Young neurologists and trainees during the hospital visit at the Istanbul University Cerrahpasa School of Medicine Neurology Department, EFNS-ENS Joint Congress in Istanbul.

ISTANBUL UNIVERSITY CERRAHPASA SCHOOL OF MEDICINE

By Anna Sauerbier, Walter Struhal and Antonella Macerollo

The first Joint Congress of Neurology, combining both the European Federation of Neurological Societies (EFNS) and European Neurological Society (ENS), was held in June in Istanbul, Turkey.

Each year, the European Association of Young Neurologists and Trainees (EAYNT) organizes several activities during the conference to give young neurologists and trainees from different cultural backgrounds all over Europe the opportunity to get together and exchange their individual, professional and personal experiences.

In addition to the lottery, the photo contest and the special EAYNT session, one of the annual highlights of the EAYNT activities is a local hospital visit followed by a get-together. This year’s hospital visit led 90 interested and curious young neurologists and trainees to the Istanbul University Cerrahpasa School of Medicine Neurology department. The visit organized by the Congress President Prof. Aksel Siva and the Turkish Neurological Society.

The participants learned about the general history of the hospital and the educational system in Turkey from hospital residents.

Young neurologists and trainees during their visit to the Istanbul University Cerrahpasa School of Medicine.

In 1933, the Neurology Department was established. This makes it to one of the oldest institutions of higher education in Turkey. The current name Cerrahapasa School of Medicine was introduced in 1963 when the hospital was restructured and an additional Istanbul School of Medicine was founded.

The Istanbul University Cerrahpasa School of Medicine is now one of the three Government Medical Universities in Istanbul and one of the main medical schools in Turkey offering Turkish and English Medical programs. The Neurology Department consists of 36 adult and six children beds. In total, six consultants, 16 residents, seven fellows and 24 master students in electroneurophysiology work in the department.

The undergraduate (medical school) training is followed by a minimum of four years of residency training in neurology, which includes three-month rotations in each neurology division, and laboratories and short periods in different other specialities. In addition, numerous graduate and postgraduate educational programs are available at the Istanbul University Cerrahpasa School of Medicine Neurology Department.

Due to the large variety of divisions and units covering the complete field of neuroscience complemented by a multidisciplinary approach, the Neurology Department is able to provide comprehensive services for the diagnosis, treatment and care of patients with a wide range of neurological conditions. Further, it is one of the most esteemed centers for research and education in Turkey.

The young neurologists and trainees then enjoyed a guided hospital visit through the Neurology Department where each unit’s health professional welcomed them and explained their field of expertise. The divisions in the Neurology Department include Clinical Neurophysiology and Epilepsy, General Neurology, Pain Disorders and Headache, Neuromuscular Disorders, Neuroimmunology/Multiple Sclerosis, Sleep Medicine, Paediatric Neurology, Vascular Neurology and Movement Disorders.

This allowed the participants to discover the Clinical Neurophysiology/EMG unit where more than 3,000 patients are investigated annually. In addition, more than 500 patients are followed up for various neuromuscular disorders and more than 300 patients for botulinum toxin injections.

Afterward, the neurologists visited the epilepsy center with an electroencephalography laboratory where annually more than 2,000 patients referred from all over Turkey are seen, and more than 6,000 patients are followed up with different epileptic disorders.

At the end, Siva invited all participants to a dinner and get-together at the restaurant of the Istanbul University. Besides this profound insight into the clinical practice and the education system of neurology in Turkey, the 90 impressed young trainees and neurologists were able to experience another important aspect of the Turkish culture by tasting the delicious flavor of original Turkish food ranging from different forms of kebab, kofte, dolma, borek, mezes and other lovely desserts.

Acknowledgment: The EAYNT is grateful to Siva and the Turkish Neurological Society for their friendly invitation and excellent organization of the hospital visit. In addition, we would like to thank Siva and the team of the Neurology Department for their kind provision of the presentations and pictures during the hospital visit.

Delegates and staff of the Dakar Conference on the formation of CAA. Paul Kioy, Anthony Zimba, Gallo Diop, Lionel Carmant, Calixte Kuate, Sokhna Ba, Mansour Ndiaye, Mareme Sene, Nico Moshe, Late Bryan Kies, Birinius Adikaibe, Emilio Perruca, Pierre-Marie Preux, Baba Koumare, Sammy Ohene, Amara Cisse, Michel Baulac, Sam Wiebe.

By Dr. Ezeala-Adikaibe Birinus

The enormous challenges facing epilepsy care in Africa, especially in poor and rural areas, cannot be overemphasized. All human development indicators, despite some improvement, remain low and unacceptable. Faced with other pressing issues and social conflicts, bringing epilepsy to the forefront has been an uphill task. In recent years, the number of training institutions for doctors and nurses has increased and more qualified personnel in the area of neurological disorders have been trained. The number of diagnostic equipment and specialist centers for neurological diagnosis has grown. However, for a reasonable impact to be made, efforts geared toward increasing awareness, advocacy, reducing the price of medications and improving access to care and research to a more coordinated approach are required.

To achieve these aims, the International League Against Epilepsy (ILAE) set up the Commission on African Affairs in 2010 in Dakar, Senegal.

The official inaugural meeting of the African commission of ILAE took place in November 2010. The parent body, ILAE, convened, and Prof. Amadou Gallo Diop of Senegal and Senegalese League Against Epilepsy served as a facilitator.

Representatives from the following countries were present: Dr. Calixte Kuate-Tegueu (Cameroon), Dr. Sammy Ohene (Ghana), Prof. Amara Cisse (Guinea), Prof. Paul Kioy (Kenya), Prof. Baba Koumare (Mali), Dr. Birinus Ezeala-Adikaibe (Nigeria), Prof. Amadou Gallo Diop (Senegal), Dr. Brian Kies (South Africa) and Dr. Angelina M Kakooza (Uganda).

ILAE delegation was led by Prof. Solomon Nico Moshe (President, U.S.), Prof. Emilio Perucca (Treasurer, Italy), Prof. Sam Wiebe (SecretaryGeneral, Canada), Prof. Michel Baulac (Second Vice President, France) and Prof. Lionel Carment (Canada). Invited observers were Prof. Alfred Njamnshi (President of Pan African Association of Neurological Sciences, Cameroon), Prof. Pierre-Marie Preux (Tropical Neurological Institute of Limoges, France) and Dr. Anthony Zimba (IBE Africa Commission, Zambia).

Prof. Mansour Ndiaye, head of the Department of Neurology of the University of Senegal, read a welcome address. Prof. Solomon Moshe followed and talked about the history of African Commission, including failures and challenges. The present and past efforts of the country’s Leagues Against Epilepsy were discussed in presentations.

Carmant, Wiebe and Preux made presentations, showing the great opportunities and prospects of working as a team to develop the African Commission. It was noted that a lot of work has been done or is presently going on in various parts of the continent, but there is a need for proper coordination and collaboration.

The second day of the meeting was dedicated to the formation of the commission (ILAE-CAA). The executive members of ILAE emphasized the benefits of working as a team and the successes achieved in other regions of the world and the scope of the future CAA based on ILAE bylaws.

Moshe encouraged the African Commission to move forward and work as a team despite the envisaged challenges. He urged them to call on the parent body for help when the need arises. He said the North American Commission is looking forward to building a partnership in Africa to promote the treatment of epilepsy and research into newer epilepsy syndromes.

Later in the day, the potential members of CAA discussed and elected the officers that will run the commission until 2015. The officers were selected (see below) and were later endorsed by the international executive. Further work was done in setting out the commission’s Action Plan for 2011-2015.

Delegates and staff of the Dakar Conference on the formation of CAA are Paul Kioy, Anthony Zimba, Gallo Diop, Lionel Carmant, Calixte Kuate, Sokhna Ba, Mansour Ndiaye, Mareme Sene, Nico Moshe, Late Bryan Kies, Birinius Adikaibe, Emilio Perruca, Pierre-Marie Preux, Baba Koumare, Sammy Ohene, Amara Cisse, Michel Baulac, Sam Wiebe.

The commission’s task was to consolidate gains and create awareness of epilepsy and related disorders. Every avenue should be used, including newspapers, radios, television and community-based programs.

Goals of the Commission

• To set up the organization of the newly formed Commission on African Affairs (CAA)
• To strengthen the communication and ILAE global outreach campaign of the CAA
• To establish and strengthen the education activities of the CAA
• To improve the access to care for patients with epilepsy
• To establish and coordinate epilepsy-related research activities in the African continent
• Work with pharmaceutical companies on programs that at term will help to lower the cost of main drugs and provide better access to care for people with epilepsy in Africa
• Provide the list of epilepsy training centers in Africa and organize the visiting professorship in these centers
• Set up biannual training courses in the two main foreign languages used in Africa: French and English

Achievement of the Commission 2010-2014

• 2011: participation in the meeting of the task force on distance education (Brussels, Belgium)
• Commission meeting. Since its inception, the commission has had three meetings: Rome (2011), Nairobi (2012) and Montreal (2013). All meetings were held either during the International Epilepsy Congresses or African Epilepsy Congress
• Organization of the first African Epilepsy Congress (June 21-23, 2012, in Nairobi, Kenya)
• Organization of the second African Epilepsy Congress (May 22-24, 2014, in Cape Town, South Africa).
• Organization of regional training courses. (June 20, 2012, during the first African Epilepsy Congress in Nairobi)
• Enhancement and promotion of the use of online training courses for health workers in the continent (VIREPA courses)
• Increase in the number of chapters from 12 to 16. Burkina Faso, Cote D’Ivoire and Congo Democratic Republic were re-activated
• Publication of the regional newsletter

Work in Progress

• Epilepsy training courses will be organized in French, English and Portuguese.
• Provide the list of epilepsy training centers in Africa and organize the visiting professorship in these centers.

Consistency and feasibility of ideas remain the goal of commission.

(ILAE/IBE member countries: Burkina Faso, Cameroon, Congo, Congo Democratic Republic, Ethiopia, Gambia, Ghana, Guinea, Kenya, Malawi, Mali, Mauritius, Namibia, Niger, Nigeria, Senegal, Sierra Leone, South Africa, Swaziland, Tanzania, Togo, Uganda, Zambia and Zimbabwe)

Wolfgang Grisold

By Wolfgang Grisold

At this unique meeting organized by the European Federation of Neurological Societies (EFNS) and the European Neurological Society (ENS), more than 5,900 neurologists from 102 countries attended. The top countries included France, Germany, Greece, Italy, Romania, Russia, Spain, Switzerland, Turkey and the United Kingdom. But, of course, many participants came from outside of Europe.

From a total number of 2,700 abstracts submitted, the Congress Program Committee led by Prof. Jacques De Reuck and Prof. Gustave Moonen selected 1,500 to be presented as electronic and paper posters. The ePoster session was a great success.

There were 68 sessions, including eight symposia, focused workshops, oral sessions and special sessions. The education program consisted of 25 teaching courses and three practical courses.

During the lunch breaks and in the evening, pharmaceutical companies organized 11 satellite symposia.

Similar to previous congresses, young scientists had the opportunity to compete in the Tournament for Young Neurologists. The Subspecialty Scientific Panels chose the best presentation in their field. All the participants were winners, since the congress evaluations showed that the scientific program as well as the organization was excellent.

The highlight of this congress was the foundation of the European Academy of Neurology (EAN). EFNS and ENS came together to found this new society. Currently, 45 national neurological societies and 800 individuals are registered members of EAN. EAN represents more than 19,000 European neurologists.

The Assembly of Delegates elected its Board on June 3. The following officers were elected:

President: Günther Deuschl, Germany

Vice President: Prof. Franz Fazekas, Graz, Austria

Secretary General: Prof. Didier Leys, Lille, France

Treasurer: Prof. Marianne de Visser, Amsterdam, The Netherlands

Chair Scientific Committee: Prof. Antonio Federico, Siena, Italy

Chair Liaison Committee: Prof. David Vodusek, Ljubljana, Slovenia

Member at Large: Prof. Per Soelberg Sorensen, Kopenhagen, Denmark

The first EAN Congress is scheduled to take place June 20-23, 2015, in Berlin.

Birgit Surböck

The First Joint Congress of the ENS and EFNS took place in June 2014 in Istanbul.

Peripheral Neuropathies

Rudolf Martini, Würzburg, Germany, was the first speaker of this session and talked about opportunities for treatment of CMT diseases. At the moment, there is no cure for these genetic neuropathies. Studies (mouse and human) with many substances studied, such as ascorbic acid, progesterone antagonist, curcumin, neurotrophin 3, carried out without success. In the German laboratory, mediators for the involvement of phagocytosing macrophages have been detected in the demyelination and perturbation of axons. These mediators are monocyte chemo-attractant protein-1 (MCP-1; Ccl2) and colony-stimulating factor-1 (Csf-1). The idea was that attenuating macrophage-related peripheral nerve inflammation could be a putative option to ameliorate disabling symptoms associated with CMT-1.

Alexander Tinchon

Phase 1 clinical trials with a highly selective Csf-1-receptor inhibitor were promising. Another approach injected human adipose-derived mesenchymal stem cells (MSCs) isolated from lipo aspirate into tail veins of Cx32-deficient mice, a model for CMT-1X. Single injection of these immune modulatory xenografts caused macrophage attenuation and mild preservation of myelin.

Next, Rayaz A. Malik, Manchester, United Kingdom, presented on diabetic neuropathy. The problem in finding the optimal tools for diagnosis and treatment is the design of studies. What should be used for primary endpoint? Clinical status, vibration threshold, electrophysiological findings?

Leyla Alpaslan

Often, there is only an evaluation of the Aβ-fibers and not C-fibers, which are responsible for pain, skin blood flow, inflammation and ulceration. An alternative diagnosis tool to skin biopsy could be corneal confocal microscopy, as there are more than 7,000 nociceptors per mm2 in the cornea. In studies, the loss of corneal C-fibers was in concordance with the progression of the neuropathy. Malik concluded with the statement: “Look into my eyes and predict my risk of amputation.”

Pieter A. van Doorn, Rotterdam, discussed how to optimize treatment in immune-mediated neuropathies. About one-fourth of patients with GBS develops respiratory insufficiency, and many have signs of autonomic dysfunction and pain. Prognostic models can help to predict the chance that an individual patient will require artificial ventilation, and to predict the probability to walk unaided after half a year. EGRIS score and mEGOS are tools that can be used early in the course of disease.

Sabrine Pollanz

Treatment is well known with intravenous immunoglobulin (IVIg) or plasma exchange. Important to consider that about 10 percent of GBS patients will have a treatment-related deterioration (TRF), requiring a repeated treatment course. Other patients initially diagnosed as GBS will turn out to have acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP). Treatment of CIDP include steroids, IVIG and plasma exchange. Most patients need intermittent maintenance treatment.

The session ended with an overview of treatment in amyloid neuropathy (AN) from David Adams, Kremlin-Bicàªtre, France. As AN is a systemic disease, patients should be screened for cardiologic, renal and ocular manifestations in transthyretin FAP (TTR-FAP) or hematologic involvement for light-chain amyloidosis (AL-amyloidosis).

Anna Grisold

To remove the main source of variant TTR, liver transplantation is performed depending on the stage of the neuropathy, the variant TTR, the age of the patient and severity of organ involvement. Liver transplantation, which has been performed since 1991, has better results in early onset. Pacemaker implantation should be discussed in case of significant conduction disorder. Heart or kidney transplantation must be discussed in end-stage cardiac or renal failure in Stage 1 neuropathy.

For light chain amyloidosis, chemotherapy, in combination with high dose dexamethasone, is used to control the underlying plasma clone that produces amyloidogenic light chain. Another option is the application of monoclonal antibodies against human serum amyloid P component. At the moment, a Phase 1 study is ongoing in the U.K.

Chemotherapy-Induced Neuropathies

Prof. Cavaletti, Italy, talked about chemotherapy-induced neuropathies (CIPN), which present an increasingly clinically relevant issue. Due to improvement and extension of chemotherapies, the overall survival rate of tumor patients is rising, though the number of the exact incidence is lacking.

However, neurotoxic drugs with substances such as platin derivates, taxanes, vinca-alcaloids, bortezomib and thalidomide are becoming a major dose-limiting factor. The epidemiology is still unclear.

CIPN are dose-dependent and occur after a cumulative dose, mostly after three to four cycles.

CIPN are predominantly sensory with dysaesthesia, paraesthesia, burning sensations, pain, ataxia and gait disorders. Motoric or autonomic, as well as, cranial nerve involvement is rare. Oxaliplatin should be highlighted, as it induces acute transient side effects as coldness-associated pain, and muscle cramps especially in the jaw and bulbar distribution.

An important issue is “coasting.” Patients treated with platinum derivates may develop worsening neuropathic symptoms after treatment has ceased.

For the clinical involvement and evaluation of CIPN, questionnaires are used, such as the Total Neuropathy Score (TNS) or the National Institute Cancer Toxicity Scale (NCI-CTC). The sensitivity is limited due to interindividual reliability.

Measurement of sensory and motor nerve conduction velocity (NCV), sensory nerve action potential (SNAP) and compound muscle action (CMAP) together with electromyography (EMG) are standard neurophysiological tests used. Diminished amplitude of SNAP in NCV is thought to reflect axonal loss from sensory nerves. In case of inconclusive results, a skin/nerve biopsy may be considered.

Pre-existing neuropathies, due to diabetes, alcohol abuse or hereditary neuropathies as well as concomitant chemotherapies may facilitate or worsen CIPN. However, the correlation remains unclear.

Neuroprotectives are still lacking. Several substances have been tried but without success. Symptomatic treatment targeted with either neuropathic pain therapy or physical therapy and rehabilitative measures must always be considered.

In summary, CIPN present a serious adverse effect due to limitation in quality of life. Studies with the evaluation of biomarkers to identify patients with the high risk of developing CIPN are needed. Data of long-time effects are lacking. Further studies are needed to achieve a standardized treatment of CIPN and its complication.

Movement Disorders

Oral sessions and teaching courses were provided throughout the congress, spanning all relevant topics from idiopathic Parkinson’s disease to hyperkinetic movement disorders. The first two days were dedicated to Parkinson syndromes in general and organized as oral sessions focused on novel diagnostic and therapeutic strategies. The first session included an interesting prospective cohort study, which explained the potential meaning of a particular olfactory assessment in early diagnosis of Parkinson’s disease in patients with rapid eye movement sleep behavior disorders.

The use of apomorphine was highlighted as good therapeutic option in patients with morning akinesia and reviewed as a reasonable drug for improved quality of life in a multicenter prospective French trial.

Atypical Parkinson syndromes were discussed in a separate session, underlining autonomic disorders as a leading symptom in the diagnosis of MSA. An extrapyramidal syndrome with rapid progression, poor response to L-Dopa and other uncommon symptoms such as early dementia, apraxia or stridor should be considered as an atypical Parkinson’s syndrome. Stridor in particular seems to be an underestimated symptom in this differential diagnosis. On the other hand, fragile-X-associated tremor-ataxia-syndrome was introduced as a rare but possible differential diagnosis in patients with atypical Parkinson syndromes.

The therapeutic approaches on MSA are still limited, since rasagiline, lithium and fluoxetine failed to prove a significant benefit. Although accompanied by a poor prognosis, MSA should be considered as a heterogeneous disease with a challenging effort in diagnostic and therapeutic efforts.

A highlight of this year´s congress was the interactive sessions in which patient videos were presented, and the audience could vote among several differential diagnoses. In the session “paroxysmal events” intermittent movement disorders, as the paroxysmal kinesigenic dyskinesias (PKD) with sudden attacks of involuntary movements, including dystonia, chorea, athetosis or ballism, precipitated by sudden movements were shown. Also presented, patients with paroxysmal non-kinesigenic dyskinesias (PNKD), that usually occur spontaneously or may be triggered by consumption of alcohol or caffeine, and the paroxysmal exercise-induced dyskinesia (PED).

Examples of faciobrachial dystonic seizures, nocturnal frontal lobe epilepsy and L-Dopa-induced dystonic-ballistic dyskinesias in Parkinson’s disease demonstrated the difficult differentiation against psychogenic movement disorders.

Improving Outcomes of CNS Infections and Autoimmune Encephalitis

Diederik van de Beek, Amsterdam, presented the first session about bacterial meningitis. He presented the dilemmas in the diagnosis of acute community-acquired bacterial meningitis and focused on strategies to optimize antibiotic efficacy in view of increasingly drug-resistant bacteria. He also discussed the role of current and future adjunctive therapies. Clinical data to support new antibiotics in the treatment of multidrug-resistant bacteria are scarce.

Whether adjunctive anti-inflammatory therapies (e.g. dexamethasone) improve outcomes in patients with bacterial meningitis remains controversial and are being tested further. In the European clinical trial from 301 adults with meningitis who started dexamethasone 40 mg/d for four days before/with first dose antibiotics, it decreases mortality and hearing loss. Another adjunctive therapy, hypothermia, tested by a randomized open-label, blind endpoint trial has no beneficial effect. Rapid diagnosis and treatment reduces mortality, therefore it should be started simultaneously with an adjunctive therapy.

Johann Sellner, Austria, then talked about strategies to improve the outcome of viral encephalitis. Early suspicion and diagnosis are crucial. A delay of more than two days between admission to the hospital and antiviral treatment has a poor outcome. The clinical spectrum of presentation is broad and leads to misdiagnosis such as altered mental status, sepsis and seizures. Herpes simplex virus (HSV) and varicella zoster virus (VZV) are most commonly involved in sporadic disease, while in about one third of the patients the agent cannot be identified despite extensive diagnostic efforts.

The correct dosage of acyclovir is given in 75 percent of cases and should be IV 10 mg/kg every eight hour for a period of 14-21 days. In cases of negative PCR and no alternative diagnosis by suspected HSE, the duration of therapy is for 10 days.

M. Titulaer, Barcelona, Spain, then discussed autoimmune encephalitis . He mainly focused on anti-NMDA receptor encephalitis, the clinical picture, treatment and outcome. Clinical symptoms, abnormal CSF with lymphocytic pleocytosis, raised total protein and oligoclonal bands; abnormal EEG and MRI are essential for the diagnosis. After a prodromal phase, the clinical deterioration starts with symptoms of agitation, psychosis, catatonia, memory deficit, speech reduction, abnormal movements and seizures to coma, hypoventilation and dysautonomia.

Other types of encephalitis as LGI1-encephalitis and its clinical symptoms such limbic encephalitis, memory loss, myoclonic-like movements, hyponatremia, seizures and its outcome were presented and discussed.

Uncommon Causes of Dementia

Philip Scheltens, Amsterdam, opened with comments about prevention for Alzheimer’s disease. It is a challenge not only for neurologists but also for all health care systems in Europe because dementia will become the most significant brain disorder in the next 30 years.

Obesity and smoking increase the risk of developing dementia, while physical activity and moderate alcohol consumption decrease the risk. Consequent blood pressure treatment is successful in prevention whereas statins and vitamin B12 substitution showed no effect. A current ongoing trial is the pre-DIVA (Prevention of Dementia by Intensive Vascular Care) study with 15,000 person years. It will be completed in 2015.

Protective genes are identified – for example, APOE 2 and APP mutation A673T.

Antiamyloid strategies with immunotherapies (bapineuzumab, gantanerumab), anti tau therapies, neuroprotection and dietary interventions are subjects of studies and are expected soon.

Jonathan Schotts, London, presented on the connection between dementia and immune mediated syndromes. Whereas patients with degenerative dementia are older and show slow progression with “little neurology” as well as atrophy in MRI-scans, autoimmune dementias occur in young/middle-aged patients with sub-acute onset. Symptoms include confusion and delirium. Progression is rapid and MRI shows signal changes. Classical antibodies are anti Hu, Ma1+2, amphiphysin, in the last 10 years, antibodies directed against the voltage-gated potassium channel complex have been detected, which can produce a sub-acute and potentially treatable limbic encephalopathy usually in the absence of an underlying tumor.

Subsequent studies have defined specific antigenic targets (LGI1, CASPR2 and contactin-2) within the potassium channel complex. Facio brachial dystonic seizures precede the development of cognitive decline and are immunotherapy responsive. As a consequence, the treatment may prevent cognitive impairment. Other rare antibody-mediated encephalopathies, including those associated with antibodies directed against NMDA, AMPAR, GABA-B, GAD and Glycine receptors, are now recognized.

Sandro Sorbi, Florence, talked about causes for rare dementias. An overlap between uncommon dementias and young-onset dementia can be derived from the epidemiological data. This could be young-onset forms of common neurodegenerative dementias such as familiar Alzheimer’s disease, dementia associated with other neurological disorders (Huntington’s disease, myotonic dystrophies, autosomal dominant cerebellar ataxia or hereditary spastic paraparesis) or late-onset forms of childhood conditions, such as mitochondrial disorders, lysosomal storage disorders and leukodystrophies. Inflammatory disorders and infectious or toxic-metabolic abnormalities also can be the causes of rare dementia. Clinical data are not sufficient. Most of them are based on single case reports.

Murat Emre from the host city Istanbul, closed out the congress talking about Parkinsonism associated with cognitive impairment. Two forms can be discriminated dementia with Lewy Bodies (DLB) and dementia associated with Parkinson’s disease (PD-D). There also are mixed forms such as “Lewy-body variant of Alzheimer’s disease.” In both biochemically a cholinergic deficit exists, which can be demonstrated in autopsy and PET studies. For this reason, cholinesterase inhibitors have been shown to provide some benefits in both conditions. Clinically, patients show decreased performance in executive functions, visual spatial functions and present with hallucinations. There are more similarities than differences in these two syndromes.

Ryuji Kaji MD, PhD

In his 2010 inauguration speech as the president of World Federation of Neurology (WFN), Prof. Vladimir Hachinski conveyed a message: “Asia has more than 60 percent of the global population, yet in some areas, the education of neurology to young neurologists does not keep up with the patients’ needs of neurological care.” He organized the Asia Initiative as a part of WFN to bring attention to this region. As the chair of this initiative, I have met many Asian neurologists and have begun to realize that there are unique problems that require attention from the rest of the world.

The 14th Asian & Oceanian Congress of Neurology was held in March in Macao, China. This is an official meeting of Asian Oceanian Association of Neurology, whose history dates back almost half a century ago. Charles M. Posner, a WFN representative, toured the Asian and Oceanian countries and challenged local neurologists to form an association that would promote and foster the advancement and exchange of information within the area. In response, Shigeo Okinaka, then the executive of Japanese Society of Neurology, invited the region’s neurologists to a planning meeting in Tokyo. This gave birth to the Asian and Oceanian Association of Neurology (AOAN) on June 26, 1961. Its official meeting, Asian & Oceanian Congress of Neurology (AOCN), had been held every four years until the current 14th meeting in China, which followed the 13th meeting in Melbourne in 2012 by two years. There was uncertainty over the financial and scientific outcomes of this short-interval meeting, but thanks to the dedication of the Hong Kong Society members, it became an unprecedented meeting with the largest number of international participants ever. Its surplus funds should significantly contribute to the activities of AOAN, chaired by Dr. Man Mohan Mehndiratta from India. The next AOCN will be held in 2016 in Malaysia.

On March 4, there was a plenary session on “Special Issues in Asia,” in which three speakers spoke. Prof. Chong-Tin Tan from Malaysia gave a talk on education in Asia. He serves as the editor of Neurology Asia, the official journal of AOAN and Association of Southeast Asian nations Neurological Association (ASNA). He pointed out that Asia accounts for 60 percent of the world population, but less than 20 percent of neurologists in the world, and stressed that education is the key to development of neurology in Asia. Next, Dr. Li-Ping Liu from China emphasized rapidly advancing frontiers of neuroscience research in China. At the last of this session, I gave a talk on neurology service in Asia. Asian countries are rapidly exploding in population and economy. Some are facing unique problems not experienced in other regions¹.

Figure 1. Projected increase of the aged (>65) populations among nations.
Adapted from Current Status and Predictions for an Aging Society with Fewer Children, Japanese Ministry of Education, Sports, Culture, Science and Technology (with permission).

Figure 1 depicts the projected increase of the population over 65 years among nations. While Western countries have a linear increase of the aged over years, Asian countries (Japan, Korea and China) have S-shaped curves indicating a steep surge of the aged population during 2000-2020 (Japan) and 2020-2030 (Korea and China). India will probably join this group by the end of this century.

Japan is the first to be exposed to this surge, and medical needs for the aged people are highlighted. Among these, neurological disorders such as stroke and Alzheimer’s disease came to the forefront. For instance, the number of stroke survivors has steeply increased from 1.7 million in 2000 to 2.8 million by 2013. Stroke was the No. 2 killer in the 1970s, but it is currently the fourth, following cancer, heart disease and pneumonia. Although the number of stroke attacks is six times as frequent as heart attack, it has largely become non-fatal, although it typically leaves disabilities; two- thirds of the patients are unable to return to pre-morbid activities.

From the global point of view, stroke is the second leading cause of death after ischemic heart disease, with an estimated 5.5 million people dying from stroke every year worldwide. Two thirds of these deaths occur in countries with limited resources². Approximately 80 percent of patients survive the acute phase of stroke, but are left with varying degrees of chronic disability.

Not only the number of deaths, but also the quality of life after stroke is an important aspect. Disease-adjusted life years (DALYs) are defined as number of years of healthy life lost by disease³. They reflect the impact of a disease in the aging societies.

Figure 2. Disease-adjusted life years (DALYs) of neurological diseases with respect to nation’s income status. Data from WHO (2005).

Figure 2 shows DALYs among various neurological diseases with respect to the economic status of a nation. Stroke is more important than Alzheimer’s disease, because many Asian nations are still in the state of low-middle income by World Bank Criteria. Japan had been among those with limited resources with high mortality of stroke, but her economy grew up in a short period to high income state, and other Asian nations should follow this path.

The drop of stroke mortality in Japan is due to change in diet, western lifestyle and the efficient social health care system. However, the main factor is the control of hypertension by medication, which decreased the number of fatal massive hemorrhages³. The impact of thrombolytic therapy is limited, rather increasing those disabled by preventing stroke deaths⁴.

The expense related to caring for stroke survivors is now exceeding $25 billion (U.S. dollars) per year in Japan. Now the Japanese economy is revolving around those aged patients and their care. In fact, a major diaper maker saw sales of adult diapers outpace infant diapers. Stroke centers with staff dedicated to thrombolytic therapy are urgently needed, and we are investing effort into increasing awareness of stroke among Asian people. Educating health professionals in neurology is the Asian Initiative’s first priority.

The roots of neurology lie in Europe and this specialty matured in the U.S. Asia has its own priorities in coping with neurological diseases. In this regard, I propose “autonomy” as a key word for activating regional neurological organizations. We need a forum to discuss the problems in each region, and to provide unique educational opportunities for neurologists, general practitioners, allied health professionals and the patients. With these aims in mind, the WFN has decided to make the best use of the existing regional neurological organizations such as AOAN to fulfill its mission.

Another key for success would be “synergy”. Symposia and workshops were held in collaboration with international organizations such as the Movement Disorder Society (MDS) and the International Federation of Clinical Neurophysiology (IFCN). These joint activities provided the financial support for the meeting and increased the attendance. The event also helped the supporting organizations increase their visibility. I hope that the March 2014 meeting in Macao becomes a model for the future meetings in Asia, and the meeting will serve as an equivalent to EFNS, ENS or American Academy of Neurology meetings.

Alzheimer’s disease is a little harder to tackle. Traditionally, Asian people had a large family, three generations living together. The aged people lived with younger family members. In my childhood in 1950s, the aged are naturally thought to have memory loss to some degree. It is still a virtue that children respect and take care of the parents by Asian standard. Re-appraisal of this system in the face of increasing Alzheimer’s disease might be a solution for countries with limited resources. For the aged, it would be appropriate to prepare for the intellectual decline.

Steve Jobs, the former CEO of Apple Computer, gave a speech at the commencement at Stanford in 2005. Facing the recurrence of cancer, his message on coping with imminent death might be a hint: in the morning he thought about his life as if ending in the evening. Whenever he thought the activities of the day were not what he really wanted to do, he changed his life. These were actually the words of the old Chinese philosopher, Confucius (551–479 BC), which he probably was familiar with. Alzheimer’s disease is still unpreventable and incurable probably for the next decades to come. “Think Oriental” might be the key for the societies and the neurological community in the world.

References

1. Kaji R Asian Neurology and Stroke (Perspectives) Neurology, in press

2. WHO, W. Neurology Atlas, (2003).

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Wolfgang Grisold

By Wolfgang Grisold

Earlier this year, the first Joint Congress of European Neurology took place in Istanbul. This was the final stage in the process of merging the two European neurological societies — the European Neurological Society (ENS) and the European Federation of Neurological Societies (EFNS) — into the European Academy of Neurology (EAN). The first elections of the new society were held during the congress, naming G. Deuschl, Germany, as the first president of the EAN, and F. Fazekas, Austria, as its first vice president. The merger of the two societies was a logical step and now that it has occurred, it seems to be a natural development, but it took a huge effort and the engagement of many individuals to make it possible.

The ENS was the first European neurological society, founded in 1986. It held its first congress in Nice in 1989. That congress was organized by G. Said.

Said along with his co-founders, P. K. Thomas and Anita Harding, were the heart of the ENS at its founding. Their idea was to create a European society based on individual membership, with a strong emphasis on science and a structure similar to that of the American Academy of Neurology (AAN). As a consequence of this philosophy, the ENS traditionally has been less engaged in political aspects of neurology, fostering individual rather than national representation. The ENS, apart from holding excellent congresses, increasingly engaged in education through teaching, scholarships and internationalsupport for countries in need. Its major publication was the Journal of Neurology, which was supplemented by informative newsletter that appeared regularly.

The EFNS was founded in Vienna in 1991 by Prof. Gerstenbrand who had a vision of creating a large European society based on prior activities in Eastern Europe, such as the International Danube Symposia and the proposed Pan-European Society of Neurology. The philosophy of the EFNS was federal in constitution and structure, where individual European countries were the constitutional members. This construction was effective. Since its founding, the EFNS has contributed greatly to European neurology. The EFNS sponsored a number of activities, many of which were originally conceived within the EFNS, promoting education, CME accreditation and supporting neurology in the former Eastern European countries, by teaching courses, holding lectures and helping in the establishment of national neurological organizations. The primary publication of the EFNS is the European Journal of Neurology, which will become the official journal of the EAN. Neuropennews is an online publication serving the purpose of a newsletter.

Due to differences in their philosophies, the two societies were effectively in competition, resulting in what many European neurologists concluded was an unnecessary duplication of effort. The initial event of their coming together was the creation of the European Board Examination in Neurology, where the examination’s creator, the UEMS – European Board of Neurology (UEMS/EBN) invited both societies to participate in its development. The presidents of the two organizations at that time — De Reuck of the EFNS and Moonen of the ENS — were both from Belgium. That facilitated the process of initiating a proposal for the creation of a joint EAN.

There were many additional steps necessary for the eventual merger. One major step was the agreement of the European delegates of the EFNS to this proposal in Florence 2009 and the subsequent agreement of ENS members. A joint task force was then formed that meticulously prepared the details for the merger and also created a new constitution and voting system.

The EAN combines the best of the developments of both societies. It is expected to foster neurology within Europe for the best of patient care, and to support and encourage science, teaching and education, as well as to be a transparent, open-minded and member-oriented society that provides an essential service for its members.

The WFN acknowledges this development toward a strong European society, and we hope to cooperate in further projects, especially where those involve furthering neurological education in countries in need of additional resources.