BOOK REVIEW

By Lawrence W. Brown, MD

In a totally unscientific anecdotal analysis, I have found that child neurologists are the most caring and empathetic physicians of all specialists. Unfortunately, most of us have neither the time, the training nor the resources to provide comprehensive care for all of our patients with severe neurological impairment.

A Life with Grace; Caring for Children Who Have Severe Neurological Impairment goes a long way in showing that it is possible to act collaboratively with parents and other members of the care team in order to provide a humane and realistic approach to intellectual disabilities and other static and progressive encephalopathies. This book may be directed at parents and caregivers, but it strikes a responsive chord in many health care providers like myself who accept and encourage a family-centered approach that redefines palliative care far beyond end-of-life support. Rather, it can aim to ensure the best possible quality of life for the child while accepting that medicine does not have all the answers. And even when we think we know the right approach, it is essential to accept families as partners in the decision-making process.

The most successful chapters are symptom-oriented discussions such as pain, seizures, GI complaints and respiratory problems. It doesn’t matter if the underlying diagnosis is Trisomy 21 or Rett syndrome since identification of a disruptive symptom such as pain and its differential diagnosis is similar in all neurologically impaired children who cannot communicate or even localize the problem. The author is frank about her opinion that favors an empirical approach, which minimizes testing and offers treatment based on likelihood of diagnosis and availability of effective non-invasive interventions. At the same time, Hauer suggests that there is a role for intensive investigations if the symptoms persist despite the first steps.

In a book written to a lay audience, there is a plethora of information including worrisome side effects and drug interactions, but little advice on how to prioritize among choices — while confusing, this is reasonable if one understands that expert diagnosticians must look at the individual child and negotiate the options with skill and finesse. She also offers some practical algorithms for specific symptoms (with validated scales where available), a time frame for the recommended clinical trial, the importance of regular assessment of outcome and a sensible guide to discontinuing medication when the treatment has failed to achieve its goals. Perhaps equally important is the emphasis (both to parents and neurologists) on non-pharmacological approaches from simple comfort measures such as massage, repositioning and reduced total volume with slower tube feeding to alternative strategies such as acupuncture.

Child neurologists may particularly appreciate the discussion that recognizes that almost any CNS symptom (from spasticity to dystonia to seizures) can worsen as a result of any factor altering comfort — pain illness, constipation, disrupted sleep, medication toxicity, etc. It is equally important to recognize that many behaviors that could be seizures in children with severe neurological impairment (even in those with definite epilepsy) have non-epileptic explanations for their arching, posturing, exaggerated startle reactions and staring.

The chapter on seizures is valuable for child neurologists with recommendations for tube-fed children, guidelines for rectal administration of drugs and the restrained use of blood levels. One disappointment is the brevity of the discussion on medically refractory epilepsy in this population. I would have preferred to see more emphasis on surgery since this is still the only real option for cure. Even though limited resection is less likely to be successful in severely involved children, there are many examples of excellent outcomes in selected cases of tuberous sclerosis and cortical dysplasia in patients with intellectual disability and autism. Beyond focal resection, it is equally important in a book emphasizing quality of life to more fully discuss the value of the ketogenic diet, vagus nerve stimulator and corpus callosotomy — all of which can be life-changing in the well-chosen patient.

The least successful chapters come early in the book – what I call Neurology 101. Indeed, it is necessary to review static versus progressive disorders, but to highlight a handful of examples trivializes the complexity of the diagnostic challenges and is confusing in the overlap of distinguishing symptoms. In the era of molecular genetics and pharmacogenomics, it is insufficient to list 12 random genetic disorders or to spend two pages explaining brain pathology in children with all of the conditions that can lead to severe neurological impairment.

There are many valuable points that can help the treating neurologist in areas that are at the edge of his or her knowledge. These include GI issues from the risk of diarrhea from excessive sorbitol in liquid medications to constipation not only as a source of pain, but also as a cause of urinary retention and bladder infections. In addition, there are consequences of delayed GI motility including distension, abdominal pain and chronic diarrhea. There are equally valuable pointers in the respiratory chapter such as techniques to manage dyspnea from stimulation by a fan to repositioning and use of benzodiazepines to reduce anxiety as well as the recognition that treatment of excessive secretions with anticholinergics must balance the goal of reduced secretion with the risk of thicker secretions that are more difficult to mobilize and can worsen constipation.

Particularly helpful for physicians as well as parents are the case examples spread throughout the book. These address the decision points that go into a fully informed plan to address critical issues like whether to perform tracheotomy or spinal surgery, how to manage recurrent respiratory crises and even withdrawal of support respecting life without extending death.

In summary, this is a unique and valuable resource for parents and neurologists alike. It beautifully manages the almost impossible by speaking at a high level without excessive scientific jargon and incorporating a glossary of medical terms for the laymen while including timely and extensive peer-reviewed references at the end of each chapter for physicians.

Brown is at the Children’s Hospital of Philadelphia, Philadelphia, PA.

By Wolfgang Grisold, Walter  Struhal and Svein Ivar Mellgren

European neurology is in the process of increasing harmonization. This is a consequence of the right of European neurologists to practice freely in Europe. This implies practically and scientifically based needs to approach the issue with care and preparation. Presently the UEMS/EBN (European Union of Medical Specialists-Section of Neurology/European Board of Neurology) provides a core curriculum for neurologic training, a definition of the neurology speciality, a training center visitation program and a European Board examination.

The UEMS (www.uems.net) is the union of European medical specialists, and is constituted of representatives from national societies and sections. The European Board of Neurology has representatives from European UEMS members and has biannual meetings. The website of the UEMS/EBN contains information of present activities, including that on examination and visitation of departments. One of the main activities of the UEMS is education. The UEMS/EBN also has provided a core curriculum, a definition of neurology within the UEMS, in addition to the department visitiation program and board examination, and is active in the accreditation of CME activities.

Content of Neurology

The content of neurology training for residents varies in different European countries of Europe.¹ This is not only depending on national traditions, but mainly on the way neurology is practiced and how health system structures are used. In addition, local and national health systems have different professional relations with related overlapping medical fields. Pediatric neurology is in most countries attached to pediatrics. The definition of European minimal standards of content and structures of neurology are available in the UEMS/EBN chapter 6.² The Core Curriculum of Neurology has also been published previously³ and is now (2013) in the process of revision.

Practical skills are trained mostly within the core competencies of neurology such as stroke, extrapyramidal diseases, multiple sclerosis and epilepsy.⁴ Some disciplines, such as clinical neurophysiology, may not be part of the neurology curriculum in several countries, but are still practiced by neurologists or specially trained neurophysiologists (in some countries with a separate specialty in clinical neurophysiology). Skills and particular competences will become more important as future stroke therapies with interventions also need the participation of neurologists.⁵ Rotation and exchange of trainees are also encouraged by the UEMS.

European Board Examinations

Many European sections, like urology, anesthesiology, ophthalmology and others have developed a  European board examination, which in some countries in part or as a whole has replaced the national examination. The attendees of these examinations have to prove that their knowledge is at a European level, and can use the examinations a sign of quality and excellence. The UEMS-SN/EBN decided to develop a European Board examination in 2004, and after several years of development the first examination took place in Milan 2009 during the ENS congress.

The choice of the instruments of the examination is difficult and much debated within the medical disciplines. This concerns the source of questions, the format of the examination, the examination format and the evaluation of results. At present, no universally applicable format of examinations is offered by the UEMS/Council for European Specialist Medical Assessments (CESMA),⁶ and the development of questions often depends on the individual section constructing these examinations according to their needs and requests.

Elements of the European board examination

It was decided that an instrument for reliable and objective testing of skills and practical issues would not be feasible within the neurology examination format, and therefore only candidates were accepted from EU/European Economic Area (EEA) countries, who had already been trained, or were declared qualified for the examination by their national society.  These European candidates are conferred the title “Fellow of the UEMS/EBN.” By 2013 also applicants from non-European countries were admitted to the examination provided they could present proof of similar qualifications.

In more detail, for the European Fellow of Neurology, a three-step approach was designed:

1: The national certification of trainees by their national society is considered as part of the examination. This certificate proves the person to be qualified for the examination. It may be based on a certain number of years of training, a national examination or status as an already certified neurologist. This mode of acceptance would ensure that practical training is confirmed by the national society. This step can practically only be accepted for UEMS states. Also for non-European states, certification of training, or national speciality, or board acceptance need to be provided at application for the UEMS/EBN examination

2: A set of 120 multiple-choice questions (MCQs) with one single best answer has been the main part of the examination. The MCQs were provided by members of the UEMS/EBN, the large European neurological societies (EFNS (European Federation of Neurological Soceties) and ENS (European Neurological Society (ENS), other neurological societies (Movement Disorder Society, European Stroke Society)) and recently also e-brain.

Before the examination all questions were graded independently by an examination board in a specific database. Only questions that obtained an average score over 5 (range 0-10) were accepted. The quality of questions and options of answers were then assessed by members of the Department of Medical Education, Ege University, Turkey, chaired by Prof. Ayhan Caliskan.⁷ This examination quality assurance concentrated on question stem, clarity, language, ambiguousness, and flaws. The final editing of the accepted questions was performed by the chairmen of the examination committee, who also distributed the number of MCQs to different topics.

3: Until 2013, the examinees also had to sit at an oral examination which consisted of four case vignettes with structured questions/answers. The oral examination was replaced from 2013 (examination in Barcelona) by EMQ (extending matching questions).  The major advantage of EMQs over MCQs is that this format not only evaluates knowledge, but also clinical reasoning. The EMQ case scenarios have been developed and written by experts in various topics on a given problem. 100 scenarios were prepared for Barcelona.

The passing limit of the MCQ questions, oral examination, and of the case presentations has been set to 75 percent in the first series of examinations. From 2012 onward, a passing limit was determined by an evaluation panel the day before the examination, based on the Nedelsky and Angoff  methods. This represents an additional effort to optimize the procedures according to educational standards.

Finally, the trainees need to orally present a case, and they thus earn extra points. This is a strictly oral presentation lasting 5 minutes, which is judged by two jurors according to a scheme.

Practical and Financial Issues

In the creation of the EBN examination, several practical questions had to be solved. The organization, including a database of questions, communication and practical aspects of the examination were handled by a professional company, the Vienna Medical University-affiliated Vienna Medical Academy.⁸ The examinations have taken place during the European neurological congresses and the societies (EFNS and ENS) have provided the necessary rooms. This has served the purpose that the examination could be combined with a congress visit. The cooperation with the societies has demonstrated their interest and engagement in the UEMS/ENB and the European board examination. In addition to sufficient rooming also secretarial staff from the ViennaMedicalAcademy have been present, and in addition handled the computer analysis of the MCQs and EMQs. As a routine, a full report of the recent examination is presented to the board of UEMS/EBN with regard to participation, questions, feedback of participants, the examination committee, assistance from the EgeUniversity and ViennaMedicalAcademy, and economical aspects.

In recent years, the development of European board examinations as a sign of quality and toward a move to harmonization has been encouraged. The UEMS group called CESMA (Council for European Specialist Medical Assessments)6 has been established, and has regular meetings to work on the format and quality of European board examinations.

Funding

The UEMS/EBN received initially two grants from the ENS and EFNS as a contribution to develop the EBN examination. All other costs have been covered from the resources of the UEMS/EBN, which has its income mostly from member fees (national societies) and by fees paid by the candidates, which do not cover by far the organizational expenses.

The largest academic input and development was done by volunteers of the UEMS/EBN and many European specialist, who wrote questions, helped to prepare the examination, and also practically participated in several tasks.

Department Visits

Several countries (like Norway) have implemented department visits on a national basis. This concept, also adapted from the UEMS, means a voluntary visit at a training center, both by a representative of the UEMS as well as of the national medical  representation. It is a structured approach to evaluate departments in the sense of equipment, staff and resources, and is based on a questionnaire with assessment of teachers, residents, head of department and also representatives of the hospital staff. The final report analyses the different aspects of teaching and training, and gives recommendations that can be used and should be implemented by the department or hospital.

Subspecialities

Subspecialties develop  in neurology worldwide. In the US, about 25 subspecialties fields have been identified and are recognized by the United Council for Neurological Subspecialties (UCNS), ⁹  which also provides an examination and certification system that is open worldwide. Within European neurology, no formal subspecialities have been identified. A possible step in this direction might be the attempt to create a multidisciplinary approach towards interventional neuroradiolgy by the division of neuroradiology of the UEMS.

Future Aspects

Since the publication of this paper, the EMQs and also the participation of non-European participants have been successfully  implemented.  A possible future cooperation with the Royal College of Physicians (UK), which offers similar examination, is under consideration.

Neurological training is based on a curriculum, on training content, methods, and also finally an assessment and certification. One could argue that structuring an exit examination at the end of training may be counterproductive, as it is too late to correct training or detect deficiencies in the individual trainee. The establishment of a quality circle, which not only evaluates trainees but also the quality of training center, is therefore a further important step in European neurology.

The implicit aspects are that a European examination increases harmonization, and the hope is that many European medical associations partly or as a whole will replace their board examination with this European examination in the future.

Paper published in Eur J Neurol. 2013 Aug;20(8):e101-4. Conflict of Interests: none

Grisold is a neurologist in Austria, chair of  UEMS/EBN examination committee and trustee of the WFN. Mellgren is a neurologist in Norway, co-chair of the UEMS/EBN examination committee, Norwegian delegate and vice president of UEMS/EBN. Struhal is  a neurologist in Austria, active in education in the YNT and EAYNT. He is a member of the education committee of the WFN.

References

1.   Grisold W, Galvin R, Lisnic V et al. One Europe, one neurologist? Eur J Neurol 2007; 14(3):241-247.

2.   UEMS European Board of Neurology http://www.uems-neuroboard.org/ebn.  2012.  Ref Type: Internet Communication

3.   Pontes C. Recommended core curriculum for a specialist training program in neurology. Eur J Neurol 2005; 12(10):743-746.

4.   Struhal W, Sellner J, Lisnic V, Vecsei L, Muller E, Grisold W. Neurology residency training in Europe–the current situation. Eur J Neurol 2011; 18(4):e36-e40.

5.   Flodmark O, Grisold W, Richling B, Mudra H, Demuth R, Pierot L. Training of Future Interventional Neuroradiologists: The European Approach. Stroke 2012.

6.   CESMA Statutes http://www.totbid.org.tr/upload/CESMA%20draft%20statutes.pdf.  2012.  Ref Type: Internet Communication

7.   Caliscan A. – http://ege.academia.edu/ayhan.  2012. Ref Type: Internet Communication

8.   V. M. A. http://www.medacad.org/vma/.  2012. Ref Type: Internet Communication

9.   UCNS http://www.ucns.org.  2012. Ref Type: Internet Communication

Christopher J. Boes, MD

Walter DeWitt Shelden was born Feb. 2, 1870, near Windom, Minn., He grew up in Reedsburg, Wis., and graduated from the University of Wisconsin in 1891.  He attended RushMedicalCollege in Chicago, spent two years as an intern at CookCountyHospital in Chicago and worked as a general practitioner in Reedsburg for four years.  He then received internal medicine training at the University of Vienna for 1.5 years, and for the next 10 years had a lucrative private internal medicine practice in Minneapolis and taught at the University of Minnesota Medical School.  Shelden was asked by William J. Mayo to start the neurology section at the Mayo Clinic in 1913.   He was head of the neurology section at the Mayo Clinic until 1930, and died on Feb. 13, 1946.

Shelden enrolled in courses at the Viennese Medical Faculty as a so-called frequentant (student) during winter term 1901/02, summer term 1902 and winter term 1902/03, staying in Vienna from Sept. 1, 1901, through Feb. 1,1903 (University of Vienna Archives).  Approximately 10,000 Americans took some kind of formal

Walter DeWitt Shelden, MD. Photo courtesy of the Mayo Foundation for Medical Education and Research.

medical study at Vienna between 1870 and 1914.   Almost the entire post-graduate work in Vienna was given in the Allgemeines Krankenhaus (2,250 beds in 1912).

It was written that “here one could do more eye operations, deliver more babies, treat more patients and conduct more post-mortems in a week than would be possible in a year in many parts of the United States (Bonner TN.  American Doctors and GermanUniversities: A Chapter in Intellectual Relations, 1870-1914.  Lincoln:

University of Nebraska Press, 1963).”  Viennese clinicians were quick to encourage and exploit the interest of foreign doctors.  Short practical courses of four to eight weeks were offered in the clinical branches of medicine, each limited to 10 or 12 students.  In addition, adjunct professors and instructors arranged private courses. The internal medicine course given by Kovacs was controlled by Americans through a self-perpetuating membership.

Shelden worked with Edmund von Neusser (internal medicine), Friedrich Kovacs (internal medicine), Lothar von Frankl-Hochwart (neurology), Ernest Finger (syphilology  and venereology) and Alois Monti (pediatrics). Frankl-Hochwart (1862-1914) was in charge of the outpatient unit for neurology and electrotherapy in the medical department at the University of Vienna, and was professor of pathology of the nervous system (although primarily a clinician) (JAMA 1915;LXIV:353; JNMD 1917;45:189-190).  His 1891 monograph Die Tetanie contained the first summary of this disease’s clinical symptoms, and he also wrote important treatises on Meniè
re’s disease and neurogenic bladder (Lesky E.  The ViennaMedicalSchool of the 19th Century.  Baltimore: The JohnsHopkinsUniversity Press, 1976).

According to one of his students, Shelden “spent two years in Vienna, attending presentations of pathologic material and following von Neusser, whom he considered the greatest clinician he had ever known, in the wards of the Allgemeines Krankenhaus.  [Shelden] said von Neusser ‘couldn’t write worth sour grapes,’ that to learn from him one had to observe him examining patients (Mayo Clinic Archives).”

Edmund von Neusser, MD. Photo courtesy of the National Library of Medicine.

Von Neusser (1852-1912), professor of internal medicine at the University of Vienna, was a master of diagnosis and differential diagnosis. Rumor had it that Neusser sent 140 patients to the dead-house without an error in diagnosis (Herrick JB.  Memories of Eighty Years.  Chicago: University of Chicago Press, 1949). Von Neusser had an active interest in diseases of the nervous system, and studied neurology in France, but did not become a specialist in neurology.  He was “master of the whole of internal medicine” (Lesky, 1976).

These trips to Vienna by Americans were criticized by some, but most of this criticism was directed at those without prior internship and practice experience who had short stays, partied too much, did not speak the native language, and/or came after World War I.  None of these things applied to Shelden.  Interestingly, most courses for Americans in Vienna were in English, but Shelden learned German there.  Learning the language was one of the reasons his mother encouraged him to go to Vienna.   Shelden translated German articles for an American neurologic journal later in his career.

Shelden’s training in Vienna honed his clinical skills, exposed him to scientific medicine at the highest level, and had a significant influence on his subsequent medical career.   His student Henry Woltman stated that “when the consultants gathered ‘round the autopsy table in the deadly game of turning face-down on the table a card bearing their name and diagnosis, Dr. Shelden was almost always right,” reminiscent of rumors of von Neusser’s diagnostic accuracy.  Shelden exemplified the benefit of post-graduate training in Vienna before World War I for the well-prepared American physician.

Boes is an associate professor of neurology at the Mayo Clinic in Rochester, Minn.