelphia, Pennsylvania, USA, from road accidents, falls, self-injury, and during the American Academy of Neurolo- war; neurological complications of HIV; gy’s annual meeting. The Task Force has and other neurological disorders. It also just completed a report for the WFN Man- reveals the lack of reliable global health agement Committee on the global burden information available. The ‘manpower’ are the very same ones where neurologi- of neurological disease, and a survey on survey revealed that some countries have cal expertise is lacking or unavailable. A the number, training, and distribution of as few as one neurologist per million pop- summary of both of these projects will be neurologists in WFN member countries. ulation, and that most neurologists work presented at the WFN Congress in June. The report summarizes the tremendous in large cities in private practice. Taken to- toll on world health taken by neurode- gether, these reports identify a ‘neurology Donna C. Bergen velopmental disabilities and cognitive de- gap’ in many regions, in that the regions Chair, WFN Task Force on Neurological cline due to malnutrition and perinatal dis- where most neurological disorders occur Services ANNUAL REPORTS OF WFN RESEARCH GROUPS YEAR 2000

Research Group on papers on a wide range of neuroepidemi- Douglas Galasko, of the San Diego VA Neuroepidemiology ologic topics were presented. An interna- Medical Center. The Board meeting of the (WFN-RGNE) tional group of neurologists and epidemi- editors of the journal Neuroepidemiology ologists participated in representing many took place prior to the WFN-RGNE meet- On March 5, 2000, the WFN-RGNE met different countries. Dr. Friedland of San ing. Gustavo Roman, of the University of in San Diego, CA at Forum Hall for its Diego delivered the keynote address. The Texas Health Center in San Antonio was annual meeting. A total of 22 scientific meeting was organized and hosted by appointed as the new Editor-in-Chief re- placing Dr. Philip Gorelick of Rush Presby- terian St. Luke’s Medical Center. Dr. Alter WFN OFFICES BURGLED will continue to serve as Editor Emeritus. Dr. Roman plans to make the journal a The building at 12 Chandos Street in London, where the WFN Secretariat is located quarterly publication. While there is no of- and which it shares with two other organisations, was broken into during the night ficial connection between the journal Neu- of 18/19 April. Computer equipment was stolen from all three offices and damage roepidemiology and the WFN, that journal done to their doors and door frames. The WFN lost two computers, a printer, a serves as a suitable medium for neuroepi- scanner and a small amount of petty cash. More important, however, was the loss demiologic publications. At the business of data and files held on computer and it would be helpful to receive current e-mail meeting of the WFN-RGNE, it was decid- and fax addresses, not only from Delegates and Committee Chairs and members ed to hold the next annual meeting again but also others, such as Research Group Officers. We ask for your patience as we in conjunction with the AAN on Tuesday or try to re-build our systems and procedures. Wednesday, May 8 or 9 in 2001 in Phila- Keith Newton - Administrator delphia. The meeting will be held in the

WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org 6 WFN ANNUAL REPORTS 2000 evening instead of at the beginning or the ference and provides a forum for those analysis. However, we started a Web-site end of the week. The WFN-RGNE meeting closer to Europe to promote the history of open to everybody (www.teamspace.net/ will last 4 instead of 8 hours and posters neuroscience in general and neurology in csf) where consensus and typical profiles as well as abstracts will be presented in particular. It may be that a more clearly of CSF abnormalities will be presented in order to accommodate 20 to 30 abstracts local flavour will be introduced into those the near future. One face-to-face meeting usually submitted. The meeting in Phila- meetings with discussion of neurohistory of the Consensus Group was organized delphia will be hosted by Drs. Milton Alter matters related to the city in which the in Göttingen on May 18th-20th, 2000 by and Gary Friday. The membership roster meeting takes place but the work of the Prof. H. Reiber. of the WFN-RGNE now includes approxi- panel is wide-ranging. For further details Another international meeting was held in mately 600 names. It will be updated reg- please also approach Christopher Gard- Marburg on September 29th-30th, with the ularly. The RGNE will continue to charge a ner-Thorpe at the e-mail or fax numbers support of the CSF Research Group. This minimal registration fee to cover costs of given above. meeting, organized by Professor Kleine, lunch and coffee for the annual meeting. was entitled “European CSF Symposium A nominating committee was appointed Christopher Gardner-Thorpe on Laboratory Diagnosis of Human Brain to elect the next President of the RGNE Injury and Inflammation” and was attend- as the term of the current President, Dr. Autonomic Disorders ed by 80 participants. Milton Alter, will end in June 2001, at the Research Group 2000 next quadrennial meeting of the WFN in Annual report Neurotoxicology London, England. Dr. Walter Rocca and Research Group Annual Dr. Gustavo Roman will serve on that com- Horacio Kaufmann, Eduardo Benarroch, mittee. Roy Freeman, Pietro Cortelli and Chris Report 2000 Dr. Milton Alter, Mathias have organized scientific activities The Neurotoxicology Research Group President, WFN-RGNE related to the autonomic nervous system (NRG) was added to the list of Research that will take place at the XVII World Con- Groups of the World Federation of Neurol- gress of Neurology in London. There will ogy in 1999; Dr. Robert G. Feldman was Research Group on the be a Scientific Symposium and an Educa- introduced as its first Chairman by Dr. Ted History of the tional Symposium to update general neu- Munsat, Chairman of the Research Com- Neurosciences rologists on new developments in the au- mittee, at the meeting held at the Hyatt tonomic field. Regency Hotel in San Diego, California on The Research Group on the History of Work is ongoing on a project studying May 2, 2000. the Neurosciences, one of the Research possible involvement of the autonomic Groups of the World Federation of Neu- nervous system in the Cuban epidemic Defining a Mission rology, is a forum for those who are inter- neuropathy. Forty-five patients have been ested in recording, exploring and publish- studied to date. This is a joint project Dr. Robert G. Feldman consulted with ing history. The Journal of the History between the Instituto de Neurologia y WFN President, Dr. James Toole immedi- of the Neurosciences, founded by Frank Neurocirugía de La Habana, Cuba, Mt. ately after the meeting in San Diego to Clifford Rose as Founding Editor, is the Sinai School of Medicine and Harvard learn more about the WFN and to benefit official organ of this group (and also of Medical School. Dr. Joel Gutierrez, Director from his experience. At another meeting, the International Society of the History of Neurophysiology at the Cuban Institute held later during the year in Boston, Dr. of the Neurosciences and of the Euro- spent three months from March to May Munsat and Dr. Feldman reviewed the pol- pean Club). The World Federation Group 2000 at Dr Kaufmann’s laboratory in New icies and procedures necessary for or- meets at each World Congress in order York to complete the analysis of collected ganizing a new Research Group. The suc- to present a series of papers. On the oc- data. cesses and pitfalls of previous committee casion of the World Congress of Neurol- programs were discussed. It was decid- ogy in London on Tuesday 19 June 2001 a The Research Group has been discussing ed that the mission of the new Neurotoxi- session is devoted to the history of clinical several initiatives, including sponsoring a cology Research Group would focus on diagnosis where five speakers will cover consensus conference on the diagnostic topics of Neurotoxicology as they relate the structure and function of the nervous criteria of pure autonomic failure. Funding to Occupational and Environmental Ex- system as viewed over the ages, the resources are still being explored. posures to chemicals, encountered by flexes, the concepts and description of Our funds are held in an account at Mount people throughout the world. Special at- disease, the examination of the patient Sinai School of Medicine under the WFN tention would be directed towards edu- and imaging. Platform presentations will name. cational and research programs dealing deal with other historical topics. Members with conditions and problems that exist of the Research Group contribute to Horacio Kaufmann, M.D. in newly emerging and industrializing na- meetings worldwide and the group is Chairman Autonomic Disorders tions. Efforts would be made to bring re- pleased to include within its membership Research Group sources and expertise from established those interested in the history of the academic centers to communities in need neurosciences who may wish to ap- CSF Research Group and desirous of developing educational proach Christopher Gardner-Thorpe at materials, programs, and research op- [email protected] or by fax The development of a network on the In- portunities. It is hoped these will lead to to + 44 1392 402721 ternet has been pursued during the year a better appreciation of the risks of ex- 2000. Interactive communications are re- posure to various man made chemicals The European Federation of Neurological stricted to some members of the group and/or biologically available substances Societies (EFNS) panel on the History in order to obtain consensus about con- that can alter the structures and functions of Neurology meets at each EFNS Con- trol quality and standardization of CSF of the nervous system.

WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org ACUTE STROKE UNITS IN AUSTRIA 7 Inviting Participation of environmental medicine. These people Funding Sources Interested Persons were invited to attend the organizational meeting of the NRG on Wednesday, June Possible sources of funding to help organ- An informational letter from the Chairman 20, 2001 from 08.00 to 09.30 in London ize the setting up of the Group include the was sent to an initial group of 35 individuals during the World Congress of Neurology. Federation of American Societies for Ex- who were selected from the membership A room has been booked and responses perimental Biology (FASEB) (MARC Pro- list of the American Academy of Neurology have already been received from several gram Activities); the British Ecological So- and from a list of names recognizable to Dr persons who plan to attend and from ciety; and Biological Databases and Infor- Feldman as having contributed to the fields others who hope to participate in the matics. of neurotoxicology, and occupational and Group in future. ACUTE STROKE UNITS IN AUSTRIA Since 1997 stroke units have been estab- unit in Austria includes a defined and favourable cases. This system does not lished in Austria to function as a nation- closed region of 4–8 beds that are ex- aim at treating all incident strokes but wide network for acute stroke treatment. clusively dedicated for acute stroke pa- only some 60–70 percent by 2005. Other At present some 25 units are functional tients. This unit requests the leadership cases of stroke are treated within general and this number is expected to double by of a neurologist, a board certified neurol- medical departments (varying by region). 2005 to form a complete network for the ogist must be present 24 hours, an in- Mostly these are cases that have a more country’s 8 million inhabitants. The Austri- ternist must be available (present or on pronounced prestroke disability and thus an health authorities have commissioned call) 24 hours, at least 1 nurse is assigned a higher probability of multimorbidity. It is a plan that has the following specifics: per bed, one physiotherapist, one occu- important not to draw a line set by age 1) Stroke units are exclusively set up in pational therapist and one speech thera- alone, as there is no scientific evidence neurological departments. 2) Isochrones pist must be assigned to the unit, continu- that favours triage by age criteria alone. (time for ambulances to cover any given ous monitoring of vital parameters (ECG, Rather, a higher probability of multimor- distance to the nearest stroke unit) should breathing pattern, pulse rate, blood pres- bidity (as is also reflected by many cases not exceed 90 minutes from any commu- sure, oxygen saturation), CT immediately with high age) tends to be treated primari- nity in the country. 3) Stroke units have a available 24 hours (MRI available during ly in medical wards. These medical wards predefined structure quality and this qual- the day hours), availability of Duplex are motivated to set up mobile stroke ity is controlled by the regional health sonography and transcranial Doppler teams where neurologists also participate authorities. 4) Reimbursement for “acute sonography within the department, arte- but need not be available 24 hours (as is stroke treatment“ is allocated to hospitals riography (reachable within 30 minutes), the case in many rural areas). only for patients treated within stroke neurosurgery (reachable within 30 min- units. utes), intensive care ward available within The total number of some 500 board certi- the same hospital. fied neurologists in Austria can cope with To acknowledge this ambitious achieve- this workload. This plan of action has also ment it is necessary to comprehend that An evaluation of the process quality of led to the establishment of new neurologi- Austria’s health system is almost com- 15 stroke units within the year 2000 has cal departments and to the growing rec- pletely state controlled. Due to a general shown that these units have in the mean- ognition of neurologists to participate in health insurance that is obligatory (a pri- time become well accepted by the medi- emergency medicine. vate one can be held additionally) social cal emergency system as well as by the or class differences for the availability of general public. A prospective documen- such a health care system are negligible. tation of more than 2,000 acute stroke It has been possible to set up a central patients treated in these stroke units has plan for acute stroke units as well as shown that 30% of all cases are admitted for additional neurological beds for early within 120 minutes after stroke onset, 50% rehabilitation that are assigned either to of all cases have an emergency CT within the same neurological department or to a 30 minutes after their admission, and 4% neurological department within the region of all patients undergo thrombolysis. 20% of the stroke unit. These rehabilitation of all patients had severe strokes, in addi- beds are financed separately. They are tion the proportion of haemorrhages was defined either by additional nursing needs 10%, and in total more than 5% were Prof. Michael Brainin, MD (1.5 nurses per bed) or by intensive reha- soporous or in coma. The average stay President of the Austrian Society for bilitation programmes (at least 3 hours of within the unit was 3–5 days and mortal- Stroke Research, Chairman of the EFNS rehabilitation per day). ity was 12%. This shows that these units fulfil all the functions for all cases of stroke Stroke Scientists’ Panel The structure quality for an acute stroke and do not only accept the prognostically E-mail: [email protected] PAN-ARAB UNION OF NEUROLOGICAL SCIENCES

History and Update Meeting in Barcelona, Spain, in 1973. This ence and the late Dr. Ahmad Al Banhal inspiration came into reality in 1975 when as Secretary. This meeting was attended The Pan-Arab Union of Neurological Sci- the Egyptian Society of Neurology, Neuro- by Dr. Jamal Al Din Behairi, Secretary- ences (PAUNS) was founded through the surgery and Psychiatry called for its first General for the Pan-Arab Physicians. The inspiration of Arab neurologists who at- meeting in Cairo, Egypt. Dr. Yahya Taher delegates who attended from the differ- tended the World Federation Neurology was elected as President of the Confer- ent Arab countries were: Immaddin Farid

WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org 8 WFN JUNIOR TRAVELLING FELLOWSHIPS

followed by a one-day Tropical Neurology Conference. This meeting was a success and attended by more than 400 members. The last meeting was conducted in Beirut, Lebanon, on 13-16 April 2000, and was held under the patronage of His Excellen- cy, Dr. Karam Karam, Minister of Public Health, who gave the opening remarks. Prof. Samir Atweh, Head of Neurology, American University of Beirut, was the President of the Conference, and the number of attendees was around 500. Many papers were submitted and several workshops were conducted, and the Con- ference was a great success. Also, regional neurological symposia in different Arab countries were held successfully under the auspices of PAUNS.

Opening ceremony during the 8th PAUNS Conference in Beirut, Lebanon (April 13–16, 2000) attended As neuroscience has been expanding in by His Excellency Dr. Karam Karam, Minister of Public Health, and Dr. Sohail Gebeily, President of the Lebanese Neurological Society. the Arab world, a few important societies have been established through the Pan- Arab. In 1994, the Pan-Arab Neurological (Bahrain), Mohammad Abadu (Algeria), The second meeting was in Tunisia (1978), Society was formed, and Dr. Mohamed Ashraf Al-Kurdi (Jordan), Daoud Mustafa and the third was in Jordan (1984). Aabed Al Thagafi is the current President. (Sudan), Tariq Hamdi (Iraq), Sohail M. Also, the Arab Neurological Society was Saleh (Kuwait), Abdul Rahman Al-Wazza- Dr. Ashraf Al-Kurdi was elected in 1984 as formed in December 1995, and Dr. Khalaf zi (Morocco), Baltayeb Abdul Karim (Tuni- the Secretary-General. The fourth meet- Al Moutaery was elected as the President. sia), Faisal Al-Sabag (Syria), Foud Hadad ing was convened in Algeria (1986), and As a member of the WFN, we have revised (Lebanon), and Yehya Taher (Egypt). the fifth meeting was held in Tunis (1993). the Constitution of the PAUNS, and as it Dr. Mohamed Emad Al-Deen Fadli was stands, it has been amended and agreed Dr. Ahmed Al Banhawi was elected as the elected as Secretary-General in Tunisia by the majority of the members. There are first Secretary-General for the PAUNS. As- (1993). The sixth meeting was held in many tasks to achieve, and we are plan- sistants to the Secretary-General were Dr. Egypt (1995), and the seventh meeting ning to have a proper established consti- Tariq Hamdi (Iraq), Dr. Mohamed Emad Al- was conducted in Saudi Arabia (1997). Dr. tution for the PAUNS. This cannot be done Deen Fadli (Egypt), and Dr. Jamal Assaf Saleh M. Al Deeb was elected as Secre- without the cooperation of all the mem- (Egypt). Mr. Abdulhakim Al Marsafawi was tary-General in Riyadh (1997). Later, the bers. elected as Treasurer. Dr. Sayed Al Jindi title of Secretary-General was changed to was elected to be the Representative be recognized as President of PAUNS. Saleh M. Al Deeb, of the PAUNS to the Arab European So- During the 7th meeting, an extensive pro- President, PAUNS, Riyadh Armed Forces ciety for Neurosciences. After the estab- gram has been done with faculties world- Hospital, Box 7897, Riyadh 11159, Saudi lishment of the PAUNS, several meetings wide. Also, in this Conference, the 1st Arabia; E-mail: [email protected] were conducted, though it was not regular. PanArab Epilepsy Conference was held

WINNERS OF 2001 JUNIOR TRAVELLING FELLOWSHIPS Name Country Meeting 1. Dr M.A. Alleem India XVII World Congress of Neurology, London 2. Dr Manvir Bhatia India 48th Annual Meeting of American Academy of Electrodiagnostic Medicine, New Mexico 3. Dr A. Nalini India XVII World Congress of Neurology, London 4. Dr Pramod Kumar Pal India XVII World Congress of Neurology, London 5. Dr Jeyaral Pandian India XVII World Congress of Neurology, London 6. Dr Atul Prasad India XVII World Congress of Neurology, London 7. Dr Nazha Birouk Morocco XVII World Congress of Neurology, London 8. Dr George Kuchukhidze Georgia XVII World Congress of Neurology, London 9. Dr Zdenek Novotny Czech Republic XVII World Congress of Neurology, London 10. Dr Paul Pasco Philippines XVII World Congress of Neurology, London 11. Dr Seyam Saeed Egypt XVII World Congress of Neurology, London

WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org VASCULAR DEMENTIA 9 WFN JUNIOR TRAVELLING FELLOWSHIP REPORTS I am very thankful to the World Federa- imagery and neurobiology. that was the icing on the cake. tion of Neurology that had permitted me We are very grateful to the WFN for ena- thanks to its award to participate in the The Travelling Fellowship also helped me bling neurologists from developing coun- 125th Congress of the American Associa- attend the 6th International Symposium tries to benefit from recent research in dif- tion of Neurology held in Boston, 15–20 on Acute Stroke and Thrombolytic Ther- ferent domains of neurology. October 2000. apy held in Hamilton Island immediately after the World Stroke Congress. I am Dr Ibtissem Ben Hamouda This event allowed me an update on most sure the experiences I have gained par- Tunisia neurological fields, particularly on cere- ticipating in these two conferences would brovascular diseases and cognitive dis- help me contribute towards improving the orders that constituted for me particular I was delighted to win a WFN – Glaxo stroke services in my country. topics of interest. Wellcome Junior Travelling Fellowship to attend the 4th World Stroke Congress in This was my first visit to Australia, and I It had also provided me the opportunity to Melbourne, Australia, in November 2000. took the opportunity to do as much ‘sight discuss with neurologists from all over the The Congress was a great experience. I seeing’ as possible within the limited time. world and to exchange with them ideas had the privilege to listen to, and share Watching the koalas and kangaroos at the concerning recent and important progress experiences with, many neurologists and Healesville sanctuary, snorkeling at the of neurology in the domain of basic sci- stroke physicians, including some of the Great Barrier Reef, and marvelling at the ences and therapy. Gene therapy sounds leading experts in the field. The scientific Penguin Parade on the Philip Island beach very promising in numerous neurological sessions dealt with many a new advance are some of the memories I will cherish for diseases. at the cutting edge of stroke medicine. I a long time. I noticed the exceptional quality of inter- was glad to be given the opportunity to Thank you, WFN and Glaxo Wellcome, for ventions during the presidential sympo- present two papers on our experience of a wonderful experience. sium that analyzed the relationship be- stroke care in Sri Lanka at the Congress. tween conscience, behaviour and emo- The best moment of all was when I won Udaya K. Ranawaka tion in light of the new data of the neuro- a “Young Investigator of the Year” award; Sri Lanka FIRST LACTRIMS CONGRESS Several years ago a group of Latin-Amer- gress to be held in Buenos Aires the fol- Also, there were 97 posters from Argen- ican visionaries interested in one of the lowing year. tina, Brazil, Canada, Colombia, Cuba, more chronic, crippling and challenging From 9 to 11 November 2000, more than Spain, Israel, Mexico, Panama, Puerto diseases decided to combine their efforts 300 neurologists and scientists from relat- Rico, Switzerland, Uruguay, USA, and to found LACTRIMS (Latin American Com- ed disciplines gathered with great enthu- Venezuela. mittee for the Treatment and Research siasm to attend this international meeting in Multiple Sclerosis) to research the real The meticulous organization, the modern in the beautiful city of Buenos Aires. The state of Multiple Sclerosis in our countries and comfortable settings, the high level of Organizing Committee was chaired by and to explore comparisons with North- the scientific sessions and the warm and the dynamic Dr. Leonor Gold with the American and European knowledge in gentle friendship made for a successful assistance of Dr. Edgardo Cristiano as order to benefit our neurologists and their 1st LACTRIMS Congress. Vice-President, Dr. Marco Lana Peixoto as patients and families. Treasurer and several neurologists from The farewell dinner was in “Senor Tango” The official foundation of LACTRIMS was different Latin-American countries. an amazing temple dedicated to the two in Cartagena de Indias, Colombia, during Argentinean traditions: the dance and the the X Pan-American Congress of Neurol- There were 44 speakers from Argentina, delicious meat, and everybody enjoyed ogy in October 1999 where the General Brazil, Canada, Chile, Colombia, Cuba, them very much. All the participants were Assembly elected Dr. Leonor Gold (Ar- Italy, Mexico, Peru, Switzerland, United agreed that we must recommend to our gentina) as President, Dr. Victor Rivem Kingdom, Uruguay, USA, and Venezuela. MS patients to dance the tango as one of (Mexico) as Vice-President, Dr. Edgardo The themes included Epidemiology in the rehabilitation strategies! Cristiano (Argentina) as General Secretary, Latin America, Natural History, Diagnostic Dr. Victor Rivera, as President, will or- Dr. Marco Lana Peixoto (Brazil) as Treasur- Criteria and Clinical Forms, Role of Im- ganize the 2nd LACTRIMS Congress in er, and Drs. Carlos Oehninger (Uruguay), agenology, Complementary Tools, Cogni- Mexico, in 2002, and we can be sure of its Jorge Luís Sánchez (Colombia) and Dr. tive Impairment, MS Centers, MS in Child- success. José Antonio Cabrera Gómez (Cuba) as hood, MS and Pregnancy, Assessment Vocals. Also, the Committee approved the Scales, Treatment, and a special session Dr. Gustavo Pradilla organization of the 1st LACTRIMS Con- dedicated to patients and their families. Bucaramanga, Colombia VASCULAR DEMENTIA: IT IS TIME FOR A NEW APPROACH Current concepts of vascular dementia are Obsolescence of impairment short of dementia (CIND). The obsolete. Conventional definitions iden- cornerstone of all criteria of dementia is tify the patients too late, miss subjects current concepts of memory impairment. This criterion works with cognitive impairment short of demen- vascular dementia very well for Alzheimer’s disease, where tia, and emphasize consequences rather memory is an early and constant feature, than causes, the true bases for treatment For each patient considered demented, but it seldom helps in identifying individu- and prevention. there is another individual with cognitive als with cognitive impairment on a vascu-

WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org 10 VASCULAR DEMENTIA lar basis. About 80% of all strokes occur in the carotid artery distribution, only 20% af- FELLOWSHIP TRAINING PROGRAM IN fecting the vertebrobasilar system, which supplies the hippocampi. While strokes in NEUROPSYCHIATRY the medial temporal lobes cause memory The Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez (Na- impairment, it usually takes bilateral le- tional Institute of Neurology and Neurosurgery) in Mexico City is opening a Fellow- sions to cause serious and permanent ship training program in Neuropsychiatry, a field that tries to understand the com- memory problems. Strokes affecting cog- plex inter-relationships between human behaviour and brain function by incorporat- nition occur most commonly in the fronto- ing the areas of psychiatry and neurology. Over the past several years, there has basal systems that subserve judgement, been an increasing interest in neuropsychiatry as a sub-specialty which focuses planning and emotion, features seldom on the applications of insights from basic and clinical neuroscience research to the tested in cognitive screens. The most fun- study of psychopathology. damental problem with current criteria of The fellowship is directed at neurologists and psychiatrists. It is a one year long dementia is that they do not work. In training program, oriented not only towards research activities but also to clinical the Canadian study of Health and Aging, evaluation and treatment. For more information, please contact Dr. Ignacio Ruiz- which is both a population and institution López: Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez based study, 1879 subjects were identi- Insurgentes Sur 3877, C.P. 14269, México, D.F. Phone number and fax: (52) fied as demented by consensus. Then six 56064532; e-mail: [email protected]. commonly used criteria of dementia were applied to the same subjects. Little over- lap emerged, and a surprising 10-fold dif- From multi-infarct risk of stroke, vascular death, cognitive ference separated the least and the most dementia to vascular deterioration and with a 3–4-fold increased sensitive criteria. According to ICD-10 cri- cognitive impairment risk of intracerebral bleeding in patients teria, 3.1% of the population over the age placed on anticoagulants. of 65 years are demented, by DSM-III cri- We suggested the term “multi-infarct de- teria 29.1% are! mentia” to emphasize that when demen- Co-existence and tia occurs on a vascular basis, it is usually possible interaction for Ascertainment and due to multiple infarcts and not to the slow strangulation of the brain’s blood supply stroke and Alzheimer’s classification biases resulting in chronic cerebral ischemia and disease neuronal starvation and death. While mul- Snowden et al have shown that among Most clinicopathological studies are car- ti-infarct dementia occurs, it is rare in pure elderly nuns having the pathological di- ried out on patients who have been identi- form. What is much more common is agnosis of Alzheimer’s disease only 57% fied through memory or Alzheimer clinics. cognitive impairment on a vascular basis were demented. Among those having the Since they come to attention because of short of dementia. Thus, Bowler and I pathological diagnosis of Alzheimer’s dis- memory problems, most of them will turn have suggested the term “vascular cog- ease plus cortical infarcts 75% were de- out to have Alzheimer’s disease. By con- nitive impairment” to describe the spec- mented and among those having patho- trast, patients with cerebrovascular dis- trum of cognitive impairment on a vascular logical Alzheimer’s disease and small in- ease usually come to medical attention basis, from mild impairment to frank de- farcts in the subcortical areas, 93% were because they have had transient ischemic mentia. The commonest context of multi- demented. Clearly the effects of Alzheim- attacks or strokes. If these patients have infarct dementia occurring in patients with er’s disease and strokes add up or per- cognitive impairment, it is often frontal Alzheimer changes i.e. “mixed dementia”. haps interact, since lesions in the brain lobe dysfunction, which is difficult to di- The “ischemic score” distinguishes very do not add up, they multiply. In a clinical agnose and test. Even if identified, these well pure Alzheimer’s disease from mul- pathological study addressing the ques- patients seldom find their way into clinico- ti-infarct and mixed dementia but not be- tion of education and dementia, no differ- pathological studies of dementia because tween the latter two. This does not matter ence was found in the progression of de- the current definitions of dementia require pragmatically, the important point being mentia once diagnosed, among individu- memory impairment as the first requisite, the identification of the treatable vascular als with a primary education, secondary and memory is seldom severely impaired component. education and college or university edu- in patients with cerebrovascular disease. Leukoaraiosis cation. Similarly, the degree of brain at- Thus there is a systematic overrepresen- rophy, presence of senile plaques and tation of Alzheimer cases in clinicopatho- We coined the term “leukoaraiosis” to de- neurofibrillary tangles was comparable logical series. This is compounded by an- scribe white matter rarefaction as seen on among the educational groups. However, other phenomenon. Most clinicopatholog- brain imaging and to emphasize that al- the least educated patients had signifi- ical studies claim an accuracy of about though ischemia is often invoked as the cantly higher prevalence of lacunar inf- 90% in the diagnosis of Alzheimer’s dis- cause, white matter changes may be due arcts, white matter lesions, macroscopic ease. However, only patients who die to multiple causes. While white matter infarcts and other vascular lesions than come to autopsy, typically 7–8 years after changes can occur in the elderly without the other two groups with higher educa- diagnosis in Alzheimer’s disease. If one any clinical correlates, consistent association. includes in the denominator the 23% of tions have been described between prov- patients who initially are diagnosed as entricular white matter changes and cog- The need for a new Alzheimer’s disease who do not deterio- nition. Subcortical white matter changes approach rate cognitively and survive, the accuracy may be accompanied by depression. Leu- drops to about two-thirds. koaraiosis is associated with an increased We need to take a pragmatic conceptual

WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org MITOCHONDRIAL ENCEPHALOMYOPATHIES 11 retreat before we are ready to advance at understanding, but will give us the basis Dr. Hachinski has been named Editor-in- an accelerated pace. We need to identify for therapy. Already, enough scientific ra- Chief of the journal Stroke. This American potential patients long before they are de- tionale exists for undertaking systematic Heart Association (AHA) publication is the mented, including symptomless individu- clinical trials in the prevention of cognitive leading scientific journal in the field. Dr. als at high risk of developing cognitive im- impairment through the control of vascu- Hachinski had previously been chair of the pairment. When subjects have cognitive lar risk factors and the use of statins, anti- Scientific Programme Committee of the impairment, this should be characterized inflammatory agents and ACE inhibitors. annual International Stroke Conference of clinically and by a commonly agreed mini- We need to overturn our current approach the AHA, the only person to hold both mum of standardized tests. We may have of gathering data into arbitrary, dogmatic, positions. His contributions include pio- to make an initial diagnosis of a “cognitive obsolete categories and begin developing neering with Dr. John W. Norris the es- syndrome” and become more precise as a new pragmatic, systematic, data based tablishment of stroke units, subsequently the data allow. approach. proven to improve morbidity and mortal- ity of stroke patients, discovering the key Conclusion role of the insula of the brain in cardiac complications of stroke including sudden death, crystallizing the concepts and coin- We should abandon current diagnostic ing the terms “multi-infarct dementia”, categories and describe cognitive impair- “leuko-araiosis” and “brain attack” and ment clinically and according to com- devising the “ischemic score” that bears monly agreed instruments that document his name. The headquarters of the journal the demographic data in a standardized have been moved to London, Ontario and manner and undertake a systematic effort Dr. Hachinski has also established associ- of identifying the underlying etiology in ate editors’ offices at the University of Hel- each case. Imaging and DNA should be sinki, Finland, the Royal Perth Hospital, obtained whenever possible. An empiric Australia, and Harvard University, U.S.A. approach and the use of mutually intelligi- Vladimir Hachinski, M.D., FRCP(c), D.Sc., ble descriptions will contribute to a knowl- Chair, Steering Committee and WFN Vice- edge base that will increase not only our President for North America MITOCHONDRIAL ENCEPHALOMYOPATHIES

Mitochondrial myopathies have been first biochemical documentation of mito- tabolism, it is unduly restrictive in that known since the early 1960s, when sys- chondrial dysfunction was provided by mitochondrial diseases, and especially tematic ultrastructural and histochemical Professor Rolf Luft and coworkers in a those due to defects in mitochondrial DNA studies, pioneered by G. Milton Shy, Ni- patient with exercise intolerance, RRF, (mtDNA), can affect any tissue in the body cholas Gonatas, and W. King Engel, re- and nonthyroidal hypermetabolism due and often cause protean multisystem dis- vealed excessive proliferation of normal- or to “loose coupling” of muscle mitochon- orders. abnormal-looking mitochondria in muscle dria. It soon became apparent, especially of patients with weakness or exercise to pediatric neurologists, that in many In 1988, the era of mitochondrial genetics intolerance. Because, with the modified patients with RRF, the myopathy was was ushered in by two seminal papers, Gomori trichrome stain, the areas of mi- overshadowed by diverse symptoms and one from the group of Anita Harding, the tochondrial accumulation looked like pur- signs of brain involvement, and the term other from the group of Douglas Wallace, plish blotches, the abnormal fibers were mitochondrial encephalomyopathies was describing the first pathogenic mutations dubbed “ragged-red fibers” (RRF) and introduced. While this label correctly high- in human mtDNA. Human mtDNA is a came to be considered the pathological lights the special vulnerability of brain 16.5-kb circular, double-stranded mole- hallmark of mitochondrial disease. The and muscle to defects of oxidative me- cule, which contains 37 genes: 2 rRNA genes, 22 tRNA genes, and 13 structural genes encoding subunits of four complex- INTERNATIONAL FEDERATION OF es of the respiratory chain (7 subunits of complex I; 1 subunit of complex III, 3 sub- MULTIPLE SCLEROSIS units of complex IV; and 2 subunits of complex V). According to the widely ac- The 2001 International Multiple Sclerosis Conference cepted “endosymbiotic hypothesis”, mi- Grand Hyatt Melbourne, Australia tochondria are the remnants of protobac- 30th September – 4th October 2001 teria that populated anaerobic nucleated Conference Program includes: • International Federation of Multiple Sclerosis cells and endowed them with the precious Meetings • MS Health Professionals Conference • MS Conference Day for people gift of oxidative metabolism. This explains with MS • International & Australian Speakers • Management of Severe Cognitive how eukaryotic cells are “genetic mosa- Disability Symposium • Research Symposium: Current Issues & Direction • Inter- ics”, containing their own nuclear genome national Summit on Nonprofit Leadership for Boards & Executives (nDNA) plus multiple mitochondrial ge- nomes. This scenario helps explain the Register online at: http://www.msaustralia.org.au central concepts of mitochondrial genet- e-mail: [email protected] ics: phone: Nadia Agostinelli on +61 3 9828 7222; fax: +61 3 9828 9054 Address: 34 Jackson Street Toorak, Victoria 3142, Australia (i) Heteroplasmy and the threshold effect. Each cell contains hundreds or thousands

WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org 12 MITOCHONDRIAL ENCEPHALOMYOPATHIES of mtDNA copies, which, at cell division, and heart block. Frequent additional signs (primary mutations). distribute randomly among daughter cells. include ataxia, dementia, endocrine prob- While exercise intolerance is a common In normal tissues, all mtDNA molecules lems (diabetes mellitus, hypoparathy- complaint in patients with mitochondrial are identical (homoplasmy). Deleterious roidism). (iii) PEO with or without proxi- encephalomyopathies, it is often over- mutations of mtDNA usually affect some mal limb weakness, often compatible with shadowed by other symptoms and signs. but not all mtDNAs within a cell, a tissue, a normal lifespan. Deletions vary in size Only recently we have come to appreci- an individual (heteroplasmy), and the clin- and location, but a “common” deletion of ate that exercise intolerance, myalgia, and ical expression of a pathogenic mtDNA 5 kb is frequently seen in patients and myoglobinuria can be the sole presenta- mutation is largely determined by the rela- in aged individuals. Duplication of mtDNA tion of respiratory chain defects. These tive proportion of normal and mutant ge- can occur in isolation or together with are due to mutations in mtDNA genes en- nomes in different tissues. A minimum single deletions and have been seen coding subunits of complex I, complex critical number of mutant mtDNAs is re- in patients with KSS or with diabetes IV, or, more frequently, cytochrome b. As quired to cause mitochondrial dysfunc- mellitus and deafness. Duplications and these disorders are usually tissue-specif- tion in a particular tissue. (ii) Mitotic seg- duplications/deletions are transmitted by ic and sporadic, they are apparent ex- regation. At cell division, the proportion maternal inheritance. ceptions to the “common wisdom” that of mutant mtDNAs in daughter cells may mtDNA point mutations are invariably as- shift and the phenotype may change ac- B. Point mutations sociated with multisystemic, maternally in- cordingly. This phenomenon, called mitot- Over 100 pathogenic point mutations have herited conditions. ic segregation, explains how certain pa- been identified in mtDNA from patients tients with mtDNA-related disorders may with a variety of usually maternally-in- actually shift from one clinical phenotype 2. Disorders due to herited and usually multisystemic disor- to another as they grow older. (iii) Mater- defects of nDNA ders. Among the encephalomyopathies, nal inheritance. At fertilization, all mtDNA common syndromes include: (i) MELAS These are all transmitted by mendelian derives from the oocyte. Therefore, the (mitochondrial encephalomyopathy, lactic inheritance and include two major sub- mode of transmission of mtDNA and of acidosis, and strokelike episodes), pre- groups. mtDNA point mutations (single deletions senting usually in childhood with recur- of mtDNA are usually sporadic events) dif- rent vomiting, migrainelike headache, and A. Mutations in genes fers from mendelian inheritance. A mother strokelike episodes causing cortical blind- encoding respiratory chain carrying a mtDNA point mutation will pass ness, hemiparesis, or hemianopia. CT it on to all her children (males and fe- subunits or “ancillary scan or MRI of the brain shows “infarcts” males), but only her daughters will trans- that do not correspond to the distribution proteins” mit it to their progeny. A disease ex- of major vessels, raising the question pressed in both sexes but with no evi- As pathogenic mutations start to over- of whether the strokes are vascular or dence of paternal transmission is strong- crowd the small circle of mtDNA, search- metabolic in nature. The most common ly suggestive of a mtDNA point mutation. ing for deleterious mutations in nuclear mtDNA mutation associated with MELAS The best way for the clinician to chart her genes controlling respiratory chain com- is A3243G in the tRNALeu(UUR) gene. (ii) course toward a diagnosis in the mare plexes has become the “new frontier” of MERRF (myoclonus epilepsy with ragged magnum of mitochondrial encephalomy- research in mitochondrial diseases. Sever- red fibers) is characterized by myoclonus, opathies is to use a classification that al mutations have already been identified seizures, mitochondrial myopathy, and combines genetic and biochemical cri- in genes encoding subunits of complex cerebellar ataxia. Less common signs teria. However, by a generally accepted I and complex II, mostly in children with include dementia, hearing loss, peripher- convention, the term “mitochondrial en- Leigh syndrome. The situation is more al neuropathy, and multiple lipomatosis. cephalomyopathies” is now restricted to complex (and more interesting) in patients The typical mtDNA mutation in MERRF is defects of the respiratory chain. Because with complex IV (cytochrome c oxidase, A8344G in the tRNALys gene. (iii) NARP of the dual genetic control of the respirato- COX) deficiency. Mutations have not been (neuropathy, ataxia, retinitis pigmentosa) ry chain, these disorders will be due either found in nuclear genes encoding COX usually affects young adults and causes to mutations in mtDNA or to mutations in subunits, but, rather, in genes encoding retinitis pigmentosa, dementia, seizures, nDNA. COX-assembly proteins, a sort of “murder ataxia, proximal weakness, and sensory by proxy” situation. Already four such an- 1. Disorders due to neuropathy. This syndrome is associated cillary proteins have been involved, again with a mutation (T8993G) in the ATPase6 mostly in children with Leigh syndrome, defects of mtDNA gene of mtDNA. When the same mu- and more are undoubtedly “waiting in the These include rearrangements (single de- tation is present in high abundance, it wings”. causes a more severe infantile enceph- letions or duplications) and point muta- B. Defects of intergenomic tions. alopathy with the neuropathological features of Leigh syndrome. (iv) LHON (Leb- signaling A. mtDNA rearrangements er’s hereditary optic neuropathy) is characterized by acute or subacute loss of As mentioned above, mtDNA is highly de- Single deletions of mtDNA have been as- vision in young adults, more frequently pendent for its proper function and repli- sociated with three sporadic conditions: (i) males, due to bilateral optic atrophy. Sev- cation on numerous factors encoded by Pearson syndrome, a usually fatal disorder eral different mtDNA point mutations in nuclear genes. Mutations in these genes of infancy characterized by sideroblastic structural genes (mostly genes encoding cause mendelian disorders characterized anemia and exocrine pancreas dysfunc- subunits of complex I) have been associ- by qualitative or quantitative alterations tion. (ii) Kearns-Sayre syndrome (KSS), ated with LHON, but only a few are exclu- of mtDNA. Examples of qualitative altera- a multisystem disorder with onset before sively present in patients and appear to be tions include autosomal dominant or au- age 20 of PEO, pigmentary retinopathy, pathogenic even when present in isolation tosomal recessive multiple deletions of

WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org BOOK REVIEWS 13 mtDNA, usually accompanied clinically of diagnosis’. It is indeed well done. The Focal Peripheral by progressive external ophthalmoplegia author has collected data on clinical man- Neuropathies (PEO) plus a variety of other symptoms ifestations of CNS angiitis both from nu- and signs. Two genes responsible for mul- merous reports as well as from his own 3rd Edition tiple mtDNA deletions have been iden- material. The author covers thoroughly tified. Mutations in one (encoding the giant cell arteritis, Takayasu’s arteritis, Ed: John D Stewart enzyme thymidine phosphorylase) are as- polyarteritis nodosa and the CNS involve- ISBN: 0-7817-1717-5 No. of pages: 580 sociated with an autosomal recessive ment in systemic lupus erythematosus as Price: US$95.00 / £69.00 syndrome known as MNGIE (mitochon- well as many other conditions. There are Publication Date: 2000 drial neurogastrointestinal encephalomy- special chapters on CNS angiitis due to Publisher: Lippincott, Williams & Wilkins opathy). Mutations in the other (encoding infection and CNS angiitis associated with an adenine nucleotide transporter, ANT1) drug abuse. The book is written in a fluent, This is the third Edition of a marvellous are associated with autosomal dominant sometimes epic style (“Behçet’s disease publication with descriptive, illustrative PEO. Examples of quantitative alterations is a mysterious disorder.” or “I hold little and clinically applicable discourse of focal of mtDNA include severe or partial expres- hope that newer imaging techniques such peripheral neuropathies. There are two sions of mtDNA depletion, usually char- as functional MRI, PET or NMK angiog- dozen chapters starting with the structure acterized clinically by congenital or child- raphy will be helpful in sorting out this of the peripheral nervous system includ- hood forms of myopathy or hepatopathy. confusing clinical problem”). He argues ing Electron micrographic description. The The mutant gene (or genes) and factors strongly that cerebral angiography is of author has given a good explanation of the associated with mtDNA depletion remain little value in the diagnosis of CNS angii- pathologic processes producing periph- unknown. tis. There is, however, only one illustration eral neuropathies, what generates electri- showing angiographic changes (possibly cal activity and how it is derailed in the pe- mis-) interpreted as showing angiitis, but ripheral neuro lesions – acute to chronic. with too few details to be convincing. The Diagnostic procedures are described in chapters on cerebrospinal fluid analyses detail. The author from the 5th chapter on- and of immunology are less informative. wards describes peripheral nerve lesions The strengths of the book are the detailed from upper cervical spinal nerve, cervical description of various forms and symp- plexus and nerves of the neck. There is tomatology of CNS angiitis, the discus- vivid description of brachial and lumul- sion of the value of leptomeningeal biopsy bosacral plexuses with illustrative details. and the documentation of the effect of The lesions of the individual nerves of Dr. Salvatore DiMauro, MD immuno-supression. upper and lower limbs and pelvis are me- Department of Neurology, ticulously given. The flow of the book is Dr. Johan Aarli Columbia-Presbyterian Medical Center, excellent and the reader will enjoy going Bergen, Norway New York, NY, USA over it. It is perhaps one of the rare books of its kind dealing only with focal neuropa- The Nervous System thies. The publication is strongly recom- BOOK REVIEWS and the Heart mended for practising neurologists, neu- rosurgeons and orthopaedic surgeons in- Ed : Gert J Ter Horst terested in dealing with focal peripheral ISBN : 0 89603 693 6 nerve diseases. It is also a must for insti- Central Nervous System No. of pages : 564 tutional Libraries. Angiitis Price : US$135.00 Publication Date : 2000 Editor-in-Chief Editor : Schmidley, J.W. Publisher : Humana Press ISBN : 0 7506 7153 X This interesting book on the association Parkinson’s Disease No. of pages : 216 between the heart and the nervous system Price : £55.00 and Movement is nicely edited by Gert Ter Horst. It con- Publication Date : 2000 sists of 5 parts and 13 sub-topics. The Disorders Publisher : Butterworth Heinemann main themes are autonomic control, the Diagnosis and Treatment hypothalamus pituitary adrenal system There have been few, if any, comprehen- and the heart, heart pain, the neuroanato- Guidance sive works covering the various aspects of my of cardiac nociceptive pathways, hu- central nervous system angiitis. This mon- Editors: Charles H Adler and J Eric moral factors, and finally heart disease ograph is therefore welcome. It is small Ahlskog and brain dysfunction. The book covers well updated to 1998 and has 578 literary ISBN: 0-896-03607-3 basic physiological, anatomical and its references. Apparently, the book is writ- No. of pages: 474 clinical implications, and explains very Price: $99.50 (hard cover) ten as an argument against the ‘wide- nicely many symptoms and signs for ex- Publication Date: 2000 spread misconceptions concerning angii- ample when there is deranged autonomic Publisher: The Humana Press tis and the central nervous system’ such dysfunction, or when there is abnormality as ‘the widespread, groundless belief that The 35 chapters of this book are written by in the renin angiotensin system. It is an es- the central nervous system manifesta- 35 different authors, each a highly experi- sential book for trainees in cardiology and tions of systemic lupus erythematosus are enced clinician and researcher. The book neurology, and is recommended for any caused by angiitis and an entirely mis- contains an immense amount of currently medical library. placed confidence in catheter angiogra- available information about various move- phy as a sensitive and specific means Adel Al Jishi (Bahrain) (cont. on p. 16)

WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org 14 ADVERTISEMENT

Special Product Highlight World Congress of Neurology

World Federation of Neurology - Seminars in As mentioned in the “Come and Meet us” section above, we will be at- Clinical Neurology tending the World Congress of Neurology from 17 to 22 June in London. Please come and visit us on booth number 3 in Earls Court 2. In addition Neurologic Bladder, Bowel and Sexual Dysfunction to free sample copies of all our key neurology journals - including Journal of the Neurological Sciences - we will also have free copies of The Lancet, Volume Editor: Clare J. Fowler and NewScientist.

ISBN 0-444-50678-0 In addition to the journals, we will have all our bestselling and most recently published neurology books and book series on display for you to Contents: browse at your leisure. Titles on display include volumes from the prestig- Editor’s Preface ious Handbook of Clinical Neurology Revised Series, Handbook of Neu- nd Chapter 1: A neurologist’s clinical and investigative approach to patients ropsychology, 2 edition, and Supplements to Clinical Neurophysiology. with bladder, bowel and sexual dysfunction - C.J. Fowler (London, UK) In the Electronic Corner you will be able to see our complete package of Chapter 2: Urinary incontinence and retention - C.M. Brady and C.J. electronic services, and be able to get hands-on experience yourself as well. Fowler (London, UK) Each exhibit day a different service will be demonstrated including the Lan- cet Neurology Network on Tuesday and Friday (www.lancetneuronet.com), Chapter 3: Constipation and fecal incontinence - C.M. Brady (London, Neuroscion on Monday and Thursday (www.neuroscion.com), and New- UK) Scientist’s website on Wednesday (www.newscientist.com). Chapter 4: Neurogenic sexual dysfunction in men and women - C.J. Fowler and E.M. Frohman (London, UK and Dallas, TX, USA) And … you will be able to win free journal subscription and other prizes, so please make sure you come and see us every day!! Chapter 5: Multiple sclerosis, other myelopathies and spinal cord injury - E.M. Frohman (Dallas, TX, USA) Clinical Neuroscience Research Chapter 6: Cauda equina disorders - J.D. Stewart (Montreal, PQ, Cana- da) Volume 1/3 out now! Chapter 7: Peripheral neuropathies - J.D. Stewart (Montreal, PQ, Cana- da) The Official Journal of the Association for Research Chapter 8: Parkinsonian disorders and pure autonomic failure - R. Sakak- in Nervous and Mental Disorders ibara (Chiba City, Japan) Editor-in-Chief: William E. Bunney, Jr., M.D., Depart- Chapter 9: Brain diseases - R. Sakakibara and C.J. Fowler (Chiba City, ment of Psychiatry, University of California, Irvine, Japan and London, UK) CA , USA. Text-based multiple choice questions Volume 1/3 is a special issue entitled “Normal and Pathological Develop- ment of the Nervous System”, Part I, guest-edited by Dr. Michael I. Posner To view the contents in more detail, go to http://www.wfneurology.com from the Sackler Institute in New York. and click on “Publications” in the left-hand bar. Contents: Come and meet us Normal and pathological development of the nervous system, Pages 173–174 Michael I. Posner Elsevier Science will have a booth at the meetings listed below. Please visit our booth to browse Neurogenesis in the adult mammalian brain, Pages 175–182 Nicholas B. Hastings et al the latest releases in our book program, see online demonstrations of our electronic products and Asymmetry, stem cells and the emergence of function, Pages 183–186 services, and to get free sample copies of our jour- Ron McKay nals. From genes to therapies: the role of animal models, Pages 187–193 Associated Professional Sleep Societies Alcino J. Silva et al 15th Annual Meeting 5-10 June 2001, Chicago, USA Tracking the decline in cerebral glucose metabolism in persons and lab- Booth number 821 oratory animals at genetic risk for Alzheimer’s disease, Pages 194–206 Eric M. Reiman et al XVII World Congress of Neurology 17–22 June 2001, London, UK Genes and attention-deficit hyperactivity disorder, Pages 207–216 Booth number 3, Earls Court 2 James Swanson et al XIV International Congress on Parkinson’s Disease 28–31 July 2001, Helsinki, Finland Williams syndrome: an exploration of neurocognitive and genetic Booth number B14 features, Pages 217–229 We look forward to seeing you! Ursula Bellugi et al

WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org ADVERTISEMENT 15

Models of temporal processing and language development, Pages Journal of the Neurological Sciences 230–237 Albert M. Galaburda et al Did you know that as a member of the WFN you are Erratum to Physiologic roles for the heme oxygenase products carbon entitled to a special membership subscription price to monoxide, bilirubin and iron: links to neuroprotection in stroke and JNS? The 2001 WFN membership price is only US$ Alzheimer’s disease [Clin. Neurosci. Res. 1 (2001) 45–52], Page 238 192 for 22 issues (Volumes 184–194)! David E. Barañano et al For more details on how to subscribe, please contact For more details on the Journal, go to: http://www.elsevier.com/locate/ Elsevier Science, Molenwerf 1, 1014 AG, Amsterdam, clires Netherlands; fax (+31) 20 485 3237, or e-mail For more details on the Association, go to: http://www.arnmd.org [email protected]

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WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org 16 BOOK REVIEWS & CALENDAR

(cont. from p. 13) ISBN: 0-7817-2385-X ment disorders and will help physicians No. of pages: 1280 CALENDAR to distinguish between them as well as Price: $115.00 * = Meeting endorsed by the Continuing Edu- providing a basic understanding of treat- Publication Date: 2000 cation Committee of the WFN ment options needed in active practice. Publisher: Lippincott Williams & Wilkins The first half of the book is devoted This is one of the most important books 2001 to the most common disease of Parkin- on neurological problems in children. The XIV International Congress on Parkinson’s sonism. The second half covers other sixth edition provides updated, compre- Disease movement disorders such as tremor, dys- hensive coverage of child neurology. It tonia, chorea, myoclonus, tics, gait dis- 28–31 July 2001 is absolutely true that a large number Finlandia Hall, Helsinki, Finland orders, the ataxias, conditions resulting of neurological problems are peculiar to Contact: CongCreator CC Ltd, PO Box in spasms, restless legs syndrome etc. childhood. These include metabolic, here- 762, FIN-00101 Helsinki, Finland In a nutshell this book provides compre- dodegenerative, chromosomal abnormal- Tel: +358 9 4542 190 hensive coverage of Parkinson’s disease ities, neuroembryology and storage dis- Fax: +358 9 4542 1930 and other movement disorders. It is most eases etc, and all these are discussed E-mail: [email protected] useful for family practitioners, neurolo- succinctly in this book of 16 chapters with URL: http:// gists, gerontologists and residents, and is contributions from 18 authors. Infections, www.congcreator.com/icpd-2001 an appropriate addition to Institutional li- trauma, tumours and cerebrovascular dis- 4th Congress of the European Paediatric braries. eases are also discussed. This book is Neurology Society Editor-in-Chief most useful not only for paediatric neurol- 13–16 September 2001 ogists but also for neurologists in many Baden-Baden, Germany Merritt’s Neurology countries of the world who have to handle Contact: AKM Congress Service GmbH, Obere Schanzstraße 18, D-79576 Weil neurological problems from infancy to old 10th Edition am Rhein, Germany age by themselves. An investment in this Tel: +49 7621 98330 Editor: Lewis P Rowland book by any institutional library will be re- Fax: +49 7621 78714 ISBN: 0-683-30474-7 warding. E-mail: [email protected] No. of pages: 1002 Editor-in-Chief European Charcot Foundation Symposi- Price: $89.00 um on The Progressive Phase of MS; its Publication date: 2000 Pathology and Treatment Publisher: Lippincott, Williams & Wilkins Neurology (including the 7th European Charcot Founda- This is the famous textbook of neurology 6th Edition tion Lecture by Prof. P. Soelberg Sørensen on dedicated to H. Houston Merritt. It con- The role of IVIG in the Treatment of Secondary tains contributions from 98 authors and all Editors: Howard L Weiner, Lawrence Progressive MS) of them have given masterly accounts of P Levitt, Alexander Rae- 18–21 October 2001 Venice, Italy their experiences in managing diseases of Grant ISBN: 0 683 30497 6 Contact: European Charcot Foundation, the nervous system. The book starts with No. of pages: 282 Heiweg 97, 6533 PA Nijmegen, the chapters related to symptoms of neuro- Netherlands logical disorders, how to select diagnostic Price: $24.95 Tel.: +31-24-3561954 tests etc, and then goes on to common Publication Date: 1999 Fax: +31-24-3540920 neurological problems such as infections Publisher: Lippincott, Williams & E-mail: [email protected] of the nervous system, vascular diseas- Wilkins es, etc. The last few chapters are related This volume is now in its sixth edition, to paroxysmal disorders, systemic diseas- having first appeared in 1973 which tes- Policy Statement es and General Medicine, environmental tifies to its popularity. It also indicates Although World Neurology is the only neurology, rehabilitation and ethical and the need by House Officers for a handy medium to go to every neurologist in legal guidelines. The majority of chapters portable guide to practical neurology. The the world (approximately 22,000 in are a source of comprehensive knowl- present edition has been brought up to 84 countries), it is not possible to pub- edge about diseases taking account of date but the “problem orientated” ap- licise every neurological meeting that technological advances in investigative proach has been maintained. Thus there takes place. The prime aim of the Cal- procedures. The authors deal with molec- are chapters on coma, vertigo-dizziness, endar is to notify those meetings that ular genetics and their impact on the di- dementia, and traumatic brain injury as are sponsored by the WFN (World, agnosis and management of patients. Ex- well as more usual chapters on stroke, Continental and Regional Congress- th tensive changes are in evidence in the 10 multiple sclerosis and epilepsy. This edi- es), its Research Groups and Corpo- edition of this one volume textbook of neu- tion has been updated with a section on rate Members of the Research Com- rology and it is an ideal book for medical scanning and I liked the remark when de- mittee. Notifications of meetings of students, practising neurologists, general scribing the technique of MRI “..pulsating National Societies are included only if physicians and institutional libraries. radio waves (heard as clicking sounds by there is significant international con- Editor-in-Chief the patient) and magnetic field (which the tribution. If space allows, others patient does not feel)”. This is a useful may advertise in the Calendar, in Child Neurology book for students and Junior doctors which case a charge of £125 is working in a well-equipped hospital in a made. Contact Mr. Keith Newton at Sixth Edition developed country. the WFN Secretariat, 12 Chandos Street, London W1G 9DR, UK. E-mail: Editor: John H Menkes and Harvey R Godwin-Austen [email protected] for further in- B Sarnat London, UK formation.

WORLD NEUROLOGY, Volume 16, Number 2, June 2001 Visit the WFN website at http://www.wfneurology.org