A Man of Art and Science

Vladimir Hachinski, famed stroke and dementia researcher, wins 2018 Killam Prize

By Crystal Mackay

Dr. Vladimir Hachinski believes medicine and art are unequivocally linked.

Schulich School of Medicine & Dentistry professor Vladimir Hachinski, past president of the World Federation of Neurology and a world-renowned stroke expert, has been named a 2018 Killam Prize winner for Health Sciences. Hachinski is only the sixth Western University researcher to be given this honor. Photo credit Frank Neufeld // Western News

“The science of medicine lies in the technology, the knowledge, the algorithms. But the art of medicine begins where the technology ends – and that’s where good doctors excel,” said the world-renowned expert in the relationship between stroke and dementia, who also happens to be a composer and poet.

Today, the coalescence of his passion for art and health care have come together. Hachinski has been named a recipient of the Killam Prize, the highest honor for research from the Canada Council of the Arts. He is the sixth Western University researcher to be given this honor, which recognizes the career achievements of eminent Canadian scholars and scientists actively engaged in research, whether in industry, government agencies, or universities.

Five prizes of $100,000 are awarded each year, one prize in each of the fields of humanities, social sciences, natural sciences, health sciences, and engineering.

“I am delighted by the honor. But I realize this is not just about me; it is about all the work we have done,” said Hachinski, a Distinguished University Professor and Professor of Neurology at the Schulich School of Medicine & Dentistry, Western University, London, Ontario, Canada. “I am left with a tremendous feeling of gratitude – gratitude for the acknowledgement, but even more importantly, gratitude because it gives an opportunity to highlight what we are doing, and where we are trying to go, which is the most exciting thing.”

When he began his career, it was the commonly held belief in the medical profession that dementia was caused by hardening of the arteries which reduced blood flow to the brain. Early on, he discovered the majority of dementias were actually caused by multiple, small strokes. Hachinski coined the term “multiple-infarct dementia” in order to classify this condition.

“In that way, medicine is like poetry; I needed to boil it down to a simple term by using language to get to the essence of something.”

His love for both the arts and medicine began when he was a boy. His family fled the Soviet Union and emigrated to Venezuela when he was 7 years old. Hachinski would watch birds and animals out of his window. Inherently curious, he would sketch and draw them, wanting to understand how they moved and how their bodies worked.

In Venezuela, there was an expectation in high school that a student had to choose to study either arts and humanities or science – not both. Hachinski said it was a difficult and defining moment for him.

“I really was torn between medicine and history. How do you choose between two passions?”

The decision was a practical one.

“If I became a historian, I couldn’t really be an amateur doctor, whereas if I became a doctor, I could be an amateur historian,” said the man who even today often seeks guidance from poets.

Since earning his medical degree from the University of Toronto after his family emigrated to Canada, Hachinski has transformed the understanding, diagnosis, treatment, and prevention of the two greatest threats to the brain: stroke and dementia.

Early in his neurology residency, Hachinski became intrigued by the interaction of the brain, its blood supply, and the heart. He developed the Hachinski Ischaemic Scale, which is considered to be the most widely used clinical method to identify the difference between degenerative dementia (like Alzheimer’s disease) and multi-infarct dementia (cognitive impairment caused by multiple strokes).

Throughout his career, he has authored more than 800 well-cited research papers and has authored, coauthored, and edited 17 books. He founded, with John W. Norris, the world’s first successful acute stroke unit, and went on to establish the first stroke clinic in London, Ontario. That has led to stroke units being established around the world and has laid the groundwork for what is now considered the standard of care.

He received the Order of Ontario and Order of Canada. He was the president of the World Federation of Neurology from 2010 to 2013 and was inducted in the Canadian Medical Hall of Fame in 2018.

With all of those accomplishments, Hachinski said there have also been setbacks, and part of what has led to his successes has been the ability to persevere.

“If you are in research, you better be prepared for setbacks. I don’t think failing makes you a failure; failing to learn from them is a failure,” he said. “There is a continuous alternation between disappointment and triumph, and this is an important lesson.”

However, Hachinski believes his most important contribution to human health isn’t in what he has done in the past, it is in what’s coming next.

He and his team are keen on using experimental, clinical, and population health data together to implement strategies to prevent stoke and dementia together. In 2006, he and a group of colleagues helped establish World Stroke Day, which has contributed to increasing awareness of the prevention of risk factors for stroke.

They have already shown a new stroke initiative in the province of Ontario correlated with a 30 percent reduction in the incidence of stroke, and in turn, a 7 percent reduction in the incidence of dementia.

“This was the first time that we were able to show if you prevent one, you can prevent the other,” Hachinski said. “The end goal is to prevent, delay, or mitigate disease, and I think that we already have. Right now, we could prevent 90 percent of strokes if we did everything perfectly. And if we did everything perfectly for dementia, we could prevent 30 percent.”

He and his collaborators are working to persuade decision-makers to enact approaches that look for ways to prevent the two as part of the same strategy. He said it all comes back to looking at stroke and dementia, not in isolation but as part of the same.

“By doing this, we may able to change things for the better in a relatively short time,” he said.

With this announcement, Hachinski becomes Western’s sixth Killam Prize winner and the most recent winner since English professor David Bentley won in 2015. Other Western winners include Economics professor John Whalley (2012), Engineering professors Alan Davenport (1993) and Maurice Bergiougnou (1999) and Robarts Research Institute co-founder Henry Barnett (1988).

This story is reprinted with permission from Western News, a Western University publication.

The Value of Shared Commitment and Contribution

As we approach the halfway mark of the current calendar year and as many watched the World Cup, perhaps the greatest display of a world team game, it is worth reflecting on the value of teamwork. Teamwork is at the center of all organizations engaged in goal-oriented endeavors, sporting or otherwise. It is especially evident in those organizations that are successful and in those within the not-for-profit area.

William Carroll, MD
 

Teamwork is essential to energize existing activities and drive new ones: At its heart is the knowledge that all team members share the same commitment and all will make the best contribution they can. Teamwork has a role in the WFN, and I would like to review this fundamental, yet largely intangible, essence in relation to our organization this year.

A solid, stable organizational base and a sound financial state are important but the most critical is the sense of teamwork, of a shared commitment, of reliable interdependence and of equality of worth through the contribution of each member society to the advancement of the WFN.

Let me illustrate by touching on some contributions. I preface this by saying how heartening it is to see these contributions grow.

Regional Training Centers in Africa. These were initiated first in Africa, though the WFN has had a program to evaluate neurology departments and institutions for a much longer period when they were commenced by Ted Munsat in Central America. Now in Africa, we have four centers either actively training young neurologists for Africa or about to. But the pleasing aspect about contributions are those offered by the Association of British Neurologists , the Societe Francaise de Neurologie (SFN), the Società Italiana di Neurologia, and the large regional organizations of the European (EAN) and American (AAN) Academies of Neurology. All have made offers of substantial contributions to the funding of the institutions and trainees.

Furthermore, the WFN has been informed that the SFN is preparing an offer for the WFN to fund another center in francophone Africa, in Abidjan, Cote d’Ivoire. This would bring the total to five such centers which will all receive funding through the WFN from the contributions from the societies I have mentioned.

Regional Organizations. There are six regional organizations in the WFN, which roughly matches those of the WHO. They range from the highly organized and well-financed AAN and EAN through to the African Academy of Neurology (AFAN), the last to be established in 2015. Now through the efforts of Neurological Association of South Africa (NASA) led by Lawrence Tucker, AFAN has its own bank account in South Africa enabling it to operate financially. However, because of the difficulties facing the African region, funding by pharma to support a biennial regular meeting is nigh on impossible at present. Such a lack of an income stream could jeopardize the future of AFAN.

Through the thoughtful teamwork of WFN member societies and individuals, promising discussions continue that should enable AFAN to maintain its development for the good of African neurology. The meeting planned for 2019 in East London, South Africa, will be held in conjunction with NASA, and thereafter additional collaboration is being planned.

World Congress of Neurology. As we all know, the 2019 WCN will beheld in Dubai and hosted by the Emirates Neurology Society (EMINS). We also know how important the WCN is to the WFN. It forms a major component of the WFN income along with the royalties from our publication, the Journal of Neurological Sciences (JNSci) and the annual subscriptions or dues from member societies. Any competition to the WCN from regional meetings has the potential to threaten the profitability of the WFN and consequently its educational programs and activities.

So it was with much pleasure that the WFN received the news that the Pan Arab Union of Neurological Societies (PAUNS) and its affiliated regional organizations have moved or cancelled all meetings in the region with the potential to compete with the 2019 WCN. While such a sacrifice by a regional organization has occurred previously it is still most heartening to see it in operation once more. Again, this reveals the actions of a team at work.

Annual Subscriptions. I have already mentioned the importance of the WFN income above. In this same sense of shared commitment and contribution, I encourage all member societies to view their annual subscriptions (known as dues) as being part of the team. While the amounts vary according to the numerical size of the member society, the very act of contributing establishes a sense of equality which in turn builds the morale of an organization. Furthermore, without funds the WFN cannot function.

On the Move. Within the London Office, there is the same sense of teamwork. As the office settles into its new operating calendar and record system (Asana), it can now look more externally. The WFN is most appreciative of the efforts of Chiu Keung Man for his direction on this and to the other office staff in general for their cheerfulness in continuing to keep everything functioning while the transition occurs. One of the more tangible features of an organization is its visibility. Logos, trademarks, and livery are the modern-day equivalents of flags and colors. They evoke a sense of pride and familial cohesion and represent the combined efforts and contribution of the organization’s many parts.

The London office is carefully reviewing all of the WFN logos and symbols on all platforms and media with which the WFN is seen so that it is immediately apparent to all viewers that an item, an article, a meeting, or a post on social media is from the WFN. Getting this right is fundamental to our visibility in the competitive world in which we exist and in signaling to our team that we do care how we are represented.

Your contribution to the WFN at the individual, member society, regional organization or WFN committee is crucial. Without it and the sense of equality of contribution, we will struggle to succeed.

Global Matters. I have mentioned in passing in my first column this year and in my statement of candidature in World Neurology last year of the desire for the WFN to develop a comprehensive registry of the needs of all member societies. This will become the Needs Registry. It will require the cooperative involvement of each of the regional organizations and their member societies in its preparation. In turn, it will provide an active survey of what is required to improve the delivery of neurological care. This will be the converse or opposite of what the WFN/WHO Atlas contains, which is the listing of resources available in each country and was obtained from a non-uniform range of sources and as such is less meaningful. Furthermore, the EAN is re-evaluating its survey of national neurological resources, the AAN plans a disparity file on its resources within the U.S., and the WHO announced at the 71st World Health Assembly in Geneva in May that it recognizes the inequality of access to health care and is preparing a more detailed and accurate inventory.

Together, if they all come to fruition, we will have a better overview of the needs, the basis for models of remedial action, and a potentially powerful tool with which to advocate for change at governmental and global levels.

At a larger scale of cooperative action is the informal support for a Global Neurology Alliance. During June’s EAN in Lisbon, there was encouraging support for the amalgamation of the World Brain Alliance and the Global Neurology Network, recognition of more regular communications to all through the WFN, and a suggestion of a more sustained and professional promotion of brain and neurological health. Clearly much needs to be done here but the sentiment of the need for teamwork at this level appears to be in line with that expressed by all neurological organizations.

Important Upcoming Events

World Brain Day (WBD)

Mohammed Wasay and his World Brain Day (WBD) team have again prepared numerous activities for individual member societies to join with to celebrate the formation of the WFN on July 22. This year, the theme is Clean Air for Brain Health (and healthy brain development).

WFN Annual General Meeting

The WFN Annual General Meeting will be Oct. 9 in Berlin during ECTRIMS. Not only must the WFN satisfy the requirements of the U.K. Charity Commission and ratify its financial position, there are also important elections.

Wolfgang Grisold’s four-year term as secretary general ends on Dec. 31. By being nominated by the Mexican, Moldavian, Moroccan, Romanian, and Italian societies, he offers himself for a further term.

The three-year term of Morris Freedman, elected trustee, is at an end, and he has offered himself again as a candidate. Three other candidates have been approved by the Nominating Committee and its outgoing chair, Marianne de Visser. All of the candidate statements are in this edition and can also be found on the WFN website. •

Bill Carroll
President, World Federation of Neurology

Victor Soriano

The Soriano Lectures at the World Congresses of Neurology were named for his contributions and lasting presence

Victor Soriano was born on Feb. 8, 1909, in the Isle of Rhodes. At the time, the Isle of Rhodes was under the possession of Turkey, and soon after of Italy. Victor was the second of five siblings. His parents were Félix Soriano and Catalina Junio, both Sephardic Jews. They emigrated to Uruguay when Victor was 9 months old. This explains why Victor considered himself Turkish, Italian, and Uruguayan.

Victor Soriano

Victor Soriano

Once in Uruguay, the Soriano family settled in its capital city, Montevideo, in the historical district, where Victor chose to live the rest of his life. His father, an efficient and respected tailor, soon became popular among the Jewish community. His income quickly increased, and he gained great respect, especially after founding the first Sephardic synagogue in the city.

Young Victor was a hard-working student who in 1925 was admitted to the school of medicine, from which he graduated in 1934. During his period as a medical student, he paid special attention to Prof. Americo Ricaldoni’s teachings of clinical neurology at the Hospital Maciel. Prof. Ricaldoni was the first academic neurologist in Uruguay. Due to his efforts, the Instituto de Neurología of Montevideo was founded in 1926, having Prof. Ricaldoni as its first director.

After graduating, Victor Soriano was appointed neurologist of the Instituto de Neurología in 1935. This was a challenging time for the institute, because of the absence of a leader after Prof. Ricaldoni’s death in 1928, scarcely one year after the inauguration of the new institute.

Two years later, the authorities finally appointed Prof. Alejandro Schroeder as Ricaldoni’s successor in 1937. Prof. Schroeder started a period of reconstruction with the help of the few remaining neurologists, Victor being one of them. In 1939, Victor was promoted to assistant neurologist. Simultaneously, he was invited to teach neurology at different medical clinics in the Hospital Maciel and the Orthopedical and Traumatological Institute. In 1943, he was appointed associate professor of medicine.

In 1939, Vlctor married Clara Benzecry (Clarita, as she was known). She was his wife and his efficient secretary as well and permanently encouraged him in all his activities, both social and medical. After Victor founded the Uruguayan Committee of Friends of the Weizmann Institute of Israel, it was Clarita who organized its meetings, which were held at the Sorianos’ home. Every Tuesday evening, she hosted scientific, literary, and musical events.

In 1945, Victor traveled to the United States as a Rockefeller fellow, staying at Yale University with John Farquhar Fulton, Sterling professor of physiology, who appointed him instructor of physiology. Fulton inspired in Victor a deep and lasting admiration. In 1947, as visiting investigator of the Rockefeller Institute for Medical Research, he worked with Prof. Hiram Houston Merritt at the Montefiore Hospital of Columbia University. Since 1948, he attended with Clarita all of the meetings of the American Neurological Association (ANA). In 1950, he was named delegate for Uruguay to the Iberoamerican College of Neurologists. Victor and Clarita were regular attendees to the International Congresses of Neurology from the initial preparatory meeting in Lisbon in 1953 and the first official congress held in Brussels in 1957.

At the congress held in Rome in 1961, Earl Walker of Baltimore, Giuseppe Moruzzi of Pisa, and Victor of Montevideo paid tribute to the memory of John F. Fulton, who had died the previous year. At that meeting, Fulton’s friends and collaborators decided to organize the Fulton Society, and Victor was elected as permanent president. This society commended its president to organize a special symposium, every two years, simultaneously with the World Congress of Neurology and the ANA meeting, where selected leaders of neuroscience should give lectures on advances in nervous system research. The first of these symposia was held in Atlantic City in 1965 and continued for many decades, sponsored by the World Federation of Neurology (WFN) and the ANA and organized by Victor and Clarita.

In 1987, Victor and Clarita decided to sponsor a lectureship to be given at the ANA, after 40 years of consecutive attendance to its meetings. The lecture must be given by a member of the ANA. When asked in 1989 why they chose to sponsor a lectureship, the Sorianos answered: “When we first went to the United States in 1945, to stay with Prof. John F. Fulton and afterward with Prof. H. Houston Merritt, we enjoyed the most cordial and warm welcome everywhere, establishing lasting bonds of friendship with outstanding promising young doctors, who are now senior members of the American Neurological Association and prestigious professors. Thus, when I became a member of the American Neurological Association several decades ago, I returned since 1948 every year with the double reward of enjoying a very outstanding scientific level and seeing dear old friends. Little by little, we have regarded the ANA as a big family, to which we are united by warm affection. Sponsoring a lectureship to be given at the ANA Annual Meeting, we like to think that in future years the Sorianos will be linked to all of you, through a brilliant lecture delivered by an outstanding scientist.”

The WFN also has two related endowed lectures in every world congress of neurology. One is the Victor and Clara Soriano lecture and the second is the Fulton Symposium/Soriano lecture. At the most recent World Congress of Neurology in Kyoto in 2017, these lectures included “Grid Cells and the Medial-entorhinal Space Network” by Prof. E.I. Moser (Norway), “Defining the Future of Neurology – Japan, Asia and Oceania” by Prof. H. Mizusawa (Japan), and “Global Neurology Challenges and Way Forward” by Prof. and WFN President Raad Shakir (United Kingdom).

Victor was the author of more than 200 scientific publications in different medical journals, covering different aspects of medicine. In neurology, he preferred epilepsies, spinal cord compression, and peripheral nerve pathology. He gave lectures in several countries in America, Europe, and Asia. Many neurological societies accepted him as honorary member.

Apart from his chief interest in experimental and clinical neurology, he had many hobbies. Boxing and football were a part of his youth. He also engaged in sports journalism. In the attic of his home, he mounted an astronomic observatory provided with a powerful telescope, and he sought relaxation by watching the sky. On occasion, he invited schoolteachers and their pupils to watch the moon and the planets. During his last four decades, he devoted much of his time to medical journalism, publishing in the most important newspapers of Uruguay weekly columns on different subjects: sanitary education, innovations in medicine, historical aspects of medical practice, with the intention of popularizing and simplifying medical knowledge. He loved all kinds of music, instrumental and lyrical, but he was also an admirer of the typical music of Uruguay and Argentina and its poetry, being able to sing from beginning to end the most popular tangos.

Victor died in May 2005 and Clarita a few years later. In 2012, Soriano’s brother-in-law agreed with the National Academy of Medicine of Uruguay to sponsor the Victor and Clara Soriano Award for international research in medicine, endowed with (U.S.$)10,000. •

Calendar of Events

June 16-19
Fourth Congress of the European Academy of Neurology (EAN 2018)
Lisbon, Portugal
https://www.ean.org/lisbon2018

July 6-7
19th European Federation of Autonomic Societies Meeting (#EFAS18)
Vienna, Austria
http://efasweb.com/

July 6-10
15th International Congress on Neuromuscular Diseases (ICNMD 2018)
Vienna, Austria
http://www.icnmd2018.org/

Editor’s Update

For many years, tardive dyskinesia was a poorly understood clinical syndrome with no effective therapy. Thanks to new research, we now have a greater understanding of the pathophysiology of tardive dyskinesia and new therapies for the condition.

John D. England, MD

John D. England, MD

A summary of this new and important information about tardive dyskinesia is now available in the Journal of the Neurological Sciences. I am pleased to announce that a special issue on Tardive Dyskinesia, edited by Drs. Daniel Truong and Robert Hauser, has been completed as volume 389 (June 15, 2018) and is available on the website for the Journal of the Neurological Sciences.

Importantly, the special issue will be “free access” via the online journal site through the year 2018. Elsevier has added a special link via the online journal site called “SI Vol 389, 2018” under the tab “Free Articles” to enhance accessibility.

The special issue is also available on ScienceDirect. I encourage all of you to access this issue, which is currently the most up-to-date monograph on tardive dyskinesia. Its publication is especially timely since there are new medications and other new treatments for the condition.

I would like to take this opportunity to thank Drs. Truong and Hauser and all of the invited authors of this special issue for their outstanding contribution to the Journal of the Neurological Sciences and the medical and scientific community. Also, I would like to acknowledge Peter Bakker, our executive publisher at Elsevier, for helping us arrange free online access of this series of articles for the remainder of the year. •

Stanley Prusiner on the Origin of the Term Prion

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

In an oral history interview for the American Academy of Neurology conducted April 27, 2017, at the Boston Convention Center, I spoke with Nobel laureate Stanley B. Prusiner, the only living neurologist to have won a Nobel Prize (Lanska and Klaffke, 2017, Lanska, 2017).

Prusiner was the sole recipient of the Nobel Prize in Physiology or Medicine in 1997 “for his discovery of Prions — a new biological principle of infection.” Here is an excerpt from that interview concerning how Prusiner created the term prion, which he introduced in a landmark paper in Science in 1982 (Prusiner 1982).

Prusiner: [In the fall of 1980,] I was with a friend of mine who was a professor at Harvard. This was [American chemist] Frank Westheimer, PhD (1912-2007). [He was receiving] an honorary professorship in the pharmacy school. He came to see me and [we] talked. I went over everything I was doing. He said, “Stan, this is really fantastic. You’ve discovered something really new, and you need to give it a name, and you need to give it a good name. You need to think about this name for a long time. A lot of work needs to go into this. Because, if you give it a crappy name, someone else will come along and give it another name, and they will end up with the lion’s share of the credit, but you will have done the work, and that’s not a good idea. This is what you’ve done with your life, and you need to make sure that you don’t screw it up. So you need to spend a lot of time on this.”

Stanley Prusiner

Stanley Prusiner

Lanska: Could you to talk about how you came up with the term?

Prusiner: OK. I’m happy to recite this… [I]t’s the spring of 1981, and I need to finish this paper [Prusiner 1982]. The only thing holding it up is the word, and I’m trying to figure out a word.
So I go through Latin dictionaries, because I knew — I still know — a lot of Latin. I’m not a scholar in it; that’s for sure. I don’t know any Greek. I don’t really know how to come up with a word. I want a word like exon. I thought that’s a great word. Where do I find somebody who can do that? I kept thinking of somebody at Berkeley [who] can help me, but then I didn’t even know who to talk to. I thought this is going to be just frivolous to go to Berkeley and try to find some professor of words who will help me.
Then, I said, “OK, I’ve got to come up with some rational approach to a word, just taking a bunch of letters, and where are these letters going to come from? Well, they’re going to come from words that have something to do … [with the responsible agent].” So I wrote out [the words] protein, infectious, and agent. I started with that. I got piaf out of that, because I wanted protein, and I wanted infectious, and then I wanted agent.

Quark: What’s in a Name?

Prusiner is certainly not alone in devoting considerable energy to devising a catchy word for a newly described entity in science or medicine, and indeed he developed exemplars based on prior scientific neologisms. Later, in the public interview, Prusiner recalled:

My model words [for prion] were virus and quark [pronounced correctly as qwork]. I thought Murray Gell-Mann was terrific with that, stealing that from Lewis Carroll [sic]. So I looked through Alice in Wonderland for another one, but I didn’t find one. (Lanska, 2017)

American physicist Murray Gell-Mann (1929-) received the 1969 Nobel Prize in physics “for his contributions and discoveries concerning the classification of elementary particles and their interactions.”

Gell-Mann coined the term quark (which he pronounced kwork) in 1963 to refer to the fundamental constituents of the nucleon (i.e., either a proton or a neutron, considered in its role as a component of an atomic nucleus) (Gell-Mann, 1964, 1995). Despite Prusiner’s recollection to the contrary, the term quark did not originate in the fantasy novel Alice’s Adventures in Wonderland (1865), written by English mathematician Charles Lutwidge Dodgson (1832-1898) under the pseudonym Lewis Carroll. Instead, Gell-Mann derived the non-phonetic spelling from a whimsical poem in Finnegans Wake (1939) by Irish writer James Joyce (1882-1941).

According to Gell-Man’s account (1995): In 1963, when I assigned the name “quark” to the fundamental constituents of the nucleon, I had the sound first, without the spelling, which could have been “kwork.” Then, in one of my occasional perusals of Finnegans Wake, I came across the word “quark” in the phrase, “Three quarks for Muster Mark.” Since “quark” (meaning, for one thing, the cry of the gull) was clearly intended to rhyme with “Mark,” as well as “bark” and other such words, I had to find an excuse to pronounce it as “kwork.” But the book represents the dream of a publican named Humphrey Chimpden Earwicker. Words in the text are typically drawn from several sources at once, like the “portmanteau” words in Through the Looking-Glass [and What Alice Found There (1871), the novel by Lewis Carroll]. From time to time, phrases occur in the book that are partially determined by calls for drinks at the bar. I argued, therefore, that perhaps one of the multiple sources of the cry, “Three quarks for Muster Mark” might be “Three quarts for Mister Mark,” in which case the pronunciation “kwork” would not be totally unjustified. In any case, the number three fitted perfectly the way quarks occur in nature. (Gell-Man, 1995, p. 180)

Joyce’s line struck Gell-Mann as particularly appropriate, because the hypothetical elementary particles combined in groups of three to form baryons, such as protons and neutrons.

Gell-Mann adopted Joyce’s spelling for his “quork,” even though Joyce clearly intended quark to rhyme with Mark.

An equivalent model to Gell-Mann’s quark model was independently proposed by Russian-American physicist George Zweig (1937-) in 1964 (Zweig 1964a,b), the same year as Gell-Mann’s model, but Zweig did not propose a similarly catchy term and ultimately Zweig did not share the 1969 Nobel Prize in Physics.
Both Prusiner and Gell-Mann described or proposed new entities that warranted new scientific nomenclature. Both saw the new names as important factors in establishing and cementing their ideas in the “scientific marketplace” (anonymous 2012), and consequently both devoted considerable time and energy to the effort of inventing a term that was short, catchy, and in some way clearly novel, but that still could be justified in a plausible way (Gell-Mann 1995; Prusiner, 2014; Lanska and Klaffke 2017; Lanska 2017). To emphasize the novelty of their terms, both chose idiosyncratic non-phonetic pronunciations and repeatedly emphasized the “correct” pronunciation in subsequent discourse.

If Prusiner’s and Gell-Mann’s later experiences are any guide, Westheimer’s advice to Prusiner was certainly prescient: The names of newly described scientific entities do matter in establishing and maintaining scientific turf. Although Lewis Carroll’s Alice character was not the source of either neologism, she might still have had something to say about this if she had been consulted: “Curiouser and curiouser!” •

Lanska: You just kind of threw on an extra “f” for fun?

Prusiner: For infectious.

Lanska: Protein, infectious, agent…p-i-a. You added an “f.”

Prusiner: So you just underline the “f” in infectious, right? That’s where the letters all come from.
I always liked [French cabaret singer and songwriter] Édith Piaf [1915-1963; nee Édith Giovanna Gassion; she adopted her stage name, Piaf, from her nickname, which is French slang for sparrow]. Now, “sparrow” is announcing all of this, right? [Prusiner laughs.]
I then sent it to a friend of mine named Sidney Udenfriend (1918-2001), an American biochemist and pharmacologist who was running the Roche Institute in Molecular Biology. This was a fabulous place that was created by Roche to do basic science that would create drugs. Eventually, they shut it all down, because they never got one drug out of spending hundreds of millions of dollars over 20 years. He reads the paper, and he says, “Stan, this is an American discovery, not a French discovery. You don’t need a French word. You need another word. Go find another word.” So that was the end of piaf.
Short. You need something short. You need to have two vowels. Great words are words like virus. That’s a fabulous word. And quark [pronounced kwork] is a great word. Those are words that I think are just A+.
So I then throw out agent, because I don’t need agent. That’s totally non-specific, and I’m left with protein and infectious. Whenever I would go to a lecture, I would write out infectious across the top and protein on the side, or vice versa. Then, I’d just pick letters randomly. I didn’t get anywhere until one day. I’d probably stumbled across the same word 10 times and never picking it out: p-r-i-o-n. I read it, and I say, “This is prion.” I could have pronounced it pry-on, but I pronounced it pree-on. Then, I said, “I’m going to write p-r-e-e-o-n as the pronunciation in parentheses.”
I leave this defunct faculty club that was now a sandwich shop across the street, and I walk upstairs. I look in my Webster’s Unabridged Dictionary, because there are no computers. I find a bird with a sawtooth beak.

Lanska: A whalebird.

Prusiner: Right. And I said, “Well, you know, this really doesn’t matter. Lots of words have more than one meaning. There’ll be two pronunciations. If I’m right, this will be the No.1 definition, and the bird will continue to live on in oblivion, because no one is interested in prions [pry-ons]. I’ve never heard of a prion [pry-on]. It’s a petrel that lives in the south, the Southern Ocean [Antarctic Ocean].”
It’s not worth worrying about the bird. I said, “There’ll be a little crap from some of my competitors who will say, ‘That’s a bird.’” But I said, “It doesn’t matter.” So that’s where it came from. It had ion, so it looked like it was something highly basic. An ion really gets right down to the essence. And it was short. It had two syllables. It was going to be OK.

Lanska: You wrote in your book, though, that one of the reviewers of your paper objected, that the name had unfortunate echoes of the author’s name, Prusiner ions (Prusiner 2014, pp. 90-91).

Prusiner: Yes. That was pretty clever, [but] there was so much vitriol in the rest of the review.

Lanska: Now, of course, proteinaceous infectious gets you proin rather than prion…

Prusiner: Right, which is like loin.

Lanska: Which has kind of a funny sound to it, of course, no matter how you pronounce it. So you made another, little flexible change in that, just to give it a more catchy flavor, I think. Is that fair?

Prusiner: Yes. Proin is not a good word.

Lanska: But proteinaceous infectious as an acronym would get you that, right?

Prusiner: Yes.

Lanska: A lot of people didn’t like the term.

Prusiner: Yes.

Lanska: There was a lot of backlash. Why do you think there was so much animosity to that term?

Prusiner: If you read what other people have said, they didn’t like it, because essentially I took over the field by putting my imprimatur on the field prematurely, because I didn’t have enough information to come up with a word. That really is what I should have done was call it a Gibbs or a Gajdusek, [after NIH researchers C. Joseph Gibbs, PhD (1924-2001) and D. Carleton Gajdusek, MD (1923-2008), who first demonstrated the experimental transmission of kuru and Creutzfeldt-Jakob disease to chimpanzees in the period from 1966-1968 (Gajdusek et al, 1966, 1968; Gibbs et al 1967)] or a Gajdusek and a Gibbs, or a Gibbs and a Gajdusek, or something like that, or a Dickinson [after British veterinary geneticist and scrapie researcher Alan Dickinson, PhD (1930-2017)]. Of course, this was nonsense from my point of view.
I did exactly what Frank Westheimer told me to do. The result was exactly what Westheimer wanted to have happen, which was to get a good word and not have somebody else take over. They had a lot of time. I was the new guy on the block. But they never could figure out anything about what was truly going on. They didn’t have a way of doing something, or they weren’t clever enough, or they weren’t sufficient showmen to figure out where all of this was going to go. But Westheimer saw it and called it completely.

Lanska: I think they were stuck in one little framework.

Prusiner: Yes.

Lanska: And they struggled to keep fiddling with “unconventional virus” [or] “slow virus.”

Prusiner: They had their own words, that’s true, but they were really dumb. “Unconventional virus” is as dumb as it gets.
There’s that great quote that’s in [my memoir] from [scrapie researcher] Iain Pattison [of the British Agricultural Research Council’s Compton Research Institute], in which he says:

“The fourth decade of my association with scrapie ended in 1978, with the causal agent still obscure, and virologists as adamant as ever that theirs was the only worthwhile point of view. To explain findings that did not fit with a virus hypothesis, they rechristened the causal agent an ‘unconventional virus.’ Use of this ingenious cover-up made ‘virus’ meaningless — for is not a cottage an unconventional castle?” (Pattison, 1988) •

References:

  • Anonymous. The scientific marketplace. Nature Materials 2012;11:259.
  • Anonymous. What Does ‘Quark’ Have to Do with Finnegans Wake? How a word used by James Joyce became the name for an elementary particle of matter. Merriam-Webster, 2017. https://www.merriam-webster.com/words-at-play/quark [Accessed 12-12-17].
  • Gajdusek DC, Gibbs CJ, Alpers M. Experimental transmission of a Kuru-like syndrome to chimpanzees. Nature. 1966;209(5025):794-796.
  • Gajdusek DC, Gibbs CJ Jr, Alpers M. Transmission and passage of experimental “kuru” to chimpanzees. Science. 1967;155(3759):212-214.
  • Gell-Mann M. A schematic model of baryons and mesons. Physics Letters. 1964;8(3):214-215.
  • Gell-Mann M. The Quark and the Jaguar: Adventures in the Simple and the Complex. New York: Henry Holt and Co., 1995:180
  • Gibbs CJ Jr, Gajdusek DC, Asher DM, Alpers MP, Beck E, Daniel PM, Matthews WB. Creutzfeldt-Jakob disease (spongiform encephalopathy): transmission to the chimpanzee. Science. 1968;161(3839):388-389.
  • Lanska DJ, Klaffke L. Stanley Prusiner, MD, FAAN. American Academy of Neurology Oral History Archive Private Interview. 69th Annual meeting of the American Academy of Neurology, Boston, Massachusetts; April 27, 2017.
  • Lanska DJ. Stanley Prusiner, MD, FAAN. American Academy of Neurology Oral History Archive Public Interview. 69th Annual meeting of the American Academy of Neurology, Boston, Massachusetts; April 27, 2017.
  • Pattison IH. Fifty years with scrapie: a personal reminiscence. Vet Rec 1988;123:661-666.
  • Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science 1982;216:136-144.
  • Prusiner SB. Madness and Memory: The Discovery of Prions—A New Biological Principle of Disease. Yale University Press, 2014.
  • Zweig G. An SU(3) model for strong interaction symmetry and its breaking. (CERN Report No.8182/TH.401. 1964a.
  • Zweig G. An SU(3) model for strong interaction symmetry and its breaking: II. CERN Report No.8419/TH.412. 1964b.

    • Douglas S. Lanska is associate chief of staff for education at the VA Medical Center, in Tomah, Wisconsin. He is also professor of neurology at the University of Wisconsin School of Medicine and Public Health in Madison, Wisconsin, and professor of psychiatry at the Medical College of Wisconsin in Milwaukee, Wisconsion and the chair of the History and Archives Committee of the American Academy of Neurology.

From the Editors

By Steven L. Lewis, MD, Editor, and Walter Struhal, MD, Co-Editor

Walter Struhal, MD

Steven L. Lewis

We are pleased to welcome you to the March-April 2018 issue of World Neurology, the official publication of the World Federation of Neurology (WFN). In this issue, a number of WFN initiatives are discussed, beginning with the Presidents’ Column where WFN President William Carroll discusses the new steps taken by the new administration of the WFN as outlined at the recent strategy meeting that took place in London.

In this issue, we are also pleased to announce the seventh year that the WFN is offering educational grants to sponsor high impact educational and outcome-based research projects. Interested young investigators should waste no time as the deadline for applications is quickly approaching. Another important announcement in this issue is the invitation for bids from national member societies in the Americas to host the World Congress of Neurology (WCN) in 2023.
The theme for this year’s World Brain Day (July 22, 2018) campaign is also announced in this issue and all neurological societies are encouraged to participate.

John D. England, the editor-in-chief of the Journal of the Neurological Sciences, the official journal of the WFN, announces a special issue devoted to tardive dyskinesia, edited by Dr. Daniel Truong and Dr. Robert Hauser. It is available free online for all readers through 2018.

Dr. Udaya K. Ranawaka reports on the 11th Annual Academic Sessions of the Association of Sri Lankan Neurologists that was held last November. Professor Jan Kuks announces the next examination of the European Board of Neurology that will be taking place on June 15, 2018, at the European Academy of Neurology Congress in Lisbon. Martin Kaddumukasa, MD, reports on his eye-opening visit to St. Josef Hospital in Cochum (University Clinic of Ruhr University) in the department visit program sponsored by the German Neurological Society and the WFN.

In this issue’s history column, Douglas Lanska reports on his interview with Nobel laureate Stanley Prusiner on the origin of the term prion, an article that should be of both historical and medical interest to all readers. Eduardo Wilson provides a biography of Victor Soriano, who is honored by the Soriano lectures held every two years at the World Congress of Neurology. Finally, Nadir Bharucha provides a touching obituary of Professor Eddie Phiroz Bharucha, a pioneer of neurology in India, who passed away in December and whose name will also live on in an endowed lectureship at the World Congresses of Neurology.

We hope you enjoy this issue, and we invite articles from neurologists around the globe to keep us all up to date about news of interest to all neurologists. •

Eddie Phiroz Bharucha

Pioneer of Neurology in India (Dec. 27, 1916-Dec. 14, 2017)

Eddie Phiroz Bharucha, though born into privilege, was in many ways a self-made man. His mother, Bachha came from a prominent business family in Karachi. His father, Phiroz C. Bharucha (1882-1952), was an eminent physician in Bombay (now Mumbai) who was consulted by M.K. Gandhi and Mohammed Ali Jinnah.

P.C. Bharucha was part of the group of highly qualified Indian physicians who vigorously pushed their claim for academic positions in Grant Medical College on par with the British members of the Indian Medical Service. Their efforts for justice resulted in the formation of a medical college, which facilitated academic advancement of Indian doctors as a matter of policy.

Eddie Phiroz Bharucha

Eddie Phiroz Bharucha

After Eddie’s mother died when he was 2, his bereaved father immersed himself in his work and left Eddie to be brought up largely by an English governess, with occasional inputs from his mother’s family.

Eddie completed his MBBS and MD in both Bombay and London. He was appointed Honorary Physician in Medicine at the King Edward Memorial and Seth G S Medical College (KEM) Hospital in 1945.

Between 1949 and 1952, he trained in neurology at the Hospital for Nervous Diseases, Queen Square and the Maida Vale Hospital for Nervous Diseases in London. He then proceeded to the United States where he worked for three months each under Dr. Houston Merritt at Columbia University and under Dr. Denny Brown at Boston City Hospital. He returned to India in 1952 and became the first neurophysician to establish a department of neurology in India, with 12 beds at the KEM Hospital in January 1953.

By the time he retired, as emeritus professor in 1974, the KEM Hospital had all of the core elements of the clinical neurosciences, including large epilepsy and neurology outpatient clinics which drew and still draws a large number of patients from all over the country, as well as neuropathology, electroencephalography and electromyography. The department of neurology had now established a reputation nationally and internationally for excellence in patient care, teaching, and academic endeavors. Dr. Bharucha later joined the Bombay Hospital, a large private hospital which also has a strong sense of social responsibility, and which subsequently became a teaching hospital.

Together with his wife, the late Dr. Piloo Bharucha, he promoted and practiced multidisciplinary care in pediatric neurology long before this became entrenched in more developed economies. From the mid 1950s onward for many years, the couple ran clinics for neurological and pediatric assessment of children with poliomyelitis and cerebral palsy at what was formerly the Children’s Orthopedic Hospital. He also played a pivotal role in establishing the Spastics Society of India (now ADAPT, or Able Disabled All People Together).

Positions held domestically included co-founder of the Indian Epilepsy Association, membership of the Neurological Society of India (he was president in 1961), the council of National Academy of Medical Sciences, inspector of the Medical Council of India, examiner for the DM (Doctor of Medicine) and DNB (Diplomate of National Board) exams throughout India, Honorary Lieutenant Colonel in the Armed Forces and physician to the Reserve Bank of India. He received the Dhanvantari Award in 1991 for his outstanding contributions to medical sciences, in particular in the field of neurology.
Overseas, he was the president of the World Congress of Neurology (1989), vice president of the World Federation of Neurology (1969-1973), honorary member of the Association of British Neurologists, special member of the International Cerebral Palsy Society, and honorary member of the American Neurological Association.

His research and publications pertained to stroke, neurological infections (tuberculous meningitis, postconjunctival myeloradiculopathy), nutritional disorders (especially the relationship between vitamin B, malnutrition, and chronic alcoholism), toxic disorders (lathyrism and mass screening of those exposed to toxic gas in Bhopal within a week of the tragedy), congenital abnormalities (atlantoaxial dislocation), and epilepsy and epidemiology of neurological diseases in the Parsi community. He was on the editorial advisory board of the Handbook of Clinical Neurology (eds., P.Vinken & G.Bruyn, 1973 onward).

Dr. Bharucha was a consummate physician who relied on clinical assessment rather than imaging studies. Notwithstanding his academic achievements and his work to advance the practice of neurosciences, his primary concern always remained the best interests of his patients. This, combined with his deep empathy (particularly toward the underprivileged) and an ingrained sense of social responsibility, led to his involvement in several projects aimed at alleviating the stigma and distress suffered by those with neurological disorders. One example of this was the 12-year campaign that he led which ultimately led to the repeal of a law that deemed people with epilepsy to be insane. Another was his importing (at his own expense) of an early EEG machine for the KEM Hospital, part by part. He also imported (again at personal expense) medicines for patients that were not available in India at that time, such as d-penicillamine for a patient with Wilson’s disease. Finally, his contributions during the riots of Partition, in 1947, should be mentioned. He was among a group of doctors from the KEM Hospital that went to Lahore, where they provided medical relief. Using the Sir Gangaram Hospital as a base, they visited various refugee camps, escorted by troops from the Gurkha regiment. The trip was organized by Lady Edwina Mountbatten and Dr. Jiwraj Mehta.

He was a man who, by virtue of character and example, came to fulfill many roles for many people – clinician, teacher, mentor, friend, husband, father, and grandfather. Piloo and he had three sons – Nadir, Manek-Phiroz, and Adil, eight granddaughters and two great-grandchildren. In 2004, his students, friends, and family created the Eddie and Piloo Bharucha Fund at the World Neurology Foundation to support a perennial named lectureship at the World Congresses of Neurology.

His ethos enveloped the departments he worked in, including other professionals, patients, students, and hospital staff. For the last 20 years of his life, he was virtually blind. In anticipation of his loss of vision, he read several journals of neurology onto a dictaphone, which he was to listen to in solitude after he could no longer see. Ultimately, his hearing was also severely impaired. Nonetheless, he retained his sense of equanimity, never complained, and was always happy to see family and visitors.

His greatest legacy (both in the profession and for his family) will be his personal qualities. He did not seek positions of power or prestige, was unconcerned about material remuneration, and perennially radiated good cheer, kindness, and caring. We mourn his passing but know he remains very much alive in the hearts and minds of all those he touched.

– Nadir Bharucha •

Next European Board Examination in Neurology

The next Exam of the European Board of Neurology (EBN) will take place on Friday, June 15, 2018 at the EAN congress site in Lisbon.

Prof. Wolfgang Grisold from Vienna successfully started this annual event in 2009. This year, we celebrate the 10th examination.

Every year, the number of participants increases. We receive a lot of mostly positive feedback from our candidates, which makes it possible to improve the exam continuously. Many young neurologists consider taking this exam to aid them while preparing for the written tests and writing their contributions for the oral exams.

Candidates at the European Board Examination in Amsterdam.

Candidates at the European Board Examination in Amsterdam.

Participating in the EBN exam is not merely a way of showing success in retrieving information accumulated during training and reading literature, but more than that a demonstration of the ability of sound reasoning, presenting insight and working with accessible knowledge. Candidates are allowed to use external reference sources and prepare work at home that will be judged – and can be modified after personal feedback – before they come up for the real exam.

They can update and expand their knowledge and abilities, take part in a unique academic experience, and get an additional sign of excellence. Thus, they show their commitment for life-long learning. Although the EBN exam has no legislation until now, the title “fellow of the European Board of Neurology” (which does not coincide with “fellow of the EAN”) may enhance the candidate’s possibilities to work in other countries within and outside Europe.
For the next years, the collaboration between EBN and EAN will be intensified in a great way. Using more technical possibilities (as provided by the Swedish Company Orzone) will allow us to extend our questions to imaging, signal analysis, and clinical judgment with the help of movies.
Candidates who are members of EAN’s Resident and Research Fellow Section for at least one year now get a considerable reduction.

More information can be found at www.uems-neuroboard.org.

We sincerely hope to get more European candidates, not only junior neurologists, but also those who are already practicing. •


Prof. Jan Kuks is the chair of the EBN examination committee, and works at the University Medical Center Groningen in the Netherlands.

WFN Invites Bids to Host WCN 2023

The World Federation of Neurology is pleased to invite bids to host the World Congress of Neurology (WCN) in 2023 from national member societies in the Americas.

If your national society would like to be a candidate to host WCN 2023, please write to the WFN Headquarters Office at the address below to express your preliminary interest by July 31, 2018.

World Federation of Neurology
Chester House
Fulham Green
81-83 Fulham High Street
London
SW6 3JA
United Kingdom
email: info@wfneurology.org

You will be sent an application form to complete and return by Sept. 18. All applications will be treated in confidence and will be reviewed by the WFN to draw up a short list of possible venues. Those that are short-listed will be notified by mid-December 2018, and a site visit by WFN officers to evaluate the proposed program and facilities on offer will take place in the ensuing three months. A report and recommendation will then be submitted to the Council of Delegates meeting during the WCN 2019 in Dubai, United Arab Emirates, where the final vote will occur.

The WFN looks forward to hearing from any member neurological society that wishes to be considered as the host of WCN 2023. •