The Diamond and the Rose

By Richard Peatfield

Richard Peatfield

Richard Peatfield

During the last 40 years, the world of headache has been blessed with two remarkable men: Dr. Seymour Diamond and Dr. Frank Clifford Rose. They have both recently published autobiographies. Rose’s autobiography is, of course, posthumous following his death in 2012, while Seymour, just the elder, is still enjoying his retirement at the age of 89.

Although living and working in different environments on different continents, Diamond and Rose have striking similarities over and above their lively personalities. They were born within 16 months of one another of Jewish immigrant stock. Rose’s parents settled in the East End of London after getting married and having two children in Romania. Rose was the youngest of seven surviving siblings.

Diamond’s parents, by contrast, had arrived in Chicago as children from Slovakia and from the Ukraine. He was the youngest of their four children. Both had that combination of inherent talent and industry that enabled them to move out of their original backgrounds.

I think it is fair to say that Rose’s path was advantaged by the grammar school and the university system of his time in Britain, while Diamond grew up in the challenging environment of wartime America. Never is this more clear than in their early clinical training. Rose was able to do all of his jobs within London, whereas Diamond tells the tale of his in-laws driving him, his new wife and all of their possessions from Chicago to Arkansas and then to Ohio every year or so.

Both spent the bulk of their careers as practicing physicians with interests in headache, though Diamond was never a board-certified neurologist as he had been accredited in family medicine and had not done a residency program in neurology.

Both set up dedicated migraine clinics. Rose’s clinic is dedicated to Princess Margaret, while Diamond’s bears his own name. Both wrote and edited a large numbers of books and conference proceedings.

Both were superb administrators and used their talents in a wider field. Rose was the more international, playing a major role in the evolution of the International Headache Society and serving as secretary treasurer general of the World Federation of Neurology.

Diamond, in contrast, devoted much of his energy to the inpatient and outpatient facility he established in Chicago and developed a nationwide reputation as a physician “who cared.” He was one of the first to try tricyclic antidepressants in headache patients and was the leading light in many of the trials of drugs that are currently part of the every physician’s drug armamentarium.

Diamond played a major role in the evolution of the American Association for the Study of Headache and was its chief executive for many years. His autobiography makes it clear that his relationship with the neurological establishment was often fraught. Nevertheless, it is a tribute to his diplomatic skills that the American Headache Society (as it is now called) covers such a broad spectrum of practitioners from neurologists and pediatricians to physiotherapists, psychologists and other colleagues. He also initiated the influential patients’ own National Migraine (now Headache) Foundation.

Rose, in contrast, was more of a neurological polymath, with interests not only in headache but also in motor neurone disease, Parkinson’s disease and stroke. He was perhaps more dependent on his junior colleagues on both the clinical and academic fronts. Diamond’s concentration on one symptom attracted devoted patients from all over the continent. Both were renowned for their approachability.

Diamond’s autobiography was written in conjunction with a medical journalist, in the third person in somewhat more popular style, with many fascinating personal details, including his prowess at Bridge and his innocent dealings with the local mafia. Rose wrote his in the first person, and his voice and characteristic energy come through.

They were both devoted to their wives and children. Diamond and his wife, Elaine, had three girls, of whom one now runs the eponymous Clinic in Chicago after his retirement. Rose and his wife, Angela, had three boys, all established in careers away from medicine.

Both autobiographies give fascinating insights into medical training during and immediately after World War II, as well as into the evolution of the clinical and academic world of headache on each side of the Atlantic. Both are fitting tributes to two outstanding men.

The Headache Godfather: The Story of Dr. Seymour Diamond and How He Revolutionized the Treatment of Headaches By Seymour Diamond and Charlie Morey. Skyhorse Publishing, Inc., New York 2015 ISBN-10: 1629145386 $24.95

Autobiography: By Any Other Name By Clifford Rose Privately printed 2014 [Copies may be available for £12 + p&p. Expressions of interest should be made to]

Neurodevelopmental Disorders in India: From Epidemiology to Public Policy

By Donald Silberberg, MD

Donald H. Silberberg

Donald H. Silberberg

Two remarkable meetings took place in March in Durban, South Africa. On March 25, the National Institutes of Health (NIH) (U.S.) convened a workshop on implementation science, that is, how to use research findings, whether epidemiologic, basic science, clinical or economic studies, to influence public policy. In a previous editorial, I referred to this as “translational research,” the concept of moving from research data to utilization.

Investigators supported by the Fogarty International Center (FIC), NIH, presented the results of studies that are beginning to achieve this, in Burkina Faso, Democratic Republic of the Congo (DRC), Nigeria, South Africa, Uganda and Zambia. Discussion centered on both impediments in achieving implementation, and on what has worked in some locales. All of the presenters and discussants agreed that further efforts are needed to develop approaches that will use research findings to improve neurologic and psychiatric health in every country, and the need to provide this information to all investigators in low- and middle- income countries. Interested investigators should watch for future notices from the FIC.

The NIH workshop was followed by the 12th International Conference of the Society of Neuroscientists of Africa (SONA). In addition to basic and clinical neuroscience presentations by invited speakers from Italy, South Africa, Spain, Sweden, the U.K. and the U.S., investigators from all regions of Africa presented 157 posters and platform talks. At least half of these dealt with clinically relevant topics, ranging from studies of trypanosomiasis in the DRC, to pain following traumatic spinal cord injury in Zimbabwe. The abstracts can be viewed at The next meeting, in 2017, will be held in Uganda. This meeting is clearly designed to include clinical neurologists as well as neuroscientists.



WFN at AAN Congress 2015

By Raad Shakir

WFN/AAN leadership meeting 67th Annual AAN Congress, Washington, DC, 2015. From left to right, Gallo Diop, Ralph Sacco, Terence Cascino, William Carroll, Tim Pedley, Catherine Rydell, Raad Shakir, Riadh Gouider, Steve Lewis and Wolfgang Grisold.

WFN/AAN leadership meeting 67th Annual AAN Congress, Washington, DC, 2015. From left to right, Gallo Diop, Ralph Sacco, Terence Cascino, William Carroll, Tim Pedley, Catherine Rydell, Raad Shakir, Riadh Gouider, Steve Lewis and Wolfgang Grisold.

The AAN is the largest single neurological association in the World Federation. The relationship goes back to the inception of the WFN. The WFN would not have existed without the generous assistance of $126,000 from the National Institutes of Health. This was only possible through the efforts of Dr. Pearce Bailey Jr., the first secretary general of the WFN and the second president of the AAN.

The AAN meeting this year was in Washington, DC, attended by 13,000 with a significant percentage of international delegates. There were 2,678 abstracts. The depth and breadth of the topics and the availability of teaching material is impressive.

Annual meetings of the leaderships of both organizations have become a tradition to discuss bilateral relations and international affairs. This year, there were several issues on the agenda, including joint support for African neurologists and full support for the burgeoning Pan American Federation of Neurological Societies bringing together Latin American neurologists. This organization is to encompass all neurological associations in Latin America and represents the Latin American region as one of six WFN regional organizations.

There was discussion on the creation of a Ted Munsat Award for Training and Education. The late Dr. Munsat was a past president of the AAN and chaired the WFN education committee for many years. There was agreement on creation of the fund, and this will be followed by more detailed discussions in the next few months.

The WFN last year created a regional liaison committee chaired by the AAN President Dr. Tim Pedley. This committee met and heard presentations from the six regions of the WFN. Closer collaboration between regions is the purpose of this committee and this is being achieved.

There is no doubt that neurological needs in Africa are paramount, and this was reflected in the leadership meetings and a special Pan African meeting, which was convened by the AAN and attended by stakeholders, including the leaderships of the two organizations and others including Dr. James Bower, Dr. Farah Mateen (chair, Global Health section) and Evelin Sipido representing the European Academy of Neurology with its special interest in annual courses for African neurologists.

Prof. Gallo Diop (Senegal) eloquently presented the needs and offered solutions with measures to ease the huge deficiency of neurologists in Africa. A tentative plan to train neurologists in Africa was discussed with the aim of opening four training centers, two in Francophone and two in Anglophone countries. Rabat and Cairo are already in operation, to be followed by Dakar and Cape Town.

The long-standing partnership between AAN and WFN continues from strength to strength. The AAN and the publisher donate the hard copies to the WFN, and the WFN distributes the course globally. It is now used in 47 countries. Dr. Steve Lewis is the editor and is a WFN co-opted trustee.

At the end of the meeting, Dr. Terence L. Cascino took over as president of the AAN, and Dr. Ralph L. Sacco was elected as president-elect.

No report on the 67th Annual Meeting of AAN could be complete without a special mention of the erudite lecture given by outgoing president Dr. Tim Pedley (link below). The title was “Moving the Academy Forward: Challenges and Opportunities,” and the talk was mesmerizing. His views on neurology as a nonprocedural specialty were spot on. Although the content understandably relates to the U.S. health care system, there are lessons for us all across the world. Indeed, our spectrum and capabilities have markedly improved but the challenges have also grown. The shortage of neurologists in the face of an aging population with increasing numbers of those affected by neurodegenerative diseases is frightening, and in a way it is now recognized by governments as the World Health Organization has emphasized in a recent meeting on dementia that this condition and its cost will cripple world economies in 30 years.

The close ties binding the WFN and the ANN will only grow stronger over the coming years.



Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: A New Name & Diagnostic Criteria

Maggie McNulty

Maggie McNulty

In March 2015, a report from the Institute of Medicine (IOM) was published in the Journal of the American Medical Association to redefine the illness known as Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS).

Over the years, clinicians and researchers have developed different diagnostic criteria for ME and CFS; however, the two terms describe conditions with similar symptoms. In the World Health Organization’s “International Classification of Diseases,” 10th Revision, both ME and CFS are coded the same and classified as disorders of the nervous system (ICD G93.3). The term “benign myalgic encephalomyelitis” was first used in the 1950s in London when describing an outbreak in patients who experienced a variety of symptoms, including “malaise, tender lymph nodes, sore throat, pain and signs of encephalomyelitis.”

The cause was never found, but it appeared infectious in etiology, and the term “benign myalgic encephalomyelitis” was used to reflect “the absent mortality, the severe muscular pains, the evidence of parenchymal damage to the nervous system and the presumed inflammatory nature of the disorder.” Then in 1970, two psychiatrists reviewed reports of 15 of these outbreaks and concluded that the outbreaks “were psychosocial phenomena” caused by mass hysteria or altered medical perception of the community.

However, the idea that the condition was psychogenic in origin was refuted by Dr. Melvin Ramsay. In 1986, he was the first to publish diagnostic criteria for ME and, at this point, the term “benign” was dropped as the disease often was severely disabling for those patients afflicted. Around the same time, in the mid-1980s, there were two outbreaks of an illness that resembled mononucleosis characterized by “chronic or recurrent debilitating fatigue and various combinations of other symptoms, including sore throat, lymph node pain and tenderness, headache, myalgia and arthralgias.” This illness was at first linked to the Epstein-Barr virus (EBV); however, further research ruled out this as the cause, and, in 1988, the term “chronic fatigue syndrome” was coined by the Centers for Disease Control and Prevention (CDC).

ME/CFS is characterized by symptoms of profound fatigue, cognitive dysfunction, sleep abnormalities, autonomic manifestations, pain and other symptoms that are worsened by any type of exertion. The syndrome affects women more than men with an average age of onset of 33 years with a wide range of distribution ranging from 10 to 77 years old.

ME/CFS is a common disorder that is currently estimated to affect 836,000 to 2.5 million Americans. Despite the prevalence of this disorder, less than one-third of medical school curricula and only 40 percent of medical textbooks include information regarding this syndrome. This likely contributes to delays in diagnosis time for these patients (e.g., 29 percent of patients report symptoms for >5 years prior to receiving a diagnosis), and it is estimated that 84 to 91 percent of people with this condition have not yet been diagnosed.

Table1There is significant economic burden associated with this condition as one quarter of patients are bed- or house-bound at some time during their illnesses. ME/CFS patients have been found to be more functionally impaired than patients with other disabling illnesses, such as diabetes mellitus, congestive heart failure, hypertension, depression, multiple sclerosis and end-stage renal disease. Unemployment rates range from 35 to 69 percent in these patients. ME/CFS patients have loss of productivity and high medical costs that lead to an estimated economic burden of $17 billion to $24 billion yearly.

In general, ME/CFS is a condition that is poorly accepted as its pathophysiological mechanisms are poorly understood, and many contest the characteristics needed to make a diagnosis. There continue to be many misconceptions regarding ME/CFS, including that it is a psychogenic illness. Many times, patient symptoms are met with skepticism or even dismissal. After diagnosis, many people with ME/CFS report being subject to hostile attitudes from their health care providers. The cause of ME/CFS is currently unknown; however, symptoms may be triggered by different infections or other prodromal events, including “immunization, anesthetics, physical trauma, exposure to environmental pollutants, chemicals and heavy metals and, rarely, blood transfusions.”

The IOM was asked by the Department of Health & Human Services, the National Institutes of Health, the Agency for Health Care Research & Quality, the CDC, the Food & Drug Administration and the Social Security Administration to convene an expert panel to review the evidence basis for ME/CFS. This was a comprehensive committee of 15 members that convened in September 2013 and took into account data from patients, clinicians and researchers while also reviewing almost 1,000 public comments. This was followed by a comprehensive literature review to help identify new diagnostic criteria to be used by clinicians. Based upon their work, a new name was recommended to replace ME/CFS. Previous studies have shown that the term “chronic fatigue syndrome” can negatively affect patients and medical professionals’ perceptions of the illness and trivialize the seriousness of the disease. “Myalgic encephalomyelitis” is also inappropriate as there is no evidence of encephalomyelitis in these patients, and myalgia is not a core symptom of this disease. The new name that has been recommended for use is systemic exertion intolerance disease (SEID); this new name is felt to encompass the central characteristic of the disease: the fact that exertion of any kind can negatively affect patients in multiple different organ systems.

A new set of diagnostic criteria was also developed by the group with the intent to ease the process of making a diagnosis of ME/CFS (SEID) and hopefully, decrease the time to make a diagnosis for many patients. The new diagnostic criteria that were developed by the IOM committee are detailed in Table 1. The core features include: fatigue and impairment, post-exertional malaise (PEM) and unrefreshing sleep. All of these features need to be present for one to be diagnosed with ME/CFS (SEID).

Fatigue as defined in the dictionary is “weariness from bodily or mental exertion.” Sufficient evidence has been found that fatigue is profound in ME/CFS (SEID). Dramatic examples of reports of fatigue in patients with ME/CFS (SEID) include feeling “too exhausted to change clothes more than every 7-10 days” and experiencing “exhaustion to the point that speaking is not possible.” More commonly, patients report fatigue as “exhaustion, weakness, a lack of energy, feeling drained and an inability to stand for even a few minutes.” The fatigue must be associated with a significant reduction or impairment in the ability to engage in pre-illness levels of occupational, educational, social or personal activities. This degree of fatigue must also persist for more than six months.

Another core symptom, post-exertional malaise (PEM), is found consistently in patients with ME/CFS (SEID) and felt to help distinguish it from other conditions. A patient’s symptoms worsen after exposure to physical or cognitive stressors that were previously well tolerated prior to onset of the disease. Descriptions provided by patients after an exertional task include “crash,” “exhaustion,” “flare-up,” “collapse,” “debility” or “setback.” PEM may occur within 30 minutes of an exertional task or be delayed up to seven days after an exertional task with the duration of PEM lasting hours to months. In studies comparing ME/CFS (SEID) patients with healthy controls, 86 percent of patients report minimum exercise makes them tired compared to 7 percent of controls. Additionally, 85 percent of ME/CFS (SEID) patients report that they feel drained after mild activity compared to 2 percent of healthy controls.

The third and final core symptom is unrefreshing sleep for which 92 percent of ME/CFS (SEID) patients report compared to 16 percent of controls. The typical sleep-related symptoms described by ME/CFS (SEID) patients include difficulty falling asleep, frequent or sustained awakenings, early-morning awakenings and nonrestorative or unrefreshing sleep (persistent sleepiness despite adequate duration of sleep). Primary sleep disorders such as sleep-disordered breathing, restless legs syndrome and narcolepsy should be considered and evaluated for if clinically indicated as treatment of these disorders can be effective in reducing or relieving symptoms of unrefreshing sleep. However, a polysomnogram is not required to diagnose ME/CFS (SEID). Currently, there is no strong evidence to identify ME/CFS (SEID)-specific sleep pathology despite some studies revealing differences in sleep architecture in a subset of ME/CFS (SEID) patients compared to healthy controls.

In addition, there are two supportive criteria included in the new definition, of which one of two is required to be present to meet a diagnosis of ME/CFS (SEID). These include cognitive impairment and/or orthostatic intolerance. Common features of cognitive impairment that are seen in ME/CFS (SEID) include complaints of problems remembering, difficulty expressing thoughts, difficulty paying attention, slowness of thought, absent-mindedness and difficulty understanding. It is suggested that slowed information processing plays a central role in the cognitive impairment associated with ME/CFS (SEID). This deficit can lead to significant disability that results in loss of employment as well as functional capacity in social environments. Neuropsychological testing has found that patients with ME/CFS (SEID) display deficits in working memory compared with healthy controls with reduced verbal and visual memory being the most consistent finding. The second feature, orthostatic intolerance, refers to worsening of symptoms upon assuming and maintaining an upright position. Symptoms are typically improved but may not be alleviated by lying down or elevating their feet. There is sufficient evidence that indicates a high prevalence of orthostatic intolerance in patients with ME/CFS (SEID) based on bedside orthostatic vital signs, tilt table testing or by patient reported worsening of symptoms with standing in day-to-day life.

The committee also described additional frequent findings found in ME/CFS (SEID) patients, which include pain, immune impairment, infection and miscellaneous symptoms. Pain was found to be common in patients who had ME/CFS (SEID) with complaints of headaches, myalgias and arthralgias being most common. There was sufficient evidence found that supported the finding of immune dysfunction in these patients with data revealing poor NK cell cytotoxicity that correlated with illness severity in ME/CFS (SEID). This finding, however, is not specific to ME/CFS (SEID). Additionally, there was evidence that ME/CFS (SEID) can occur after infection with EBV, but there was not sufficient evidence to conclude that all cases of ME/CFS (SEID) are caused by EBV. Less frequent symptoms that were found in patients with ME/CFS (SEID) include gastrointestinal impairments, genitourinary impairments, sore throat, painful or tender axillary/cervical lymph nodes and sensitivity to external stimuli (e.g., foods, drugs, chemicals).

In summary, ME/CFS (SEID) is a serious, chronic, complex and systemic disease that often significantly limits the day-to-day activities of those affected. It is characterized by a prolonged, significant decrease in function; fatigue; post-exertional malaise; unrefreshing sleep; difficulties with information processing, especially under time pressure; and orthostatic intolerance. A thorough history, physical examination and targeted evaluation are necessary and can be sufficient to make a diagnosis. Despite the high prevalence of this condition with associated high economic burden, little research has been conducted to study the etiology, pathophysiology and effective treatment of this disease. Moving forward, it will be vital to distinguish this disease against other complex fatiguing disorders as the majority of previous research has compared ME/CFS (SEID) patients to healthy controls. Additional research into ME/CFS (SEID) is essential for further progress to be made, and the term “chronic fatigue syndrome” should no longer be used due to the associated stigma, which often precludes patients from receiving appropriate care. It is critical that we do our part to help stop the stigma associated with this condition and provide optimal care of these patients.

Maggie L. McNulty is an assistant professor at Rush University Medical Center, Department of Neurological Sciences.