By Soma Sahai-Srivastava, MD

Soma Sahai-Srivastava

Cambodia is a Southeast Asian country of 15 million people with a per capita annual income of $1,080. For 100 years until 1953, it was a French colony, followed by formation of a kingdom. In 1970, civil war led to the rise of Pol Pot and his Khmer Rouge communist agenda, a classless society with no economy. Under Pol Pot (1975–1979), the educated class was targeted, and 2 million to 3 million Cambodians were executed. Only a handful of doctors remained, as many died or left the country.

In my many trips to Cambodia in the last decade as a solo volunteer, I realized there are no short-term volunteer opportunities for neurology clinical care, unlike other specialties, such as emergency medicine, pediatrics and surgery. No neurology outpatient clinics or inpatient specialized programs existed until recently. There were only three neurologists in the country reported in 2012 (Loo, 2012). Currently there is no adult or pediatric neurology residency program. Pediatric patients with epilepsy, autism or developmental disorders are treated in a tertiary care mental health clinic by psychiatrists. The first internal medicine residency program launched in 2011.

Residents at the University of Health Sciences undergo a two-week hands-on session covering internal medicine, psychiatry and neurosurgery.

I realized that our first priority was clinical education and curriculum development rather than patient care, since neuroscience education at all levels —undergraduate, medical school and postgraduate — is lacking in modern Cambodia. There are only four pathologists in the country; none are neuropathologists. Anatomy teaching does not include 3-D models or cadaver or animal dissection. With the help of an educational two-year grant award from the WFN, a neurology outreach program for Cambodian health care professionals was convened July 25–Aug. 10, 2015, in Cambodia.

Our first goal was the development of appropriate undergraduate and residency neurology curriculum in collaboration with the only government academic medical center, the University of Health Sciences (UHS), which sets the curriculum for undergraduate and postgraduate education in Cambodia for all academic institutions. We met with educational leaders and reviewed the current curriculum, especially in context of a standard Western neurology curriculum. Challenges to establi

At the University of Puthisastra, medical students take part in three days of neurology education.

shing a standard neuroscience curriculum were identified and included lack of cadaver training and neuroanatomists in Cambodia. We decided to establish of two neurology teaching modules for their Train-the-Trainers program— a basic neurosciences module and a clinical neurology module.

Our second goal was to provide workshops on basic clinical neurology skills to health care professionals in all the major cities in Cambodia. We conducted a two-week hands-on session for 50 residents at UHS in Phnom Penh, covering internal medicine, psychiatry and neurosurgery. Tools used for these seminars included PowerPoint presentations, a brain model for basic neuroanatomy, a neurological examination video, neurology tool kits for neurological examination, and pre-test and post-test outcome evaluations. We produced a video on neurological examination and used brain models as a teaching tool. Fifty neurology tool kits were distributed to the residents, the contents of which included a Snellen eye chart, penlight, dermatome chart, tongue blade, reflex hammer, tuning fork and pins. We also taught at the University of Puthisastra, where 150 medical students received three days of neurology education.

Teaching rounds were held for a neurology inpatient team comprising internal medicine residents and faculty from several hospitals, including Calmette Hospital, Kossamak Hospital and Khmer Soviet Friendship Hospital in Phnom Penh. In addition, we visited the Children’s and Adolescent Mental Health Referral Center at Chey Chumneas Hospital in the Takhamu-Kandal Province, where we held a half- day seminar and hands-on teaching session on neurological examination along with distribution of neurology tool kits. In the beautiful colonial city of Battambang, we conducted teaching rounds in the outpatient clinic over two consecutive days for children up to 18 years with neurological disorders, including epilepsy and developmental disorders. This program was held at the Outpatient Free Clinic of the Battambang Catholic Church. We also trained physiotherapists in the department of physiotherapy on basic neurological skills.

We then traveled to Siem Reap, where we met with the Deputy Director of the Apsara Authority, the government agency that supervises all national monuments and will be setting up future workshops at the 400-square-kilometer Angkor Heritage Complex (one of the seven ancient wonders of the world) in Siem Reap for health care professionals.

Some of the lessons learned during our trip:

1. Barriers to global neurology in developing countries include lack of an organized clinical setup for patient care.
2. It is important to understand the current state of neuroscience curriculum to determine the needs.
3. Often one needs to return to the drawing board with basic neuroscience.
4. Initiate clinical education of existing medical docs (our focus: psychiatry, internal medicine and neurosurgery).
5. Developing many levels of peer-to-peer relationships improves networking and learning (e.g., resident to resident, faculty to faculty).

We will return to Cambodia in June 2016 for two weeks with the following objectives:

1. To provide an additional year of continuity to our neurology basic training program and provide neurology toolkits for Cambodian health care professionals.
2. To train Cambodian neurology trainers in two neurology modules: basic and clinical neurosciences.
3. To establish and deliver the first neurology skill lab for international program students at UHS.
4. To establish and deliver a revised curriculum and course syllabus design for a neurology module undergraduate medical students.
5. To develop curriculum for neurology residency training, with the goal of assisting in readiness in establishing the first residency program in the fall of 2016.
6. To develop culturally, geographically and medically relevant assessment exercises that include formative assessment (i.e., pre-test, post-test) and summative exams with rating scores that are consistent with learning objectives.

Ed. David Oliver, Gian Domenico, Wendy Johnston
New York: Oxford University Press
326 Pages, with index

By Colin Quinn, MD

“Nothing we can do.” This is a commonly used phrase when discussing the management of amyotrophic lateral sclerosis with recently diagnosed patients. It is often uttered at a time when patients are at their most vulnerable. They have just discovered they have a relentlessly progressive neurodegenerative disease and simultaneously they are left with the impression that they are on their own because there is “nothing we can do.”

In more than 20 concise and thoughtful chapters, Palliative Care in Amyotrophic Lateral Sclerosis provides an excellent guide for clinicians and patients regarding the particular needs of patients with ALS and their families, from diagnosis to death.

For many patients, the journey with ALS begins at diagnosis, and this is the focus of the opening chapter. As there is not yet a distinct biomarker for ALS, exclusion of other conditions is a critical part of the diagnostic approach, and is well described here. Up-to-date information regarding new genes associated with ALS and new understandings of the pathology behind ALS are briefly discussed.

The chapters which immediately follow include an honest and data-driven discussion covering both the purpose and challenges in delivering palliative care to ALS patients and an approach to breaking the news of an ALS diagnosis.

The subsequent chapters are devoted to a wide range of specific issues regarding the needs of patients, from frontotemporal dementia to frozen shoulder. All chapters provide clinically relevant information (“90% [of patients with ALS] are able to die peacefully”) and detailed descriptions of the variety of approaches to deal with the problems facing this patient population. The tone is nonjudgemental and inclusive of a variety of points of view, particularly regarding physician-assisted suicide and complementary and alternative medicines.

The chapters are written by authors with a depth and breadth of experiences on particular topics. This is demonstrated by a clear understanding of the variety of challenges facing patients and the pros and pitfalls of interventions. One minor criticism of this multiple-author approach is that there is often overlap between chapters and, at times, this means that the information in one portion of the book maybe somewhat inconsistent or incomplete when compared to another section. For example. in Chapter 7 (Control of Symptoms: Dysphagia), scopolamine is the first medication listed as a potential intervention for sialorrhea, however in Chapter 9 (Pain, Psychological distress and Other Symptoms), scopolamine is not mentioned at all.

This one minor critique aside, this should be required reading for anyone involved in the management of patients with this ALS. While there may be no cure for this disease, there is a great deal we can do.

By Orrin Devinsky, MD

Oliver Sacks (left) and Orrin Devinsky.

Oliver Sacks is likely the world’s best-known neurologist. From the early roots of a pure subspecialty in the early 19th century to now, it would be hard to identify another neurologist who has touched as many lives. He did it all outside of the mainstream of traditional academic or private practice neurology. He forged his own path.

Born in 1933 in London, his early life was an intellectual cauldron of family and friends. His mother was a surgeon and anatomist. His father was a general practitioner who taught Sacks that every patient was a special story. His aunts, uncles and cousins included inventors, chemists, United Nations diplomats, Nobel laureates, celebrated cartoonists, directors, writers, etc. His two best childhood friends were Jonathan Miller and Eric Korn. Miller trained as a neurologist, but went on to become an acclaimed opera director, actor, author, television presenter, humorist and documentarian. Korn was a polymath who loved science, theater, poetry and literary criticism. He was an antique bookseller who helped recreate Darwin’s library at Down House.

This idyllic childhood was destroyed by the blitz of London in World War II. Sacks was sent to a boarding school in the country, where a cruel headmaster reigned. He went on to college at Oxford, where he first encountered his literary mentor, W.H. Auden. After medical school at Oxford and an internship at Middlesex Hospital in London, Sacks came to America with his motorcycle. Shortly after arriving, he sent his parents a one-word telegram, “Staying.” In 1965, after completing an internship at Mount Zion in San Francisco and a residency in neurology and neuropathology at the University of California-Los Angeles, Sacks moved to New York, which would be his home until his death 50 years later.

He attempted a career in neuropathology, but clumsy hands held him back, and he moved to his passion, clinical medicine and writing. His first book, Migraine, was published in 1970. He interwove the neurological history of migraine with his case studies and his own migrainous experiences. While working at a chronic hospital in the late 1960s, Sacks cared for patients with postencephalitic parkinsonism, who had been left for custodial care. He convinced the hospital’s administration to give them the chance to be treated with L-dopa. He prevailed, and the complex stories of triumph and defeat remain among the most fascinating and beautifully written case studies in medicine. Awakenings propelled Sacks into a literary arena no neurologist had ever reached before. Twenty years later, Penny Marshall would direct the movie in which Robin Williams played Sacks, and Robert De Niro played one of the postencephalitic patients.

His career continued to rise and through his many books — from The Man Who Mistook His Wife for a Hat to Musicophilia, from An Anthropologist from Mars to Hallucinations, he created a remarkable neuroliterary legacy. In addition, he published more than 50 papers and letters in the mainstream academic literature. His detailed case studies — from achromatopsia to temporal lobe epilepsy — gave new insights to neurologic disease and brain mechanisms. His last year of life was remarkably productive with the release of his memoir, On The Move, and several extended essays in the New Yorker and the New York Review of Books. In his final months of life, he wrote his most provocative and compelling pieces — three essays in the New York Times that revealed his terminal diagnosis and how he planned to live what time remained to him. These essays resonated with readers around the world.

On a personal side, Oliver Sacks loved his patients, family and friends. He understood how to love in a simple and primal way — to see all of nature as alive and interesting and intersecting. Jewish in heritage, his religion was science. His passions were endless — from eating fresh herring and smoked salmon — to venerating colors, such as indigo and orange, and life’s creations, from lemurs and ferns to cycads and sea urchins. In being himself, following his heart, exposing his feelings and thoughts, he made this world richer and more special.

The legacy of a man is impossible for his friends and contemporaries to measure. Yet for Sacks, it is a safe bet that his writings will instruct future generations on how to practice medicine, how to write honestly and boldly, how to see diversity and differences as wondrous, how to live and how to die. Ben Jonson gambled well and said that Shakespeare was “not of an age, but for all time.” Sacks was certainly not of our age — he loved fountain pens and paper, read books and wrote letters, disliked talking on the phone, and felt that the Internet, texting and emailing, for all they had added to our world, also subtracted from the richness of experience and education. Sacks was certainly not of our world. He was more at home in water than on land and preferred reading a dictionary to watching television, and watching a mineral of tantalum and tungsten to reading a novel.

Sacks’ social network of friends, colleagues and correspondents had no clear limits, including from animal people like Jane Goodall and Katy Payne to Nobel laureates in chemistry, physics, and economics. Neuroscience and neurology were his home, and he was close to Stan Prusiner, Eric Kandel, Gerald Edelman, Francis Crick, D. Carleton Gajdusek and many others. With some, like Jun Wada, whom he had not seen in more than 50 years, he continued to correspond. He loved Wada’s letters with their pressed maple and ginkgo leaves. He was friendly with fern enthusiasts and lichen lovers, and with actors like Robin Williams, Dustin Hoffman, and Robert De Niro. He loved the herring mavens of Norway and Iceland and Houston Street’s Russ and Daughters.

Neurology owes a debt to Oliver Sacks. He helped rudder the ship, which was heading on a course of abstraction, of pinpointing the exact location of a problem in the nervous system, oft-times at the expense of caring for patients.

As a medical student in the 1980s, I was attracted to neurology as a way to study the brain and behavior. Yet neurologists were the butt of endless jokes. For stroke, MS, ALS, brain tumors and neuropathy, we could tell you where the problem was and maybe even what caused it, but we couldn’t help the patient. At best, it was said, we could only refer them to physical or speech therapy.

Sacks never accepted this paradigm. Many patients ask doctors what they would do if they were in the patient’s situation. Oliver always observed from the patient’s perspective. In doing so, he gave neurology two priceless gifts. First, he made caring for the whole patient an essential element of practice and showed that it was essential not only for reasons of human dignity but for its therapeutic effect as well. Whatever special insights his education and training brought, he respected the patient and family’s views as equally priceless in understanding illness and people and charting a course of caring.

While I was in medical school researching Tourette syndrome, a pediatric neurologist told me I must read Awakenings — that it was the most informative and insightful account of tics in the neurological literature. I was blown away. My doubts about what neurology could be dissolved. And my understanding of what a physician could be transformed.

One of the greatest blessings in my life has been my friendship with Oliver and what he has taught me. His medical lessons were many: to listen, to hear, to find the story and to see the life; to see yourself as a partner, an explorer and a healer; to doubt accepted answers whenever your mind will let you; to remember the curiosity and dreams that lead you to become a doctor; and to hold that gift for as long and tightly as you can. His life lessons were more profound. His life was infused with the joy and creativity and the simplicity and honesty of a child, and layered on to that was an intensity and depth of knowledge across a dizzying array of disciplines, and an intellectual passion that could charm and amaze in the same moment. Most of us speak and think in single notes; Oliver thought and wrote in chords.

Oliver loved Darwin, and their lives had many parallels. Darwin honed his craft, his theory and his reputation on an eight-year project in which he precisely dissected, classified and redefined our understanding of barnacles while suffering a severe illness with GI symptoms and headaches. Oliver suffered migraine headaches and wrote his first book precisely describing, analyzing and organizing migraines. A few years later Darwin published his major work, The Origin, and Oliver had his, Awakenings. Darwin was a London boy whose first major trip was to sail around the South America on a boat; Oliver left London and flew across North America on a motorcycle. Darwin loved the comfort of his family and world at Down House; Oliver had his in NYC’s west village. Darwin loved the Royal Botanical Gardens at Kew; Oliver, the New York Botanical Garden in the Bronx. Darwin’s mind was unparalleled in churning countless facts into general theories; Oliver’s mind churned science and experience into gorgeous tapestries of humanity.

We must celebrate this man whose playful heart, sweet smile, poetic pen and magical mind will remain a bright star in the neurological sky.

Oliver connected to our world at so many levels, and connected to many other worlds that escape our notice. He heard chords while we barely heard notes, and he let us listen through his ears. He was deeply loved and will be missed terribly.

Figure 1. We recommend a therapeutic strategy based on regular monitoring that aims to maximize lifelong brain health while generating robust real-world evidence.

By Gavin Giovannoni, MD, on behalf of the MS Brain Health Steering Committee.

The goal of treating patients with multiple sclerosis (MS) should be to maximize lifelong brain health. This is the central theme of a new international consensus report from a multidisciplinary author group. Brain Health: Time Matters in Multiple Sclerosis calls for greater urgency at every stage of diagnosing, treating and managing MS. Its recommendations aim to facilitate a therapeutic strategy that minimizes disease activity (Figure 1), involving:

• Shared decision-making
• Early intervention
• Improved treatment access
• Proactive monitoring

The report was launched during a symposium, “Is the MS Community Ready to Promote Brain Health?” on the eve of the European Committee for Treatment and Research in Multiple Sclerosis Congress Oct. 6, 2015.

Professor Tim Vollmer outlined the brain health perspective on MS, presenting recent data underpinning its scientific basis. People with relapsing–remitting MS can lose brain volume up to seven times faster on average than age-matched controls, according to a 2015 meta-analysis of clinical trials.¹ Up to a point, this damage can be compensated for by neurological reserve — the brain’s finite capacity to reroute signals or adapt undamaged areas to take on new functions.^2,3 Preserving neurological reserve protects against cognitive decline⁴ and physical disability progression⁵ in people with MS. Once neurological reserve is exhausted, however, the long-term progressive phase of the disease will be unmasked, Vollmer argued. Adopting a therapeutic strategy that preserves neurological reserve by minimizing disease activity therefore should be of primary concern when people with MS and their clinicians make treatment decisions.

Shared decision-making relies on education. People with MS need to understand the potential long-term effects of the disease as early as possible after diagnosis, stressed George Pepper. As co-founder of the patient forum Shift.ms, Pepper stated that engaging people with MS in their own health care could be “the blockbuster drug of the 21st century.”⁶ He concluded by encouraging health care professionals to help people with MS to take active roles in treatment decisions and disease management, starting at the time of diagnosis.

Early intervention is vital, in order to preserve cognitive function. This was emphasized in a joint presentation by Dr. Gisela Kobelt and Professor Helmut Butzkueven, who examined MS from economic and real-world perspectives. Dr Kobelt presented some of her own economic data demonstrating that the greatest effects of the disease on employment occur at relatively low levels of physical disability.⁷ Professor Butzkueven concurred, and referred to results from a recent study showing that people with cognitive impairment at diagnosis were three times more likely to reach a Kurtzke Expanded Disability Status Scale score of 4.0 and twice as likely to develop secondary progressive MS 10 years after diagnosis.⁸ This underscores the importance of the report’s recommendation of aiming to alter the disease course through lifestyle measures and early treatment with a disease-modifying therapy.

Improved treatment access will ultimately come from demonstrations of cost-effectiveness. Assessing the difference that treatment makes to long-term health and economic burden is of vital importance in a chronic progressive disease. To date, however, long-term economic evaluations largely have been based on models owing to a lack of data, explained Dr. Kobelt.

By monitoring disease activity and recording data in registries, health care professionals can generate robust real-world evidence that can be used to inform individual treatment decisions. Butzkueven demonstrated the power of registry data to compare the effectiveness of different disease-modifying therapies in propensity-matched people with MS.⁹ The overall conclusion was that the biggest change in long-term health and economic outcomes could come from targeted intervention to maximize lifelong brain health — and that real-world registry data should be generated and used to test whether this is the case. (See Figure 1)

Summing up, Symposium Chair Giovannoni stated: “I’d like every clinician, every health care professional, to think that someone with MS is giving us their brain to protect. It’s our responsibility to make sure that they get to old age with a healthy brain, so that they can withstand the ravages of aging. As a treatment philosophy, it’s as simple as that.”

Health care professionals who believe in this treatment philosophy are invited to pledge their commitment to embrace the recommendations of the report. Download the full report.

References

1. Vollmer T et al. J Neurol Sci, 357 (2015):8-18.
2. Rocca MA et al. Neuroimage, 18 (2003):847-55.
3. Rocca MA, Filippi M. J Neuroimagin;17 Suppl 1 (2007):s36–41.
4. Sumowski JF et al. Neurolog, 80 (2013):2186-93.
5. Schwartz CE et al. Arch Phys Med Rehabil, 94 (2013):1971-81.
6. Rieckmann P et al. Mult Scler Relat Disor, 4 (2015):202-18.
7. Kobelt G et al. J Neurol Neurosurg Psychiatr, 77 (2006):918-26.
8. Moccia M et al. Mult Scler (2015): doi:10.1177/1352458515599075.
9. Spelman T et al. Ann Clin Transl Neuro, 2 (2015):373-87.

Acknowledgement

Independent writing and editing assistance for the preparation of this article was provided by Oxford PharmaGenesis, Oxford, UK, funded by grants from AbbVie and Genzyme and by educational grants from Biogen, F. Hoffmann-La Roche and Novartis, all of whom had no influence on the content.