Medical Observations by European Physicians in the Colonies

Philippe Fermin’s Observations in 18th-Century Surinam

BY Peter J. Koehler, MD, PhD

Fig. 1. Title page of Fermin's Traité des Maladies (1764)

Fig. 1. Title page of Fermin’s Traité des Maladies (1764)

The stay of Europeans in tropical countries offered opportunities for the observation not only of unknown (manifestations of) diseases, but also of the flora and fauna of these areas. Extensive descriptions were published in books, and specimens were brought to Europe, where cabinets of curiosities were filled with objects from natural history, archeology, geology, and ethnology.

Fermin

One of the adventurers who visited a Dutch colony was Philippe Fermin (1729-1813). The son of French Huguenots was born in Berlin and went to the local French gymnasium. Then he moved to London, where he apprenticed with an elderly physician. Not wishing to have to train for several years, he obtained a certificate for the practice of surgery in Rotterdam and, at the age of 25, he moved to Surinam (a former Dutch colony, now the Republic of Suriname) in 1754 to serve for the government. He was offered 20 florins a month for providing medication. Although he realized that his training had been insufficient, he started to practice. Much of the information that is known about him was provided by his correspondence (116 letters between 1753 and 1789) with the Berlin theologist, philosopher, and historian Jean H.S. Formey (1711-1797), contributor to Encyclopédie (Diderot, d’Alembert) and member of the Berliner Akademie der Wissenschaften. Fermin dedicated his first book to him, adding, “Depuis ma plus tendre jeunesse vous m’avez honoré d’une protection aussi constante qu’efficace; je n’ai cessé d’en ressentir les salutaires effets …,” which translates to, “Since my tender loving youth, you have honored me with a continuous, as well as efficacious protection; I have experienced the salutary effects continuously” (Traité, 1764; fig. 1). He often asked him for letters of recommendation or merchandise that was lacking in Surinam. He married the widow of a well-to-do apothecary, Maria Magdalena Morin, and became a person of good report. Although it is unknown how he managed to do so, he obtained a degree, probably a doctorate honoris causae, from the University of Aberdeen in 1758. Meanwhile, he observed and described flora and fauna and sent specimens of the local flora and rare insects to the Akademie in Berlin. After an eight-year stay in Paramaribo, he had made a good deal of money, more than 20,000 florins, which is more than his fellows would have at the end of their lives. He estimated the sum to carry interest rates to live decently. He and his wife departed for the Netherlands on May 5, 1762. Upon arrival in the Netherlands, he settled in Maastricht and published a number of books. He was considered a specialist in natural history of equatorial America.

Treatise on the Most Frequent Diseases in Surinam

His first book, Traité des Maladies les Plus Fréquentes à  Surinam et des Remè des les Propres à  les Guérir, which translates to The Most Frequent Diseases in Surinam and the Appropriate Remedies to Treat Them, was published two years after returning from Surinam (1764). In the introduction, he wrote: “Un Médecin nouvellement débarqué dans ce Païs, a deux choses principales à  faire. La première, c’est d’observer avec la derniè
re exactitude la Nature du Climat & les variations qui influent si considérablement sur l’état des corps & sur l’effet des remèdes …” or “A physician recently arrived in that country, has two principle things to do. The first is to observe the nature of the climate with the greatest accuracy and the changes that influence the state of the body in a considerable way and the effects of the remedies …” He opined that many diseases of the people living in Suriname, Creols, as well as Europeans, resulted from excesses of debauchery.

Neurological Disorders

Fig. 2. Title page of his Description Générale (1769)

Fig. 2. Title page of his Description Générale (1769)

Several neurological disorders were described in his Traité, although the author sometimes had problems making a diagnosis. In a chapter named “De la Fiè
vre Ardente” (“Burning Fever,” possibly American typhus or yellow fever), he described an often mortal disease accompanied by heat, thirst, nausea, anxiety, insomnia, vomiting, delirium, coma, and convulsions. Pain was felt in the region of the stomach. Fermin prescribed bloodletting, as well as all kinds of drugs, including cort. Peruvian (Peruvian bark, cinchona) and Sem. Papaver (semillas de papaver, or seed of papaver for insomnia). Another  disease, known as “klem” (grip), Fermin believed, resembled apoplexy, as well as catalepsy, but more probably he thought it was “tetanos.”

In a chapter named “Du Boisi,” he described leprosy as, “Quand cette Maladie qui peut durer dix, vint, jusqu’à  trente ans, est une fois parvenue à  son plus haut degré, les doigts et les orteils se détachent insensiblement d’eux-màªmes, sans que le Malade en soie douloureusement affecté,” meaning “When that malady that may last 10, 20, up to 30 years, has reached its highest degree, the fingers and toes come off insensibly, without the patient being painfully affected. It has a poor prognosis, he writes. “Cette horrible Maladie est absolument incurable. Elle n’est pas fréquente parmi les blancs, mais elle attaque souvent les Esclaves.” It translates to “That horrible disease is absolutely incurable. It is not frequent among the whites, but it often attacks the slaves.” The disease was considered contagious and could become epidemic. A patient would be indicated a place in the woods, where he had to end his days without any communication with the others. Fermin himself had learned to distinguish the spots of leprosy from those of other skin diseases, like “ringworm” (tinea), through a lesson from “une vieille Négresse” — stick a needle into the spot while pinching it; if it is insensible, then it’s leprosy.

Fig. 3. Trembling eel from Gronov's Zoophylacium (1758)

Fig. 3. Trembling eel from Gronov’s Zoophylacium (1758)

Another chapter (no. 9 “Du beillac”) is of neurological interest. The name derives from the Creol or original inhabitants, Fermin believed. It was associated with the work of the devil because of the severe colic pain. He believed it was nothing else than the well-known “colica pictonum” (an affliction known since the Roman Empire, of which it was assumed later that it was due to chronic lead intoxication; the lead was used to sweeten the wine). However, the Surinam beillac was more severe, Fermin opined, and was caused by immoderateness (drink, lack of sleep). He thought it should not be confused with other types of colic and believed it was often treated in the wrong way, leading to paralysis or death. He presented a long list of drugs and regimens to apply. The combination of colic pain and paralysis could indeed fit a chronic lead intoxication, particularly when the term colica pictonum was used. Today we could also think of Guillain-Barré syndrome preceded by an infectious bowel disease and porphyria, although intermittent disease would be expected in the latter. However, we have to be careful with making retrospective diagnoses. Furthermore, it is important to realize that, at the time, differences in diseases across the world were believed to be of degree, not of kind. Humidity, fluctuating and high temperatures, and the sun were considered the most important factors to weaken the Europeans, increasing their vulnerability to disease. The pathophysiology was still humoral and mentioned conditions were thought to cause unbalance of the humors.

One year after his Traité, Fermin published Histoire Naturelle de la Hollande Equinoxale ou Description des Animaux, Plantes, Fruits et Autres Curiosités Naturelles, que se Trouvent Dans la Colonie de Surinam Avec Leurs Noms Différents Tant François que Latins, Hollandois, Indien et Nègre-Anglois, which translates to Natural History of Equatorial Holland or Description of Animals, Plants, Fruits and Other Natural Curiosities, that are Present in the Colony of Surinam With Their Different Names be it French, or Latin, Dutch, Indian and Negro-Anglian (Amsterdam, Magérus, 1765).

Fig. 4a: Frontispices from Du Bois-Reymond's volumes depicting a torpedo

Fig. 4a: Frontispices from Du Bois-Reymond’s volumes depicting a torpedo

In 1768, he published “Instructions Importantes au Peuple sur les Maladies Chroniques. Pour Servir de Suite à  l’Avis au Peuple de M. Tissot, sur les Maladies Aiguës.” That translates to, “Important Instructions for the People on Chronic Diseases. To Serve as a Continuation of Advice to the People by Mr. Tissot, on the Acute Diseases.” Indeed, the well-known Swiss physician Samuel Auguste Tissot (1728-1797), author of Traité des Nerfs, had published his “Avis au Peuple sur sa Santé, ou Traité des Maladies les Plus Fréquentes,” which translates to “Advice to the People on its Health, or Treatise on the Most Frequent Maladies” in 1761, with several new editions to follow. Tissot’s original purpose had been to publish it as an “ouvrage composé en faveur des Habitans de la Campagne, du Peuple des Villes, et tous ceux que ne peuvent avoir facilement les conseils des Médecins” — “text put together in favor of the inhabitants of the countryside, the people of the cities and those who are not able easily to consult physicians.” Fermin’s first volume contains 20 chapters on anatomical subjects; the second volume contains 58 chapters on diseases and surgical subjects. About 14 of these deal with neuropsychiatric subjects, including tremor, spasm, catalepsy, melancholia, mania, headache, speechlessness, and memory loss.

In 1770, Fermin pleaded for better treatment of the slaves in his “Dissertation sur la Question: S’il est Permis d’Avoir en sa Possession des Esclaves et de s’en Servir Comme tels dans les Colonies de l’ Amérique,” which translates to “Dissertation on the Question: Is it Allowed to Keep Slaves in One’s Possession and Make Use of Them in the Colonies of America.”

Trembling Eel

Fermin’s “Histoire Naturelle” of 1765, mentioned earlier, was later augmented and published in two volumes Description Générale (Amsterdam, Van Harrevelt, 1769; fig. 2), which were translated into German and Dutch. At least one part of this book is of neurological interest, notably where he described the trembling eel (fig.3). This animal had been of interest, in particular during the 18th and 19th centuries, and was studied by colonists as well as scientists in Europe. “If one touches it with the hand or a stick, it causes an involuntary or forced trembling, resembling the vibration.” Fermin was able to keep the fish alive in a tub for six weeks and showed a keen interest on electric transmission of potentials from one person to another.

Fig. 4b: A horse brought down by the discharge of an electric eel

Fig. 4b: A horse brought down by the discharge of an electric eel

He described the numbness being felt in the arms up to the shoulders and in the legs when the fish touched his hand or foot. He intended to further investigate the fish, but the heat of the country prevented him to completely dissect the animal. However, he observed two muscles that were distinct from the other muscles. He supposed that these were the principal instruments of the trembling. Indeed, it was in 1776 that the electric nature of the fish was proved at the Royal Society (London) by drawing sparks. At the time, the ability to produce a spark was considered a fundamental criterion for something to be electrical in 18th century science. These and other observations influenced Luigi Galvani, when he described animal electricity (finding Alessandro Volta as his opponent). About half a century later, the Berlin physiologist Emile du Bois-Reymond proved the electric nature of nerves by discovering the action potential (figures 4a and b).

Fermin kept on writing about his observations and published another extension of his 1769 Description Générale in 1776, “Tableau Historique et Politique de l’état Ancien et Actuel de la Colonie de Surinam et des Causes de sa Décadence,” (Maastricht, Dufour and Roux), which translates to, “Historical and Political Depiction of the Ancient and Actual State of the Colony of Surinam and the Causes of its Decline.” It was translated into German in 1778 and English in 1788.

During the last decades of his life, Fermin served as juror and alderman in the city of Maastricht, and, when the Netherlands were under French rule (1795-1813), he became judge of the civil court of justice, and from 1803 a substitute judge at the Cour Criminelle.

Peter J. Koehler, MD, PhD, is the editor of this history column. He is a neurologist at Zuyderland Medical Centre, Heerlen, the Netherlands, and co-editor of the Journal of the History of the Neurosciences. Visit his website at www.neurohistory.nl.

 

 

BOOK REVIEW: Emergency and Critical Care Neurology

By Katharina M. Busl, MD, MS

BookReview_Jul2016-coverThis is a comprehensive, extremely well-written, single-author textbook on emergency and critical care neurology by one of the most renowned, experienced, published, and respected neurointensivists. In his preface to this second edition, the author outlines his intention for this book to serve as a practical and data-driven guide to management of the critically ill neurological patient, rather than a textbook detailing theory. This is exactly how I perceived this book. It is organized into 12 chapters that include a symptoms-based approach, organizational aspects, general critical care aspects, and management of specific disorders, complications, or consultation situations. Some of the chapters are geared more toward the neurologist assessing emergent consultation, some to the neurologist or neurointensivist managing the specific neurocritical disorders, and some to the neurointensivist or critical care physician staffing an intensive care unit. Complex topics and concepts are presented in a clear and concise manner, and enhanced by plenty of original illustrations and imaging examples. The text is supported by available, up-to-date data, as well as the vast clinical experience of the author himself. Sensitive topics, such as end-of-life discussions or ethical dilemmas, are addressed with vision and care. The statements are clear with virtually no redundancy or ambiguity. Flow is excellent, and style and organization allow for both rapid, continuous reading, as well as rapid look-up.

KATHARINA M. BUSL, MD, MS

KATHARINA M. BUSL, MD, MS

There are very few minor aspects that could be mentioned critically. The chapter on critical care support appears rather truncated, which is understandable given the breadth of these topics. However, a more specific link to the neurological patient could possibly have been made. Similarly, the chapter addressing systemic complications touches only little on dilemmas in the neurological critically ill patient, such as gastrointestinal bleeding in patients who require antiplatelet medications, or pulmonary embolism in patients with intracerebral hemorrhage. However, the author acknowledges the biggest problem — absence of data for the neurocritical care population. Furthermore, a chapter on critical illness myopathy or neuropathy, a problem often addressed in the general critical care literature, but paid less attention to in the neurocritical care population, could have completed the chapter on complications.

The textbook comes with a handy pocket book (in print or mobile device version) that contains a selection of the most relevant tables and figures and a compilation of practical notes.

In summary, this book is very impressive in its composition, scope, and style. I wholeheartedly recommend this wonderful book for any clinician primarily caring for critically ill neurological patients, specifically for practicing neurointensivists and neurocritical care fellows. In the larger scheme, it is also of interest to any neurologist or physician of other specialty consulting on critically ill neurological patients.

Katharina M. Busl, MD, MS, is chief of the Division of Neurocritical Care and co-director of the neuroscience intensive care unit at the University of Florida Health Shands Hospital and associate professor of neurology, neurosurgery, anesthesiology, and bioengineering at the UF College of Medicine, Gainesville.

A New Regional Organization: PAFNS

By Marco T. Medina, MD, MPhil, FAAN, and Gustavo C. Román, MD, DrHC, FAAN

WFN President Raad Shakir, MD, (seventh from left), WFN Regional Director for Latin America Marco T. Medina, MD, MPhil, FAAN, (eighth from left), and Latin American WFN representatives gather during the Pan-American Federation of the Neurological Societies (PAFNS) meeting at the World Congress of Neurology Nov. 5, 2015, in Santiago, Chile, where the PAFNS legal status as a nonprofit organization under Chilean Law received approval.

A new regional organization, the Pan-American Federation of Neurological Societies (PAFNS), has been founded with the support of 20 Latin American countries, the World Federation of Neurology (WFN), and the American Academy of Neurology (AAN).1 Fifty-three years ago, the first Pan-American Congress of Neurology organized by the WFN, under the leadership of Professor Julio Oscar Trelles, met in Lima, Peru.2 Since then, neurologists from Latin America have attended the regional Pan-American Congresses organized by the WFN every four years. During that time, most of the regional educational and research activities for the region were promoted by the WFN.1-4 However, the need for an official regional organization became clear, mainly due to the growth of clinical neurology outside the United States and Canada on the American continent, as well as the need for up-to-date neurological information provided in Spanish and Portuguese.

Representatives from several Latin American countries recognized the particular educational needs of the region. This led to the Declaration of Morocco, which was signed by WFN Latin American delegates on Nov. 15, 2011, during the 20th World Congress of Neurology in Marrakech. The declaration stated that a regional continental organization was needed: “to coordinate and support the efforts of the member societies towards improvement of neurological services for the peoples of the American continent, as well as to optimize neurological care, education and research, and to promote public health initiatives to increase awareness of the importance of brain health.”

A commission formed by representatives from Chile, Brazil, and the Dominican Republic implemented the bylaws required for the creation of the PAFNS. On March 5, 2012, delegates attending the 13th Pan-American Congress of Neurology in La Paz, Bolivia, endorsed the declaration.4 On March 20, 2013, all Latin American delegates attending the 65th Annual Meeting of the AAN in San Diego, California, formally approved the PAFNS constitution.

The following countries approved and signed the constitution as founding and active ordinary members: Argentina, Brazil, Bolivia, Chile, Colombia, Costa Rica, Cuba, Dominican Republic, Ecuador, El Salvador, Guatemala, Honduras, Mexico, Nicaragua, Panama, Paraguay, Peru, Puerto Rico, Uruguay, and Venezuela. The Ibero-American Stroke Society, Commission on Latin American Affairs of the International League Against Epilepsy, and the World Sleep Society have requested to be associate members.

The enthusiastic regional support from all the Latin American member societies of the WFN; steady leadership of Gustavo C. Román, chairman of the WFN Latin America initiative, and Marco T. Medina, WFN regional director for Latin America1,4; and the support of Dr. Briseida Feliciano, Dr. Ana Robles, Professor Renato Verdugo, and others were critical for the foundation of the PAFNS. Two presidents of the WFN provided strong patronage to the project, Professor Vladimir Hachinski and Professor Raad Shakir. Enthusiastic support for this initiative was received from Professor Timothy Pedley, then president of the AAN, and Professor Morris Freedman, Canadian representative to the WFN.

On Nov. 5, 2015, during the XII World Congress of Neurology in Santiago, Chile, the legal status of the PAFNS as a nonprofit organization under Chilean law was signed, having as witnesses Professors Shakir, Medina, Román, Verdugo, and Sergio Castillo, as well as numerous Chilean and Latin American neurologists.

Legal counsel for elaboration of the PAFNS bylaws and establishment of the non-for-profit tax status was made possible thanks to grants provided by the WFN and the AAN.

During the upcoming Pan-American Congress of Neurology in Cancun, Mexico, at the end of 2016, the Council of Delegates will elect the new PAFNS Board of Directors. The PAFNS will be the preeminent neurological association of the Americas, working toward maximizing the neurological health of the people in all countries in the American continent through education and awareness of the importance of early care of brain diseases and dissemination of advances in neuroscience and the goal of optimizing neurological patient care. The PAFNS’ vision is to reach the highest level of neurological health in all the countries of the American continent. The creation of the PAFNS represents a major step for the improvement of regional neurological care, education, and research.

Marco T. Medina, MD, MPhil, FAAN, is WFN Latin American regional director and dean of the faculty of medical sciences, National Autonomous University of Honduras, Tegucigalpa. Gustavo C. Román, MD, DrHC, FAAN, is WFN chairman, Latin America Initiative, and the Jack S. Blanton Distinguished Endowed Chair and a professor of neurology, Weill Cornell Medical College, Methodist Neurological Institute, Houston, Texas.

References

  1. M.T. Medina, G.C. Román. The Pan-American Federation of Neurological Societies (PAFNS): A New Regional Organization, J. Neurol. Sci. 366 (2016) 195-196.
  2. Johan A. Aarli (Ed.), The History of the World Federation of Neurology: The First 50 Years of the WFN, first ed. Oxford University Press, Oxford, 2014.
  3. M.T. Medina, T. Munsat, Neurology Education in Latin America and the World Federation of Neurology, J. Neurol. Sci. 298 (2010) 17-20.
  4. T. Munsat, J. Aarli, M. Medina, G. Birbeck, A. Weiss, International issues: Educational Programs of the World Federation of Neurology, Neurology 72 (2009) e46-e49.
  5. V. Hachinski, President’s Column: The Formation of the Latin America Federation of Neurological Societies, World Neurol. 27 (3) (2012)

 

Observership Report: WFN and Austrian Neurological Society Department Visit Program

From left to right, Kalpesh Deraji Jivan, MD, Bettina Pfausler, MD, and Ralmund Helbok, MD

From left to right, Kalpesh Deraji Jivan, MD, Bettina Pfausler, MD, and Ralmund Helbok, MD

I was fortunate to receive funding to join the neurology intensive care unit at Innsbruck Medical University in Austria as an observer for one month in October 2015. There are no specialized neuro-ICUs in South Africa, where I am from. In South Africa’s public health care system, critical care for patients with neurological illnesses remains in the domain of our pulmonologists and anaesthetists. However, due to lack of beds in the general ICU, patients with critical neurological illness are often overlooked for admission to the ICU, and are unfortunately, left in the general neurology ward to receive basic care. This results not only in higher morbidity and mortality rates, but a sense of despair among the medical staff in not being able to deliver the best treatment.

The neuro-ICU at Innsbruck Medical University was set up in the early 1980s with the principle aim of offering rigorous neurological and neuro-rehabilitative support to acutely ill neurological patients. The unit has developed into a world-leading neurological ICU with 10 ICU beds and six step-down, high-care beds. The unit is equipped with modern mechanical ventilators and multi-modality cardiovascular, respiratory, and neurological monitoring.

Their patient population includes traumatic brain injury, subarachnoid and intracerebal hemorrhage, severe ischemic strokes, status epilepticus, neuromuscular disorders, severe central nervous system infections, brain tumors, encephalopathies (metabolic/toxic/anoxic), etc.

The unit is run by Professor Erich Schmutzhard and his team of neurological intensivists (Dr. Bettina Pfausler, Dr. Ralmund Helbok, and Dr. Ronny Beer). There are usually three to five junior doctors, including interns, medical officers, and neurology and neurosurgery resident doctors. There are approximately 60 ICU trained nurses and a full complement of allied medical personnel, including speech therapists and physiotherapists. Its monitoring capabilities are similar to that of other neurocritical care units and include measurement of intracranial pressure, cerebral perfusion pressure, brain tissue oxygen saturation, cerebral microdialysis, continuous electroencephalography, near infrared spectroscopy, transcranial Doppler and transesophageal echocardiography. The team routinely performs placement of central venous and arterial lines, continuous renal dialysis, and intravascular cooling for prophylactic normothermia and therapeutic hypothermia. Apart from clinical work, the department is very academic, dishing out leading research publications on a regular basis.

The morning starts off with a meeting with the entire neurological department discussing all neurological patients admitted in the past 24 hours and reviewing their scans. Frequently, this is followed by an academic presentation. Thereafter, the neuro ICU team has a handover round, during which the doctor covering the ICU the previous night hands over the patients to the day staff. Upon cessation of this round, the team proceeds with the actual ward round led by one of the consultants, meticulously examining each and every patient, and deciding on the treatment plan for the day. Thereafter, I would accompany the consultant to see any consults elsewhere in the hospital and observe procedures finishing in the neuro-ICU.

The experience was well above my expectations and gave me a wider understanding of the management of neurological conditions in a first world setting and how different (and occasionally how similar) it is to South Africa. I was impressed by the efficiency with which things get done in the hospital. Due to limited resources available in the public hospitals in South Africa, patients can wait up to two weeks (if not longer) for scans, whereas scans requested at the Innsbruck neuro-ICU were completed on the same day. One of the highlights was to follow patients with invasive intracerebral monitors (intracranial pressure, brain tissue oxygen saturation, cerebral microdialysis, etc). It was interesting to see how this monitoring played a role in daily management of these patients. This type of monitoring is not readily available in the public hospitals in South Africa. Another highlight was observing therapeutic hypothermia being performed on a 72-year-old woman who developed cardiac arrest in a primary hospital. She recovered spontaneous circulation after CPR. She was flown by helicopter to Innsbruck. Following the therapeutic hypothermia and neurological intensive support, she woke up a few days later with no significant neurological deficit. Upon seeing patients with severe brain pathologies recover, this visit has invigorated my enthusiasm in providing the best available care to my patients and not to give up too easily when no other resources are available.

Acknowledgement:
 
First, I would like to thank the World Federation of Neurology (WFN) and the Austrian Neurological Society for selecting me to participate in the inaugural department visit program and to gain valuable experience. I am grateful to Professor Girish Modi for bringing the advertisement for the observership to my attention and assisting me in applying for it. I would also like to extend my gratitude to Tanja Weinhart, the executive secretary of the Austrian Society of Neurology, for arranging my flights and accommodation, thus making my stay in Innsbruck very comfortable. A big thanks goes to Professor Reinhold Schmidt and Professor Wolfgang Grisold for taking time out of their busy schedule to invite me to lunch in Vienna, and not only educating me about health care in Austria, but also adding to my waistline by spoiling me with their local delicacies. I am grateful to Professor Schmutzhard and his team for accommodating me in their department. Lastly, a big thanks goes to Dr. Bettina Pfausler for ensuring that I learned a lot about neuro-ICU. I would have to admit that I struggled with the language, as all meetings and ward rounds are conducted in German; however, Dr. Pfausler and Dr. Helbok were on hand to translate for me. Dr. Pfausler, a self-proclaimed “proud Tyrolean,” not only ensured that this observership was successful from an academic standpoint, but she also educated me on the history of the Tyrol region of Austria.

John Sutherland and Multiple Sclerosis in Scotland and Australia

J. Eadie, Brisbane, Australia

M. J. Eadie

M. J. Eadie

Over several centuries, those born in Scotland have often left their homeland because of the perceived lack of opportunity, and then made successful lives in other countries. This behavior was particularly frequent during the so-called Highland clearances of the 18th and 19th centuries. Scottish landowners dispossessed their tenant farmers who had been engaged in small-scale agriculture for generations, to make land available for more profitable sheep rearing. As one consequence, more people of Scottish ancestry now live elsewhere in the world than in Scotland itself. In 1956, there was a Highland clearance on a micro scale, when lack of available consultant positions in the British National Health Service resulted in a trained neurologist of Highlander origin emigrating to Australia.

An outline map of Australia found among John Sutherland's papers, with dotted latitude lines added to it in his hand. The map includes names of the cities where he studied multiple sclerosis prevalence, and the local prevalence figures per 100,000 of population he obtained, have subsequently been inserted; the figures from his earlier survey in red, and from the later one in purple.

An outline map of Australia found among John Sutherland’s papers, with dotted latitude lines added to it in his hand. The map includes names of the cities where he studied multiple sclerosis prevalence, and the local prevalence figures per 100,000 of population he obtained, have subsequently been inserted; the figures from his earlier survey in red, and from the later one in purple.

John Sutherland (Fig 1) was born in Caithness, in the extreme northeast of mainland Scotland, in 1919. He was educated in Glasgow, and graduated in medicine from the University of Glasgow in 1943. After service in the wartime Royal Navy, he returned to the Western Infirmary, Glasgow, in 1946 as medical registrar to Douglas Adams, a consultant physician with neurological interests, particularly concerning multiple sclerosis. Adams launched Sutherland into clinical and laboratory research related to the disease, resulting in the addition of a research medical degree (Glasgow) to Sutherland’s existing basic medical qualifications. After his appointment in Glasgow ended, Sutherland went to Inverness, the so-called “capital” of the Scottish highlands, in 1950, as senior medical registrar. He remained there for five years, followed by some months in a similar level position in Aberdeen, a little further south on the east coast of Scotland. In Inverness, though without the formal title, he in effect functioned as neurological consultant for the Scottish Highlands, the Hebridean Islands off the northwest coast of the country, and the Orkney and Shetland Islands north of the mainland. From Inverness he investigated a suspicion that arose out of his multiple sclerosis clinical studies in Glasgow, namely that there might be an uneven distribution in the occurrence of multiple sclerosis between parts of Scotland.

Because of his close professional relationships with medical practitioners in the areas he serviced, he achieved a high multiple sclerosis case ascertainment rate and, after various field expeditions, found that the prevalence of the disease in the shaded areas of the map (Fig 2) covered over in red was approximately twice that in the remaining shaded parts. He realized that the red areas were those where the population was predominantly of Nordic origin, a later-day result of Viking invasions and settlement centuries earlier. The remaining shaded areas had populations largely of Gaelic lineage and who still could understand and employ the Gaelic language. Thus, he obtained persuasive evidence, perhaps the earliest, that genetic and racial factors might play a role in the etiology of multiple sclerosis.

John Sutherland's map of Scotland. The shaded areas show where he investigated the prevalence of multiple sclerosis, with the original version modified by coloring over in red in the higher prevalence areas.

John Sutherland’s map of Scotland. The shaded areas show where he investigated the prevalence of multiple sclerosis, with the original version modified by coloring over in red in the higher prevalence areas.

In the year that this study was published,1 Sutherland arrived in Australia to take up an academic position in Brisbane, the capital of the state of Queensland that occupies most of the northeast quarter of the continent (Fig 3). After a few years, he commenced consultant neurological practice in that city. In the 1950s, it was generally believed locally that multiple sclerosis rarely occurred in the northern half of Australia, and that native-born Queenslanders never suffered from it. Sutherland soon showed that these ideas resulted from a local medical unfamiliarity with the spectrum of clinical manifestations of the disorder, so that it simply was not being recognized.

Knowing that multiple sclerosis prevalence increased with increasing distance from the equator in the northern hemisphere, and because of his earlier investigation being alert to the possible relationship between geographical and racial factors and disease distribution, Sutherland proceeded to investigate whether a similar disease prevalence distribution in relation to latitude existed in the southern hemisphere. No adequate data were then available. Australia was a peculiarly suitable site for investigating the matter. It possessed a 3000 km -long north-to-south dimension, a uniform and high standard of medical practice throughout, main population centers spaced reasonably evenly along its eastern seaboard, and was populated almost exclusively by those of British and other European stock who spoke English (though some of them may have initially wondered if Sutherland also did, until his broad Scotch accent gradually became somewhat attenuated).

John Sutherland in his 60's

John Sutherland in his 60’s

Sutherland initially carried out a field study of multiple sclerosis prevalence in three tropical Queensland coastal cities (Cairns, Townsville, and Mackay) and in sub-tropical Toowoomba (about 100 km west of Brisbane).2 Recognized cases of the disease were found to have a lower prevalence in the tropical than in the sub-tropical population, though the disease prevalence was low relative to that in Northern hemisphere populations.

Spurred on by this finding, he then collaborated with local neurologists and epidemiologists in selected southern cities (Newcastle, Perth and Hobart) to show a higher multiple sclerosis prevalence the further south the matter was studied.3 Later still, in another collaborative study that took in New Zealand, overall closer to the South Pole than the southernmost parts of Australia, further evidence was obtained.4 Thus Sutherland’s name became associated with a series of investigations that demonstrated beyond reasonable doubt that multiple sclerosis prevalence increased with greater distance from the equator in the southern as well as in the northern hemisphere.

He also initiated other multiple sclerosis epidemiological investigations in Australia. One study showed that the prevalence of the disease correlated with the notifications of paralytic poliomyelitis in epidemics of that disease at different latitudes in Australia. This finding led to speculation that exposure to some unidentified organism, occurring earlier in childhood in hotter climates, while some residual immunity of maternal origin persisted, might have prevented later clinical disease. However, in cooler climates, exposure to the cause occurred later and resulted in overt disease.5 He also drew attention to a correlation between decreasing average annual sunlight exposure in regions of Australia and increasing multiple sclerosis prevalence,4 a finding largely forgotten until resurrected by recent interest in the relation between vitamin D and the disease. Additionally, he proposed a speculative hypothesis regarding multiple sclerosis prevalence and the capacity of regional soils to bind molybdenum in preference to copper.6

In Australia, Sutherland became a very successful consultant who developed an additional major interest in medico-legal neurology. With the years, his active investigative work into multiple sclerosis diminished, though he retained a watch on the relevant literature until the end, which came in 1995.

Scotland’s inability to provide appropriate professional opportunity for another of its many sons thus provided Australia with one of that country’s pioneer neuroepidemiologists, a man whose investigative endeavors helped awaken ongoing broader interest into multiple sclerosis far from his homeland.

References

  1. Sutherland JM (1956) Observations on the prevalence of multiple sclerosis in northern Scotland. Brain 79: 635-654.
  2. Sutherland JM, Tyrer JH, Eadie MJ, Casey JH, Kurland LT (1966) The prevalence of multiple sclerosis in Queensland, Australia. Acta Neurologica Scandinavica 42 (Suppl 19): 57-67.
  3. McCall MFG, Brereton TLG, Dawson A, Millingen K, Sutherland JM, Acheson ED (1968) Frequency of multiple sclerosis in three Australian cities – Perth, Newcastle and Hobart. Journal of Neurology Neurosurgery and Psychiatry 31:1-9.
  4. Sutherland JM, Tyrer JH, Eadie MJ (1962) The prevalence of multiple sclerosis in Australia. Brain; 85:149-164.
  5. Eadie MJ, Sutherland JM, Tyrer JH (1965) Multiple sclerosis and poliomyelitis in Australasia. British Medical Journal 1: 1471-1473.
  6. Layton W, Sutherland JM (1975) Geochemistry and multiple sclerosis: a hypothesis. Medical Journal of Australia. 1:73-77
Mervyn Eadie is emeritus professor of clinical neurology and neuropharmacology, University of Queensland, Brisbane, Australia. Peter J. Koehler is the editor of this history column. He is a neurologist at Atrium Medical Centre, Heerlen, Netherlands. Visit his website at www.neurohistory.nl.

Overcoming Untreated Epilepsy in the Developing World: the Way Forward

Mamta Bhushan Singh, MD, and Michael F. Finkel, MD

Mamta Bhushan Singh, MD

Mamta Bhushan Singh, MD

The medical world thinks of the developed and developing societies in terms of diagnostic and treatment differences. However, there are realities that are common to both spheres concerning most medical conditions.

As a medical student, one would read of common disorders that were not being treated. Epilepsy was a striking example. In many rural Indian communities, up to 90 epilepsy patients out of every 100 were not getting treatment. In bureaucratese, this was a treatment gap of 90 percent.

Michael F. Finkel, MD

Michael F. Finkel, MD

The treatment of epilepsy has since evolved. Over two-dozen antiepileptic drugs are currently available. Many drugs are off patent and generically manufactured, and available in India and other developing nations at reasonable prices. But the epilepsy treatment gap of up to 90 percent still exists in many rural Indian communities! So, why has nothing changed for the lot of millions of epilepsy patients?

Let us humanize the numbers. By using calculations from an often-quoted epilepsy prevalence figure of 1 percent of the population, at least 12 million Indians are struggling with epilepsy.1 Large prevalence studies are unavailable, and many experts agree that this number is more likely to be closer to 15 million or even higher. This implies that more than 10 million epilepsy patients in India are untreated. Extrapolating worldwide, there are more than 50 million epilepsy patients and at least 40 million of these reside in developing countries. The estimate is that 75 percent of patients in developing countries are outside the domain of any kind of effective treatment. This implies at least 30 million untreated persons.

Ironically, epilepsy is one of the relatively easily controllable neurological diseases.

Untreated epilepsy with frequent generalized tonic-clonic seizures results in serious injuries and burns. Patients may lose sight, digits, or limb. The visible scars are further stigmatizing for patients who struggle with societal acceptance. Photo credit: Priya Jain.

Untreated epilepsy with frequent generalized tonic-clonic seizures results in serious injuries and burns. Patients may lose sight, digits, or limb. The visible scars are further stigmatizing for patients who struggle with societal acceptance. Photo credit: Priya Jain.

What is life like with untreated epilepsy? Most persons with epilepsy who do not have any other neurological illness are essentially normal at all times other than during their seizures. A seizure generally lasts for a few minutes during which, amongst other things, the person is unaware of his or her surroundings, and after which the person may remain confused for a variable amount of time or may sleep off the effects. In either case, the individual is back to normal within a few hours, often without even requiring a trip to the doctor’s clinic or the emergency room.

Then what is the great problem about having untreated active epilepsy with an occasional seizure? Imagine having brief episodes of unawareness just once or twice in a month, in six months or even in a year, but not knowing when these would happen. So, you may be driving to work, crossing the street, climbing a tree, in bed with your partner, swimming in the pool, or speaking mid-sentence in that all important meeting. Suddenly, out of the blue, you start convulsing or just fidgeting with your buttons or speaking in an unintelligible jargon or wet your clothes in full public gaze. How would it feel to know that you had done either of these, and then have to go back to the same people in front of whom this had happened? Well, that is assuming that these people agree to work or socialize with you after witnessing a seizure.

The economic impact of untreated epilepsy is formidable in all societies. Many untreated epilepsy patients remain unemployable, especially if local attitudes consider it to be a contagious disease, a mental illness, or a demonic possession. Thus, they are removed as contributors to the economy. Even if they are poor and have minimum wage jobs, this still adds up to a substantial amount of money loss because of the millions affected.

There are other grave consequences of untreated epilepsy. If the patient is of school or college age, they are very likely to have to drop out. This is either due to their parents’ fear that their child may be injured during a seizure, or on the insistence of teachers. With limited scope for education, landing a good job later in life is subsequently reduced. The intrigue and misinformation surrounding epilepsy reduces social acceptance of patients. Any relationship, including a stable marriage, especially for women with epilepsy, is unlikely. Abandonment of women with epilepsy and their children from marital relationships is common. Such women, with little or no education, no skills and sometimes also with small children to take care of, are often left at the mercy of an impoverished community.

So, is epilepsy difficult to treat? No. Epilepsy is a relatively easy disease to treat in most patients. The diagnosis is clinical and based upon eliciting a history of stereotyped episodes during which the patient may behave abnormally, have involuntary movements or jerks, generally become unaware of his surroundings and then recover in a short time. After the clinical diagnosis, a couple of investigations— EEG and brain imaging — may be done. Investigations may help in deciding the best anti-epileptic drug, in prognosticating how long the treatment is likely to be needed, and how responsive to treatment the patient’s epilepsy might be.

However, even if investigations are not available, as is the case in many developing countries, most patients can still be started on treatment based on clinical history and examination alone. Treatment costs are not very high with generic medications. For example, in India, patients who are treated with one antiepileptic drug spend about $2-$5 per month. Many other developing countries face severe shortages of antiepileptic medication and also have to contend with high medication costs. Along with starting antiepileptic drugs, it is necessary to educate and inform patients at the first visit and at every subsequent opportunity, about their disease and it’s treatment. Without backing up drug treatment with education or “epilepsy literacy,” outcomes are unlikely to be good. After starting treatment, regular reviews at least once or twice a year are needed. Patients have to continue treatment for several years. At least 60-70 percent of epilepsy patients will become seizure-free on medication quite easily. The remaining minority have more difficult forms of epilepsy and need further evaluation and treatment at a specialized epilepsy center. Surgery may be an option for some of these difficult patients.

What are the main challenges? Despite the relative simplicity of the process of epilepsy diagnosis and initiating treatment, millions remain untreated in India and the developing world. To our minds, the biggest hurdles are reliance on specialist doctors who are either entirely missing or extremely scarce and located only in a few large metropolitan cities. This produces a lack of organized, credible, and easily accessible primary care throughout these countries.

Hence, we have the current situation in these countries, where effective systems are not in place. For example, even if an epilepsy patient in a rural area is aware that visiting a doctor and getting the condition treated is possible, what can he do? To whom does he or she go? There is no easy track for one to follow. In India, a hierarchy of health centers exists with a scaling up in staff skill and numbers, and improvement in facilities based upon the size of the population being served. However, doctors and services are not reliably available at these facilities. As a result, an epilepsy patient in a village can either spend an entire lifetime untreated and seizing, or, if he or she is feisty, may make a long and expensive journey to a big city for treatment. No patient should have to do that. In India, there are about 638,000 villages with difficult access. Therein lies the problem. Unless treatment is made available within a radius of about 25-50 kilometers of every small village and town, epilepsy patients and the nation’s economy will continue to suffer needlessly.

Some epilepsy is also preventable. Millions of epilepsy patients in India and worldwide have what is best described as “preventable epilepsy.” In other words, their epilepsy should not have even happened in the first place. Tapeworms cause epilepsy associated with neurocysticercosis. It reflects lack of access to clean food and water, poor sanitation and hygiene, and is an illustration of the public health aspect of epilepsy. Accidents, including frequent road traffic accidents, may lead to head injury and posttraumatic epilepsy. This form of epilepsy is notoriously difficult to treat and often requires surgery. Birth injuries and hypoxic brain damage are more likely to occur with unsupervised childbirth at home. This practice is diminishing, but still prevalent in many rural communities in the developing world. Such babies may also have epilepsy in addition to cerebral palsy, mental subnormality, and behavioral abnormalities. Other preventable epilepsies include epilepsy associated with other brain infections, and drug and alcohol abuse.

How can we overcome epilepsy? Clearly, what the medical community and governments have been doing so far has not worked. The International League Against Epilepsy celebrated its centenary in 2009. The WHO has existed for seven decades. Work has definitely been done and some progress made. But it is too little and at a very slow pace. Times have changed, and many newer resources are available. The need of the hour is a paradigm shift in all aspects of epilepsy care.

We need to think about who will shoulder the responsibility of caring for epilepsy patients in communities where there is a shortage of doctors and specialists. Can the epilepsy care workforce be expanded beyond doctors? Can epilepsy only be diagnosed by seating the patient in front of a doctor in a hospital clinic setting, or are there other viable, safe, and acceptable options? Do patients have to be transported to big city hospitals for investigations or can this also be done in heretofore-overlooked settings?2 Once started on treatment, can regular patient reviews only be done in clinics, or can they be done remotely by telemedicine? Untreated epilepsy can no longer be considered just another medical condition. It is a public health issue. Lives are being impacted as adults become unemployable and the caretaker is likewise reduced in their workforce role.

If diagnosing and treating epilepsy remains only an epileptologist’s, neurologist’s, or even a general physician’s brief, then in developing countries like India, untreated epilepsy will never go away. Non-specialists such as paramedical personnel, district health workers, and nurse clinicians need to be trained and enlisted to expand the epilepsy workforce. Technology is now becoming available for various aspects of epilepsy care and can be pressed into action. Evidence is accruing that epilepsy diagnosis by non-physicians using a phone application is possible.3 This can at least be used as a screening tool in remote rural communities. Nurse-led epilepsy clinics may be considered.4 Diagnosing and starting treatment using mobile rural outreach clinics and patient follow-up using the ubiquitous mobile phone are all viable options that hold promise.5,6

Finally, unless efforts at diagnosing and starting treatment are not accompanied by concerted efforts of raising epilepsy literacy amongst patients, caregivers, and laypersons, success will remain elusive. An Epilepsy Resolution approved by the World Health Assembly on the May 26, 2015 is considered a historical landmark. This resolution was overdue and now societies need to push hard to make it work.

Are countries really too poor to bargain for generic drugs for their citizens? The defense budget of India for 2015-16 is $40 billion, and even a 1 percent reduction in this may be enough to treat the currently untreated epilepsy patients for one year. Reducing the country’s nuclear spend marginally or even getting a sliver of funds spent by the Board of Cricket Control in India may be enough for funding epilepsy treatment in untreated patients. Savings can be achieved in developed countries by allowing the treating personnel to use the appropriate generics rather than the cheapest ones, a common conundrum for U.S. physicians, since the cheapest are not always the most effective. This promotes more seizures and more expenses related to caring for the additional seizures and related injuries. A statement has been attributed to former Secretary of State Condoleezza Rice that the U.S. budget for military bands is higher than that for the U.S. State Department. Certainly these funds can be diverted to effective treatments for uninsured U.S. citizens, rather than to ceremony. Even where universal health care exists, there are expenses that can be cut to fund these physician-prescribed needed medications.

References

  1. www.who.int/mediacentre/factsheets/fs999/en/
  2. www.bostonglobe.com/business/2015/05/21/mobile-health-technology-helps-tackle-epilepsy bhutan/c6vmO7XOznyDegIa4BYSAM/story.html
  3. Patterson V. Singh M. Rajbhandari H. Vishnubhatla S. Validation of a phone app for epilepsy diagnosis in India and Nepal. Seizure 30, 2015, 46–49
  4. Paul P. Agarwal M. Bhatia R. Vishnubhatla S. Singh MB: Nurse-led epilepsy follow-up clinic in India: is it feasible and acceptable to patients? A pilot study. Seizure; 2014;23(1): 74-6
  5. Bigelow J. Singh V. Singh M. Medication adherence in patients with epilepsy after a single neurologist visit in rural India. Epilepsy Behav. 2013 Nov;29(2):412-5

Mamta Bhushan Singh, MD, DM is an additional professor, department of neurology, All India Institute of Medical Sciences, New Delhi, India.
 
Michael Finkel is a retired neurologist, and a member of the AAN, Child Neurology Society, ENS, EFNS, and BMA. He is a fellow of the AAN.

 

IN SEARCH OF LOST TIME:
Can We Finally Have a Pan American Neurology?

J.Eduardo San Esteban, MD

J.Eduardo San Esteban, MD

J.Eduardo San Esteban, MD

From Oct. 29th to Nov. 3rd, 2016, Cancun, Mexico, will host the 14th Pan American Congress of Neurology. This will be the second time for the Pan American Congress to be held in this country. Mexico hosted the fourth Pan American Congress in 1975. There have been more than 50 years of Pan American neurology meetings and maybe the time has come to question ourselves as to whether we have made some progress in this continent in building a real Pan American Neurology and more important, how prepared we are to develop an alliance to help each other in the pursuit of improved care of our patients with neurological illness.

The American continent, with a population of around one billion is marked by inequality in human development. We also have a diversity of geographic, economic, political, and cultural systems. And, of course, we also have different health systems to provide care to an ever more demanding population. The obvious differences between the countries north and south of the Rio Grande have provided the grounds for a multinational effort to increase our cooperation. There are social and economic treaties, cultural and professional agreements, some of them with better results than others, but all of them with the idea of finding an improved quality of life. The cooperation among the Latin American states has been also prolific. Despite some differences, our similarities have prevailed, and in multiple examples we can see the results of intelligent and generous collaborations.

In-Search-of-Lost-Time-posterNeurological disease has increased its burden over the world population. Naturally, the Pan American community is no exception. There are some particular situations in different areas— infectious and parasitic disease are still everyday events in some areas, malnutrition and metabolic in others, children with sequelae of difficult pregnancies and deliveries are prevalent in some communities, but the frequent pathologies of the nervous system are similar in all of our countries. Cerebrovascular disease, epilepsy, neurodegenerative disease, developmental difficulties, headaches, and behavioral syndromes have a very similar prevalence. Increases in communication and migration have also contributed to provide a more universal panorama of illness. And, of course the appearance of new threats like Zika virus has swiftly become a multinational preoccupation.

The neurological community has an ideal opportunity to play a significant part in this continental effort. The southern countries have a common language, a very similar cultural background, and a rather equal social, ethnic, and developmental history. We also have a significant increase in technology and communications that have made distances shorter and real-time interaction an everyday occurrence. The northern countries have developed scientific and technological strength that keep them in the frontier of new knowledge. The advance of educational technological facilities have made teleconferences, telemedicine, online courses, and consultations an everyday event.

The question is: Can we, the neurologists of the American continent, build a professional, scientific, social, and educational alliance to help with the possibilities of improving the care of neurological patients around the continent?

What should we do and what can we do to achieve a situation of true and universal cooperation? A north, south, east, and west alliance can be a reality in the fields of education, epidemiology, interchange of information, online consultations, collaborative studies, and so on.

No doubt, the area of neurological education seems the immediate possible goal. There are already multiple programs ongoing. The traditional journey of Latin American professionals to the United States and Canada for an update in the state-of-the-art of neurology, and programs for the neurological subspecialties are a common-day reality. But the extent of these educational efforts hardly meets the needs of a large population of specialists who do not have the opportunities to travel. Well-planned alliances between countries could help to reduce these gaps. Online education is a useful tool. Teleconferences and meetings, and the distribution of information in accordance with a well-planned program that avoids commercialization of information, are all possible ways of collaboration.

The production of human resources for neurological care is another area where alliances can prove beneficial. Based in carefully performed studies that provide information about needs, a combined program of online and onsite education can help to reduce the lack of the most needed professional and technical resources. Even at the level of pre-graduate students, a multinational effort could address the needs of medical schools for a more complete neurological education. Education of first-contact physicians, family and general practitioners, and even paramedical personnel that provide attention in remote areas can be tackled by such an alliance.

Time is short. Population growth is fast, and new challenges wait for us everyday. Maybe the time has come to stop the search for the lost time and start a true alliance of Pan American countries to improve the health of our people who ail from neurological diseases. Maybe, as multinational independent professionals, with no other aims but to help our patients, we can put forth a special effort to develop such an alliance. Maybe we can have a continental Pan American neurology that can help bring the benefits of science, knowledge, and technology to a large population of patients who do not currently have opportunities to enjoy the same care as others. There are more than 30 countries in our continents united by geographical chance, but with the need to be united by more than that.

The forthcoming Pan American Congress in Cancun could be an opportunity to talk about these themes and ideas, and perhaps come up with an incipient program to help us make up the lost time.

J.Eduardo San Esteban, MD is the educational coordinator and former president of the Mexican Academy of Neurology, and director of education of the Neurology Center, American British Cowdray Hospital, Mexico City.

BOOK REVIEW
Duchenne Muscular Dystrophy, 4th Edition

Alan EH Emery, Francesco Muntoni, and Rosaline Quinlivan
Oxford University Press
271 Pages

John F. Brandsema, MD

John F. Brandsema, MD

In the preface of their 4th edition, the authors recall the necessity of a second edition being published only a year after their first in 1987, due to the isolation and cloning of the dystrophin gene and the identification of its protein product. The subsequent 25 years have seen an explosion of research in both basic science and clinical medicine regarding the biochemical underpinnings and the clinical management of Duchenne muscular dystrophy (DMD), a broad field that is expertly and concisely summarized in the current edition.

This trim (roughly 270 pages plus references) and well-written textbook by a highly respected group of neuromuscular clinicians in England is a pleasure to read. The flow is excellent, with little of the repetitiveness or ambiguity of thought that can occur in multi-author endeavors. There is a fascinating exploration of the history of DMD, from the first depictions of what is believed to be muscular dystrophy in Ancient Egyptian hieroglyphics to acknowledgement by the authors of prominent contemporaries in the field today. Complex concepts and topics regarding the biochemistry and genetics of DMD are conveyed in a concise and clear manner, with supportive data largely drawn from the vast clinical experience of the authors themselves.

Book-review-MuscularDystrophyThere were very few aspects of the book that disappointed me as a reader who specializes in care of pediatric neuromuscular disorders, and none were overly striking. While all figures are interpretable regarding what they are meant to illustrate, many of the pathology figures in particular would be much better appreciated in color rather than the black and white version in the text. A color section of the text or an online color supplement would greatly augment the reader’s appreciation of the beautiful examples selected for presentation regarding the pathology of DMD. The recommendation to perform muscle biopsy in every patient for direct dystrophin studies, in addition to molecular genetics, is somewhat strongly worded for the current practice of most of today’s neuromuscular clinics. However, the authors do temper this by emphasizing in other sections of the text the particular populations where this can be especially useful, such as a young patient with an identified dystrophin gene mutation which has previously been reported as having variable phenotypic expressions. It is also easily appreciated that the multi-systemic management issues in older Duchenne patients are complex: in the text, topics such as spinal surgery and cardiopulmonary management are dealt with rather briefly, in contrast to the more extensive discussion of musculoskeletal management of the ambulant child with DMD, for example. These are all minor points, and none was jarring enough to detract from the book’s many admirable qualities.

Overall, this book is impressive in its scope. The timeliness of any textbook suffers somewhat from the time delay related to its publication schedule, but the discussion of therapeutic trials in this text, addressing both genotypic and phenotypic modification of DMD, nicely introduces most of the concepts still actively being studied today. Another particular strength is the unblinkingly honest and insightful discussion of sensitive topics, such as disclosing a diagnosis of DMD, and psychological problems in boys with DMD and their families. These aspects of DMD management are essential to excellent care, but are less often addressed in scientific publications about DMD.

In summary, I strongly recommend this book as a natural addition to the library of any clinician who specializes in treating neuromuscular disorders, and it would be of interest to anyone who may encounter patients with DMD in their practice, including physical/occupational therapists, nurses, genetic counselors, social workers, dietitians, neurologists, physiatrists, geneticists, cardiologists, pulmonologists, orthopedists, and more. As the field of research related to DMD continues to evolve at a rapid pace, hopefully another edition will soon update discoveries and advances.

14th International Congress on Neuromuscular Diseases to Feature the World’s Leading Experts

By Vera Bril, MD

VERA BRIL, MD

VERA BRIL, MD

The 14th International Congress on Neuromuscular Diseases (ICNMD 2016), under the auspices of the World Federation of Neurology, will be held in Toronto, Canada from July 5–9, 2016 at the Sheraton Centre Toronto Hotel. The ICNMD is the formal meeting of the Research Group on Neuromuscular Diseases, formally affiliated with the World Federation of Neurology. It has been 14 years since the ICNMD Congress was held in Canada, with Vancouver, British Columbia, hosting the 2002 Congress.

Building on the success of preceding Congresses, in Vancouver, Istanbul, and Naples, it was decided at the previous meeting in Nice, France (ICNMD 2014) that the Congress be moved to a two-year cycle instead of meeting every four years.

While much encouragement and progress is going on in the field of neuromuscular diseases, especially with the recent support of the ALS Ice Bucket Challenge, the disease remains a huge global health problem.

The Congress will address all aspects from muscular dystrophies, other myopathies, myasthenia gravis, polyneuropathies, spinal cord disorders, and neurofibromatosis, to name just a few of the major themes within the Congress. The Congress will include updates on understanding of the genetics, pathogenesis, evaluation, and treatment of neuromuscular disorders.

The Scientific and Program Committee invited some of the world’s leading experts in the field of neuromuscular diseases, with the hope that at the end of the Congress, attendees will have garnered the most up-to-date information available in neuromuscular disorders.

The Congress is CME Accredited from the University of Toronto. For the total number of credits, please visit the Congress website.

The Program and Scientific Committee has worked tirelessly to plan an exemplary and intellectual program to inspire, educate, and support those individuals and organizations that fight against neuromuscular diseases.

The Congress will unofficially start on Tuesday, July 5 with a full day of Teaching Courses. These courses will be small classroom-sized sessions with a focus on the practical applications and knowledge of specific topics.

An Opening Ceremony is scheduled for Tuesday evening, and from Wednesday, July 6 to Saturday, July 9, the Congress will host workshops that offer multidisciplinary exploration of focused topics related to neuromuscular diseases.

There will be four exciting Plenary Sessions that will feature experts in their fields. Topics include genetics, hot topics, muscular dystrophy, and motor neuron disease.

Toronto is one of the most multicultural diverse cities in the world, where more than 140 languages are spoken, and microcosms of different cultures are thriving in unique cohesion. Only a short distance to world renowned attractions like Niagara Falls and the Canadian wilderness, ICNMD 2016 promises to be a highly rewarding meeting on a social and intellectual basis.

For more details around the scientific program, and registration, please visit the Congress website at http://icnmd2016.org. 

Vera Bril, MD, is president of the 14th International Congress on Neuromuscular Diseases (ICNMD) 2016, professor of neurology, Krembil Family chair in neurology, program medical director, Krembil Neuroscience Program, University Health Network, head of neurology, University Health Network, Mt. Sinai Hospital, deputy physician-in-chief, finance, University Health Network, Mt. Sinai Hospital, Toronto.

 

Neurology International Residents Videoconference and Exchange (NIRVE) Connects Neurology Residents Around the World

By Meah Mingyang Gao, MD; Russell Rasquinha, MD, MASc; Manav V. Vyas, MBBS, MSc; Mary Jane Lim Fat, MD; Yuri Kiryanov, MD; Clecio Godeiro, MD, PhD; Fernando Morgadinho Coelho, MD, PhD; Dalia Rotstein, MD, MPH, FRCPC; Tim Patterson; and Morris Freedman, MD, FRCP

A screenshot of NIRVE rounds in September 2015 — First round for the new cycle (2015-16) where all sites introduce themselves. Top left: Slides being presented at the NIRVE rounds from Toronto. Top right: Trainees and staff at Ufa, Russia; and below, trainees and staff at St Petersburg, Russia. Bottom left: Natal, Brazil, and Sà£o Paulo, Brazil.

A screenshot of NIRVE rounds in September 2015 — First round for the new cycle (2015-16) where all sites introduce themselves. Top left: Slides being presented at the NIRVE rounds from Toronto. Top right: Trainees and staff at Ufa, Russia; and below, trainees and staff at St Petersburg, Russia. Bottom left: Natal, Brazil, and Sà£o Paulo, Brazil.

The 2013 WFN neurology training survey highlighted great variability in neurology training programs across the world.1 Neurology trainees are interested in international clinical experiences, but are often constrained by limited flexible time as well as financial, educational or logistical support.2 With the advent of modern technology, 95 percent of current international neurology trainees have reliable Internet access.1 Video-conferenced medical rounds are a new and proven way to supplement medical education across distances and may represent a sustainable solution to global peer learning.3,4

The Neurology International Residents Videoconference and Exchange (NIRVE) is a resident initiative sponsored by the Peter A. Silverman Global e-Health Program, the Canada International Scientific Exchange Program (CISEPO) and the Baycrest Center for Geriatric Care in Toronto. In 2009, Dr. Dalia Rotstein, a former neurology resident and now faculty member at the University of Toronto, established NIRVE with the vision to connect neurology residents across various geographical sites. NIRVE was modeled on the International Behavioral Neurology Videoconference Rounds.5

Figure 1. 61 distinct round topics (main case and image challenge) at 45 NIRVE rounds

Figure 1. 61 distinct round topics (main case and image challenge) at 45 NIRVE rounds

NIRVE was designed to develop leadership skills and create opportunities for residents at all levels to participate in medical education and peer learning, raise awareness of global health concepts in neurology, increase resident advocacy of global health issues, enhance international and national collaboration among neurology residents and act as a gateway for organizing on-site exchanges.

The participating international sites on a rotating basis host the rounds every first Thursday morning of the month. Residents from the host site present a neurology case through videoconferencing technologies, and neurology resident moderators encourage the audience to actively engage in discussions and exchange opinions in real time.

Since its inception, NIRVE has continued to grow and expand. Neurology residents, fellows and faculty from Toronto with international contacts or affiliations initially recruited partner sites. For example, Olga Finlayson, a former University of Toronto neurology resident, helped establish a lasting collaboration and later clinical exchange with the First State Pavlov University in St. Petersburg, Russia. Our current partner sites include Natal, Brazil; Sà£o Paulo, Brazil; Grenoble, France; Jos, Nigeria; and Ufa, Russia. Participation is free and any site in the world with videoconference technology is welcome to join NIRVE as an equal partner.

Methods

Table 1. Results for educational value of NIRVE rounds in 2015 (N = 25)

Table 1. Results for educational value of NIRVE rounds in 2015 (N = 25)

The rounds start at 8 a.m. Eastern time, with a 30-minute case presentation and a 15-minute “image challenge” focused on a radiological or pathological diagnosis, with accompanying neuro-images. The rounds include sufficient time to engage residents in discussions involving diagnostic steps and therapeutic management across the different international sites. The current video-conferencing equipment (H.323/SIP connection protocol) is free within Ontario. International sites connect through a video conference MCU or bridge (Resolve Collaboration) at an hourly rate of slightly more than $35 per site (sponsored by the Peter A. Silverman Global e-Health Program, CISEPO and Baycrest Center for Geriatric Care).5 As of 2015, webcasting with password protection has been implemented using the Ontario Telemedicine Network, allowing any resident with Internet to connect to our rounds.6

As a resident-led initiative, NIRVE values all input from its participants and actively seeks feedback to further improve the program and curriculum to cater to participant needs. In 2015, a formal survey was distributed to all NIRVE participants and site directors from 2014-2015. The questionnaire consisted of four parts: demographic information, questions on the main case presentation, questions on the image challenge and exchange participation. The survey assessed both qualitative and quantitative responses from the participants and was administered using Survey Monkey®.

Results

More than 100 trainees from 10 different sites have attended NIRVE rounds since its inception. Figure 1 shows the categories and number of presentations in each category that have been covered at the NIRVE rounds, with neurovascular and neuroinfectious diseases being the two most common.

Twenty-seven, or 60 percent, of the 45 trainees across four different sites participated in the survey. The average age of these trainees between the ages of 24 to 53 was 29.4 years, and 16, or 60 percent of trainees, were females. While most were neurology residents at different levels of training, two  sub-specialty fellows, one postdoctoral fellow and two PhD students also participated in the survey. Slightly more than 66 percent of trainees identified languages other than English as the language of instruction in their medical training. Of the 25 residents surveyed, 91.6 percent thought that the rounds were relevant to their level of training, 95.8 percent identified that the rounds contributed to their existing knowledge and 87.5 percent agreed that the topics align with their academic interests (Table 1). Seventy-two percent of trainees were interested in presenting at the rounds in the future, and 80 percent indicated an interest in participating in a future clinical exchange program. Most trainees identified the case discussions as the most beneficial aspect of the rounds and suggestions were made to increase emphasis on global health topics and comparing and contrasting practices across the world.

Discussion

There are considerable variations in the occurrence and management of neurological conditions across the world. As neurology trainees prepare for their future careers in an increasingly globalized world, providing early exposure to a variety of cases and management strategies can be challenging. NIRVE provides an opportunity to fill this gap while fostering a platform for potential collaborations.

Over the past years, NIRVE has encountered considerable challenges limiting its expansion. The difference in time zones across countries, various costs associated with room rental and equipment purchase for some international sites, and English as the main language for the rounds have limited the number of trainees we have been able to engage. Looking to the future, creating a bigger role for webcasting, and password-protected archived webcasts could be a more cost-effective strategy to expand our reach. However, increased connectivity may come at the price of reduced real-time interaction. Finally, an on-site clinical exchange is planned for May 2016 in Toronto, including participants from Brazil, Canada and Russia.

Conclusion

Despite challenges including technological, logistical and language-related constraints, NIRVE rounds continue to supplement resident learning across different geographical, political and cultural backgrounds. We welcome residents and fellows from other programs to contact us at nirve.utoronto@gmail.com for more information about NIRVE or to participate in NIRVE. We are happy to provide further information on some technical requirements and further details.

References

  1. Steck A, Struhal W, Sergay SM, Grisold W and the Education Committee World Federation of Neurology. The global perspective on neurology training: the World Federation of Neurology survey. J Neurol Sci. 2013; 334(1-2):30-47
  2. Lyons JL, Coleman ME, Engstrom JW, Mateen FJ. International electives in neurology training: a survey of US and Canadian program directors. Neurology. 2014; 82(2): 119-25
  3. Ali J, Sorvari A, Camera S, Kinach M, Mohammed S, Pandya A. Telemedicine as a potential medium for teaching the Advanced Trauma Life Support (ATLS) course. Journal of Surgical Education. 2013; 70(2): 258-264
  4. Ricci MA, Caputo MP, Callas PW, and Gagne M. The use of Telemedicine for delivering continuing medical education in rural communities. Telemedicine and e-Health. 2005; 11(2): 124-129
  5. Patterson T, Gouider R and Freedman M. Canada, Tunisia Link up for Long Distance Education. World Neurology. 2012; 27(1): 8
  6. Brown EM. The Ontario Telemedicine Network: a case report. Telemed J E Health. 2013; 19(5):373-6
Meah Mingyang Gao, Russell Rasquina, Manav V. Vyas, Mary Jane Lim Fat and Dalia Rotstein are with the division of neurology, department of medicine, University of Toronto. Yuri Kiryanov is with the department of neurology, First Saint-Petersburg Pavlov State Medical University, St Petersburg, Russia. Clecio Godeiro is with the department of neurology, Universidade Federal do Rio Grande do Norte, Natal, Brazil. Fernando Morgadinho Coelho is with the department of neurology, Universidade Federal De Sà£o Paulo, Sà£o Paulo, Brazil. Tim Patterson is with the department of telehealth, Baycrest, Toronto, Canada. Morris Freedman is with the department of medicine, division of neurology, Baycrest Health Sciences, Mt. Sinai Hospital, and University of Toronto; Sam and Ida Ross Memory Clinic, Baycrest; Rotman Research Institute, Baycrest, Toronto, Canada.