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      EAN News

      Plans in progress for 30th Anniversary of the European Journal of Neurology (1994-2024)

      December 6, 2023

      Advocacy

      Executive Page: European neurology united as ever heading into milestone year

      December 6, 2023

      Paper of the Month

      Research Paper of the Month: Apixaban for Stroke Prevention in Subclinical Atrial Fibrillation

      December 4, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – December 2023

      December 1, 2023

      Executive Page

      Executive Page: European neurology united as ever heading into milestone year

      December 6, 2023

      Executive Page

      Executive Page: Join or renew your membership now and celebrate 10 years of the EAN

      November 6, 2023

      Executive Page

      Executive Page: Advocacy for brain health gaining momentum across the neurological landscape

      October 1, 2023

      Executive Page Dear EAN members, dear friends, It is my pleasure to give you an update on behalf of the EAN Scientific Committee following a busy year since my appointment as chair. It has been a privilege to be so deeply involved in the scientific activities of the EAN and to work closely with an amazing community of people who are so dedicated to the advancement of neurology. One of the strongest threads within that community is the EAN’s group of Scientific Panels, which are often referred to as the organisation’s ‘scientific backbone’. The panels have a hand in everything the EAN does from a scientific point of view, including coordinating clinical research, disseminating good practice, assisting in planning the EAN annual congress, supporting the EAN’s educational efforts, and producing guidelines that assist neurologists in daily practice. All of this work requires a considerable amount of oversight, and this is why each panel is run by its own Management Group, elected by the panel members. Being a part of one of these Management Groups (PDF) is an opportunity to play a direct part in determining the direction of scientific progress within Europe, and I am excited to say that the call for applications to stand for election will be announced in September. All panel members and EAN individual members are eligible to apply to be candidates, with the vote being held among the existing panel members in autumn. I would like to urge every neurologist with a passion for the scientific future of their special area of interest to consider standing for election to the relevant Management Group and to look out for the call for applications later this month. I can tell you from personal experience that fulfilling a role such as this within the structure of the EAN is a fascinating and extremely rewarding experience. The chance to take on such a unique leadership position should not be missed! The most satisfying aspect of being involved in the EAN’s organisational structure is surely seeing all the hard work and planning come to fruition when a small idea evolves into a newly launched project. One such example is the 1st EAN Science School, taking place in Salzburg next March. The idea of the Science School is to offer early career neurologists with an interest in translational research a new avenue for learning about basic pathomechanisms of neurological disorders. Application for this very first edition, on ‘Pathophysiology of disorders of the nervous system’, closed last week and we have been delighted with the level of interest. It was a pleasure to be part of the task force in charge of planning this new project and I look forward to seeing it officially transferred to the hands of the Scientific Committee after a successful event and evaluation in spring. Of our ongoing activities, one of the most significant is the EAN Neuro-covid Registry, which aims to collate epidemiological data on neurological manifestations in patients with COVID-19 infection reported by neurologists in outpatient services, emergency rooms, and hospital departments. We currently have 1,542 patients entered in the database, from 35 centres across 23 countries. So far, 29 patients have been followed for 12 months and 387 have completed the 6-months follow up. This is a decent start, but I would like to strongly encourage EAN members and their institutions to get involved and contribute cases to what will become an invaluable resource for neurologists and clinicians to improve care for Covid-19 patients. Finally, I would like share a word on a forthcoming collaboration between EAN and the Human Brain Project in December, that I think will interest a lot of EAN members. The EAN-EBRAINS joint workshop on ‘The future of medical data sharing in clinical neurosciences’ will be a three-day virtual event that aims to discuss issues and challenges associated with data sharing in Europe, from ethics to data safety and privacy, including those specific to data federation, such as the development and validation of federated algorithms. The whole workshop is completely free of charge and registration is open until 25 November, so please check out the details and sign up! I wish you all a healthy and pleasant autumn and I look forward to updating you again. Best wishes Thomas Berger Chair of the EAN Scientific Committee

      Executive Page: Management Group elections are coming – run for office and help shape our future!

      September 1, 2023

      EAN News

      Plans in progress for 30th Anniversary of the European Journal of Neurology (1994-2024)

      December 6, 2023

      EAN News

      Executive Page: European neurology united as ever heading into milestone year

      December 6, 2023

      EAN News

      Research Paper of the Month: Apixaban for Stroke Prevention in Subclinical Atrial Fibrillation

      December 4, 2023

      EAN News One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – December 2023

      December 1, 2023

      EAN Congress news

      EAN 2024 – Abstract submission and registration OPEN!

      November 22, 2023

      EAN Congress news

      Burden of Sleep/Wake Disorders Project at EAN Congress 2023

      October 19, 2023

      EAN Congress news

      African Academy of Neurology features in Special Studio Session at EAN 2023

      July 25, 2023

      EAN Congress news

      Find photos, facts, figures and reports in our EAN Congress Review

      July 24, 2023

      Interviews

      Video Interview: Prof. Mary Reilly, Moritz Romberg lecturer at EAN 2023

      July 4, 2023

      Interviews

      Video Interview: Mark Hallett, C. Brown-Séquard lecturer at EAN 2023

      July 3, 2023

      Interviews

      Video Interview: Josep Dalmau, Camillo Golgi Lecturer at EAN 2023

      July 2, 2023

      Interviews

      Video Interview: Dementia & Cognitive Disorders Panel Co-Chairs

      September 8, 2022

  • Research
    • All Paper of the Month Research Highlights
      Paper of the Month

      Research Paper of the Month: Apixaban for Stroke Prevention in Subclinical Atrial Fibrillation

      December 4, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – December 2023

      December 1, 2023

      Paper of the Month

      Research Paper of the Month: Effects of oral anticoagulation in people with atrial fibrillation after spontaneous intracranial haemorrhage (COCROACH)

      November 6, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – November 2023

      November 6, 2023

      Paper of the Month

      Research Paper of the Month: Apixaban for Stroke Prevention in Subclinical Atrial Fibrillation

      December 4, 2023

      Paper of the Month

      Research Paper of the Month: Effects of oral anticoagulation in people with atrial fibrillation after spontaneous intracranial haemorrhage (COCROACH)

      November 6, 2023

      Paper of the Month

      Research Paper of the Month: Transdermal Nicotine Treatment and Progression of Early Parkinson’s Disease

      October 1, 2023

      Paper of the Month

      Research Paper of the Month: Soluble Nogo-Receptor-Fc decoy (AXER-204) in patients with chronic cervical spinal cord injury in the USA

      September 1, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – December 2023

      December 1, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – November 2023

      November 6, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – October 2023

      October 1, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – September 2023

      September 1, 2023

  • Education
    • All eLearning Student Corner Resident and Research Fellows Fellowship reports
      eLearning

      End 2023 with highlighted content on the eanCampus!

      December 2, 2023

      Resident and Research Fellows

      EAN Spring School 2024 – Apply NOW until 31 January 2024

      December 1, 2023

      Resident and Research Fellows

      EAN Autumn School 2023: Report

      November 30, 2023

      eLearning

      Call for Applications: e-Learning Platform Associate Editors

      November 7, 2023

      eLearning

      End 2023 with highlighted content on the eanCampus!

      December 2, 2023

      eLearning

      Call for Applications: e-Learning Platform Associate Editors

      November 7, 2023

      eLearning

      Kick off the new season with highlights on the eanCampus

      October 13, 2023

      eLearning

      World Brain Day with the European Academy of Neurology

      July 21, 2023

      Student Corner

      Student Teaser Fellowship reports 2022 – Pisa, London, & Lausanne

      September 13, 2023

      Student Corner Illustration of a vertical pencil point on a blue background, with a yellow light bulb above it

      Student Corner: My EAN Congress experience

      September 5, 2023

      Student Corner

      Student Teaser Fellowship reports 2022 – Rotterdam, London, & Paris

      August 22, 2023

      Student Corner

      Student Teaser Fellowship winners 2023

      June 28, 2023

      Resident and Research Fellows

      EAN Spring School 2024 – Apply NOW until 31 January 2024

      December 1, 2023

      Resident and Research Fellows

      EAN Autumn School 2023: Report

      November 30, 2023

      Resident and Research Fellows

      Research Fellowship Report 2022 – Oxford Autoimmune Neurology Group

      September 16, 2023

      Resident and Research Fellows

      Research Fellowship Report 2022 – Centre Hospitalier Universitaire Vaudois, Lausanne

      September 15, 2023

      Fellowship reports

      Research Fellowship Report 2022 – Oxford Autoimmune Neurology Group

      September 16, 2023

      Fellowship reports

      Research Fellowship Report 2022 – Centre Hospitalier Universitaire Vaudois, Lausanne

      September 15, 2023

      Fellowship reports

      Research Fellowship Report 2022 – Department of Neuromuscular Diseases Institute, University College London

      September 14, 2023

      Fellowship reports

      Student Teaser Fellowship reports 2022 – Pisa, London, & Lausanne

      September 13, 2023

  • Other News
    • All EAN Staff EBC News in general Surveys
      EAN Staff

      EAN Head Office Profiles: Ulla & Piyapat

      December 1, 2023

      EAN Staff

      EAN Head Office Profiles: Maria & Immanuel

      November 7, 2023

      Advocacy

      Brain community launches Call to Action during Brain Health & Research Day at UNGA78

      October 19, 2023

      EAN News

      Brain Innovation Days 2023: Meet the speakers!

      October 2, 2023

      EAN Staff

      EAN Head Office Profiles: Ulla & Piyapat

      December 1, 2023

      EAN Staff

      EAN Head Office Profiles: Maria & Immanuel

      November 7, 2023

      EAN Staff

      EAN Head Office Profiles: Kathrin & Sasha

      August 28, 2023

      EAN Staff Anja Sander receiving flowers at the end of EAN Congress 2023

      Celebrating 20 Years of Excellence: Anja Sander, EAN’s Executive Director!

      August 3, 2023

      EBC

      Brain community launches Call to Action during Brain Health & Research Day at UNGA78

      October 19, 2023

      EBC

      Brain Innovation Days 2023: Meet the speakers!

      October 2, 2023

      EBC

      Brain Innovation Days: Discover the early programme and register today!

      July 21, 2023

      EBC

      Join the community’s calls for a European Brain Research and Innovation Plan

      July 21, 2023

      News in general

      WHO’s Intersectoral Global Action Plan on epilepsy and other neurological disorders (IGAP) published in all six UN languages

      August 3, 2023

      News in general

      WHO recognises the public health need for effective and affordable treatments for multiple sclerosis

      August 3, 2023

      News in general

      Brain Innovation Days: Discover the early programme and register today!

      July 21, 2023

      News in general

      Join the community’s calls for a European Brain Research and Innovation Plan

      July 21, 2023

      Surveys

      Neurorehabilitation Across Europe after Ischemic and Traumatic Brain Injuries: a survey to increase knowledge on brain-injury-specific neurorehabilitation

      July 14, 2023

      Surveys

      ILAE survey on genetic testing in the epilepsies

      July 14, 2023

      Surveys

      COVID-19 and neurology: a survey of previous and continued restrictions on clinical practice, professional education and neuroeconomics

      March 3, 2023

      Surveys

      Help EAN contribute towards the development of a European Pain Research strategy

      December 5, 2022

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eanpages

  • EAN News
    • All Executive Page EAN News EAN Congress news Interviews
      EAN News

      Plans in progress for 30th Anniversary of the European Journal of Neurology (1994-2024)

      December 6, 2023

      Advocacy

      Executive Page: European neurology united as ever heading into milestone year

      December 6, 2023

      Paper of the Month

      Research Paper of the Month: Apixaban for Stroke Prevention in Subclinical Atrial Fibrillation

      December 4, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – December 2023

      December 1, 2023

      Executive Page

      Executive Page: European neurology united as ever heading into milestone year

      December 6, 2023

      Executive Page

      Executive Page: Join or renew your membership now and celebrate 10 years of the EAN

      November 6, 2023

      Executive Page

      Executive Page: Advocacy for brain health gaining momentum across the neurological landscape

      October 1, 2023

      Executive Page Dear EAN members, dear friends, It is my pleasure to give you an update on behalf of the EAN Scientific Committee following a busy year since my appointment as chair. It has been a privilege to be so deeply involved in the scientific activities of the EAN and to work closely with an amazing community of people who are so dedicated to the advancement of neurology. One of the strongest threads within that community is the EAN’s group of Scientific Panels, which are often referred to as the organisation’s ‘scientific backbone’. The panels have a hand in everything the EAN does from a scientific point of view, including coordinating clinical research, disseminating good practice, assisting in planning the EAN annual congress, supporting the EAN’s educational efforts, and producing guidelines that assist neurologists in daily practice. All of this work requires a considerable amount of oversight, and this is why each panel is run by its own Management Group, elected by the panel members. Being a part of one of these Management Groups (PDF) is an opportunity to play a direct part in determining the direction of scientific progress within Europe, and I am excited to say that the call for applications to stand for election will be announced in September. All panel members and EAN individual members are eligible to apply to be candidates, with the vote being held among the existing panel members in autumn. I would like to urge every neurologist with a passion for the scientific future of their special area of interest to consider standing for election to the relevant Management Group and to look out for the call for applications later this month. I can tell you from personal experience that fulfilling a role such as this within the structure of the EAN is a fascinating and extremely rewarding experience. The chance to take on such a unique leadership position should not be missed! The most satisfying aspect of being involved in the EAN’s organisational structure is surely seeing all the hard work and planning come to fruition when a small idea evolves into a newly launched project. One such example is the 1st EAN Science School, taking place in Salzburg next March. The idea of the Science School is to offer early career neurologists with an interest in translational research a new avenue for learning about basic pathomechanisms of neurological disorders. Application for this very first edition, on ‘Pathophysiology of disorders of the nervous system’, closed last week and we have been delighted with the level of interest. It was a pleasure to be part of the task force in charge of planning this new project and I look forward to seeing it officially transferred to the hands of the Scientific Committee after a successful event and evaluation in spring. Of our ongoing activities, one of the most significant is the EAN Neuro-covid Registry, which aims to collate epidemiological data on neurological manifestations in patients with COVID-19 infection reported by neurologists in outpatient services, emergency rooms, and hospital departments. We currently have 1,542 patients entered in the database, from 35 centres across 23 countries. So far, 29 patients have been followed for 12 months and 387 have completed the 6-months follow up. This is a decent start, but I would like to strongly encourage EAN members and their institutions to get involved and contribute cases to what will become an invaluable resource for neurologists and clinicians to improve care for Covid-19 patients. Finally, I would like share a word on a forthcoming collaboration between EAN and the Human Brain Project in December, that I think will interest a lot of EAN members. The EAN-EBRAINS joint workshop on ‘The future of medical data sharing in clinical neurosciences’ will be a three-day virtual event that aims to discuss issues and challenges associated with data sharing in Europe, from ethics to data safety and privacy, including those specific to data federation, such as the development and validation of federated algorithms. The whole workshop is completely free of charge and registration is open until 25 November, so please check out the details and sign up! I wish you all a healthy and pleasant autumn and I look forward to updating you again. Best wishes Thomas Berger Chair of the EAN Scientific Committee

      Executive Page: Management Group elections are coming – run for office and help shape our future!

      September 1, 2023

      EAN News

      Plans in progress for 30th Anniversary of the European Journal of Neurology (1994-2024)

      December 6, 2023

      EAN News

      Executive Page: European neurology united as ever heading into milestone year

      December 6, 2023

      EAN News

      Research Paper of the Month: Apixaban for Stroke Prevention in Subclinical Atrial Fibrillation

      December 4, 2023

      EAN News One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – December 2023

      December 1, 2023

      EAN Congress news

      EAN 2024 – Abstract submission and registration OPEN!

      November 22, 2023

      EAN Congress news

      Burden of Sleep/Wake Disorders Project at EAN Congress 2023

      October 19, 2023

      EAN Congress news

      African Academy of Neurology features in Special Studio Session at EAN 2023

      July 25, 2023

      EAN Congress news

      Find photos, facts, figures and reports in our EAN Congress Review

      July 24, 2023

      Interviews

      Video Interview: Prof. Mary Reilly, Moritz Romberg lecturer at EAN 2023

      July 4, 2023

      Interviews

      Video Interview: Mark Hallett, C. Brown-Séquard lecturer at EAN 2023

      July 3, 2023

      Interviews

      Video Interview: Josep Dalmau, Camillo Golgi Lecturer at EAN 2023

      July 2, 2023

      Interviews

      Video Interview: Dementia & Cognitive Disorders Panel Co-Chairs

      September 8, 2022

  • Research
    • All Paper of the Month Research Highlights
      Paper of the Month

      Research Paper of the Month: Apixaban for Stroke Prevention in Subclinical Atrial Fibrillation

      December 4, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – December 2023

      December 1, 2023

      Paper of the Month

      Research Paper of the Month: Effects of oral anticoagulation in people with atrial fibrillation after spontaneous intracranial haemorrhage (COCROACH)

      November 6, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – November 2023

      November 6, 2023

      Paper of the Month

      Research Paper of the Month: Apixaban for Stroke Prevention in Subclinical Atrial Fibrillation

      December 4, 2023

      Paper of the Month

      Research Paper of the Month: Effects of oral anticoagulation in people with atrial fibrillation after spontaneous intracranial haemorrhage (COCROACH)

      November 6, 2023

      Paper of the Month

      Research Paper of the Month: Transdermal Nicotine Treatment and Progression of Early Parkinson’s Disease

      October 1, 2023

      Paper of the Month

      Research Paper of the Month: Soluble Nogo-Receptor-Fc decoy (AXER-204) in patients with chronic cervical spinal cord injury in the USA

      September 1, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – December 2023

      December 1, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – November 2023

      November 6, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – October 2023

      October 1, 2023

      Research Highlights One glowing light bulb that stands out from unlit or dim lamps on a blue background

      Research Highlights of the Month – September 2023

      September 1, 2023

  • Education
    • All eLearning Student Corner Resident and Research Fellows Fellowship reports
      eLearning

      End 2023 with highlighted content on the eanCampus!

      December 2, 2023

      Resident and Research Fellows

      EAN Spring School 2024 – Apply NOW until 31 January 2024

      December 1, 2023

      Resident and Research Fellows

      EAN Autumn School 2023: Report

      November 30, 2023

      eLearning

      Call for Applications: e-Learning Platform Associate Editors

      November 7, 2023

      eLearning

      End 2023 with highlighted content on the eanCampus!

      December 2, 2023

      eLearning

      Call for Applications: e-Learning Platform Associate Editors

      November 7, 2023

      eLearning

      Kick off the new season with highlights on the eanCampus

      October 13, 2023

      eLearning

      World Brain Day with the European Academy of Neurology

      July 21, 2023

      Student Corner

      Student Teaser Fellowship reports 2022 – Pisa, London, & Lausanne

      September 13, 2023

      Student Corner Illustration of a vertical pencil point on a blue background, with a yellow light bulb above it

      Student Corner: My EAN Congress experience

      September 5, 2023

      Student Corner

      Student Teaser Fellowship reports 2022 – Rotterdam, London, & Paris

      August 22, 2023

      Student Corner

      Student Teaser Fellowship winners 2023

      June 28, 2023

      Resident and Research Fellows

      EAN Spring School 2024 – Apply NOW until 31 January 2024

      December 1, 2023

      Resident and Research Fellows

      EAN Autumn School 2023: Report

      November 30, 2023

      Resident and Research Fellows

      Research Fellowship Report 2022 – Oxford Autoimmune Neurology Group

      September 16, 2023

      Resident and Research Fellows

      Research Fellowship Report 2022 – Centre Hospitalier Universitaire Vaudois, Lausanne

      September 15, 2023

      Fellowship reports

      Research Fellowship Report 2022 – Oxford Autoimmune Neurology Group

      September 16, 2023

      Fellowship reports

      Research Fellowship Report 2022 – Centre Hospitalier Universitaire Vaudois, Lausanne

      September 15, 2023

      Fellowship reports

      Research Fellowship Report 2022 – Department of Neuromuscular Diseases Institute, University College London

      September 14, 2023

      Fellowship reports

      Student Teaser Fellowship reports 2022 – Pisa, London, & Lausanne

      September 13, 2023

  • Other News
    • All EAN Staff EBC News in general Surveys
      EAN Staff

      EAN Head Office Profiles: Ulla & Piyapat

      December 1, 2023

      EAN Staff

      EAN Head Office Profiles: Maria & Immanuel

      November 7, 2023

      Advocacy

      Brain community launches Call to Action during Brain Health & Research Day at UNGA78

      October 19, 2023

      EAN News

      Brain Innovation Days 2023: Meet the speakers!

      October 2, 2023

      EAN Staff

      EAN Head Office Profiles: Ulla & Piyapat

      December 1, 2023

      EAN Staff

      EAN Head Office Profiles: Maria & Immanuel

      November 7, 2023

      EAN Staff

      EAN Head Office Profiles: Kathrin & Sasha

      August 28, 2023

      EAN Staff Anja Sander receiving flowers at the end of EAN Congress 2023

      Celebrating 20 Years of Excellence: Anja Sander, EAN’s Executive Director!

      August 3, 2023

      EBC

      Brain community launches Call to Action during Brain Health & Research Day at UNGA78

      October 19, 2023

      EBC

      Brain Innovation Days 2023: Meet the speakers!

      October 2, 2023

      EBC

      Brain Innovation Days: Discover the early programme and register today!

      July 21, 2023

      EBC

      Join the community’s calls for a European Brain Research and Innovation Plan

      July 21, 2023

      News in general

      WHO’s Intersectoral Global Action Plan on epilepsy and other neurological disorders (IGAP) published in all six UN languages

      August 3, 2023

      News in general

      WHO recognises the public health need for effective and affordable treatments for multiple sclerosis

      August 3, 2023

      News in general

      Brain Innovation Days: Discover the early programme and register today!

      July 21, 2023

      News in general

      Join the community’s calls for a European Brain Research and Innovation Plan

      July 21, 2023

      Surveys

      Neurorehabilitation Across Europe after Ischemic and Traumatic Brain Injuries: a survey to increase knowledge on brain-injury-specific neurorehabilitation

      July 14, 2023

      Surveys

      ILAE survey on genetic testing in the epilepsies

      July 14, 2023

      Surveys

      COVID-19 and neurology: a survey of previous and continued restrictions on clinical practice, professional education and neuroeconomics

      March 3, 2023

      Surveys

      Help EAN contribute towards the development of a European Pain Research strategy

      December 5, 2022

Forum

Neurological News from Turkey – the land of EFNS/ENS 2014: Behçet’s disease

June 1, 2014

by Aksel Siva

Behçet’s disease (BD), originally described in 1937 by the Turkish dermatologist Hulusi Behçet, as a distinct disease with oro-genital ulceration and uveitis known as the “triple-symptom complex”, is an idiopathic chronic relapsing multisystem vascular-inflammatory disease of unknown origin. The disease affects many organs and systems, causing mucocutaneous lesions, eye inflammation, musculoskeletal problems, and major vessel disease; there is cardiac, pulmonary, and gastrointestinal involvement as well as nervous system involvement.

Due to the lack of specific laboratory, radiologic, or histologic findings of BD, accurate diagnosis of BD depends only on clinical findings. According to the International Study Group’s classification criteria, a diagnosis of BD requires recurrent oral aphthous ulcerations plus two of the following: genital ulcerations, skin lesions, eye lesions, or a positive pathergy test.

The epidemiology of the disease shows a geographical variation, seen more commonly among the Silk Route countries that extend from the Mediterranean region to Japan. This is coupled by a similar variation in HLA-B51, which is strongly associated with the disease in high prevalence areas. The prevelance of BD is highest in Turkey (1/250). Its prevalence has been reported to be less than 1/105 in Northern and Central Europe and the US, and goes up to 2.5/105 in the north-western Mediterranean region, and then increases considerably in the Eastern Mediterranean region.

The usual onset of the BD is in the third or fourth decade; however, although rare, onset in children has also been reported. BD shows equal frequency among males and females. However males have a more severe course due to the morbidity of pulmonary vascular diseases.

The etiology of BD is unknown, but clinical and laboratory data suggest that there is dysfunction of both innate and adaptive immune systems, resulting in an exaggerated response to viral or bacterial insults. Debate is ongoing about whether this hyperreactivity is an autoimmune phenomenon, or — as suggested by more recent data — an autoinflammatory phenomenon. The core histopathologic phenomenon seems to be a vasculitic involvement in some cases and a low-grade, chronic, nonspecific inflammation in others.

NEURO-BEHÇET SYNDROME

Primary neurological involvement is named as Neuro-Behcet’s Syndrome (NBS) and occurs approximately in 5-10% in all patients with BD. The onset of neurologic involvement is about 3-6 years after the diagnosis of the systemic disease and NBS is 3–4 times more frequent in males than women. However, although extremely rare, onset with neurological involvement prior to the systemic features of BD have been reported.

The suggested diagnostic criteria for NBS in a patient that fulfills the International Diagnostic Criteria for Behçet’s Syndrome is the occurrence of neurological symptoms not otherwise explained by any other known systemic or neurological disease or treatment, and in whom objective abnormalities consistent with NBS are detected either on neurological examination, and/or with neuroimaging studies (magnetic resonance imaging) and/or abnormal cerebrospinal fluid examination.

Parenchymal syndrome – Intra-axial NBS: The majority of patients with neurologic involvement due to BS present with parenchymal CNS involvement, most commonly affecting the brainstem-diencephalic region. This pattern of involvement is seen in up to 70% to 80% of the patients with neurologic involvement and is termed “parenchymal Neuro-Behçet’s Syndrome” (pNBS) or “intra-axial NBS.” These patients present with a subacute (or rarely, acute) onset of severe headache, dysarthria, ataxia, and hemiparesis. Cognitive-behavioral changes, emotional lability, a self limited or progressive myelopathy, urinary sphincter dysfunction and to a lesser extent other CNS manifestations such as extrapyramidal signs and seizures have been reported. Isolated optic neuritis are rare in BS, and most visual symptoms in BS are due to ocular inflamation. Onset of pNBS is usually associated with a flaring of the systemic features of BD. Some patients may have only a single attack, with or without residual neurologic deficits, but most will have recurrences and some will have secondary progression. pNBS is usually associated with a poor prognosis, with approximately 50% of the patients becoming severely disabled within 10 years.

Cranial MRI will show an almost stereotypical edematous lesion involving brainstem, mainly the midbrain and upper pons, and extending to the diencephalon and basal ganglia; it appears hyperintense on T2 sections and hypo/iso-hypointense in T1 sections; usually there is a much smaller area of enhancement, and occasionally small hemorrhages can be seen. These lesions regress overtime but enlargement of the third ventricule and brainstem atrophy may develop. Rarely, subcortical or periventricular white matter lesions can be seen, which may be difficult to distinguish from other vasculitic lesions or multiple sclerosis. When there is spinal cord involvement, it may tend to be longitudinally extensive but the AQP4 antibodies are absent.

The systemic arterial involvement is infrequent in BD, and so is cranio-cervical arterial involvement, but there are a number of case reports with bilateral internal carotid artery occlusion, vertebral artery dissection, intracranial aneurysms and intracranial arteritis.

A neurobehavioral syndrome, which consists of euphoria, loss of insight, disinhibition, psychomotor agitation or retardation, with paranoid attitudes and obsessive concerns may develop in some patients with or without other neurological symptoms of NBS which is named as “Neuro-Psycho-Behçet Syndrome”.

Non-parenchymal syndrome – extra-axial NBS: The second most common form of neurologic involvement is cerebral venous sinus thrombosis (CVST), which may be seen in up to 20% of the patients with neurologic involvement. This is termed “vascular- NBS” or “extra-axial NBS”. Clinical manifestations resulting from thrombosis of the intracranial venous system vary according to the site and rate of venous occlusion and its extent.

CVST in BD evolves gradually so that a fulminating syndrome with violent headache, convulsions, paralysis, and coma is uncommon. Papilledema and sixth nerve paresis are the most common signs reported. Interestingly, in the pediatric age group affected with BD, the neurologic involvement is early and mostly in the form of CVST. A close association between CVST and systemic major vessel disease in BD has been reported. Neurologic disease in the form of CVST has a better neurologic prognosis than pNBS. CVST is often visible on MRI and MRV and cerebral angiography is not indicated. A spinal tap will reveal increased cerebrospinal fluid (CSF) pressure, but the cellular and chemical composition mostly is normal.

These two types of involvement occur in the same individual very rarely, and presumably have a different pathogenesis.

Headache: Headache is the most common neurologic symptom seen in BD and may be a manifestation of both forms of NBS, or may be in association with ocular inflammation. The prevalence of co-exiting primary headaches are no less than the general population. However, a paroxysmal migraine-like pain occurring with exacerbation of the systemic features of the disease may also be seen, which may be explained by a vascular headache triggered by the immunomediated disease activity in susceptible individuals.

Peripheral nervous system involvement: Primary peripheral nervous system (PNS) involvement is extremely rare in BD and when seen secondary polyneuropathy as a side effect of treatment should be considered.

Treatment of parenchymal NBS: There are no controlled data for the management of neurologic involvement and treatment strategies mostly currently depends on reports based on the clinical experience of neurologists.

High-dose intravenous methylprednisolone (IVMP) for up to 10 days, which is followed by either a slow oral tapering, is the first choice for treating acute relapses. In pNBS azathioprine is the most commonly used agent in the long-term treatment, however with limited efficacy but better risk/benefit profile than many other immunosuressants. Currently a growing number of case reports are suggestive that infliximab alone or combined with immunosuppressive therapies could be useful in preventing disease progression and providing clinical improvement in NBS patients who are resistant to conventional immunosuppressive treatment.

CVST is usually treated with either high-dose or medium-dose steroids because it is accepted that the clot formation in the veins is caused by a low-grade endothelial inflammation rather than by hypercoagulability. The use of anticoagulation in CVST however, remains controversial.

Aksel Siva is Professor of Neurology at the Department of Neurology, Istanbul University, Cerrahpaşa School of Medicine Cerrahpaşa in Istanbul, Turkey.
Together with Ugur Uygunoglu, MD and Sabahattin Saip, MD Prof he leads the neurology team of the I.U.Cerrahpaşa School of Medicine Behçet’s Disease Research Center Study Group.

Currently (May 2014) there are records of about 10.000 patients with BS who are registered at the multidisciplinary outpatient clinic at Cerrahpasa Medical Faculty. This dedicated clinic was founded in 1977 by Prof. Hasan Yazici and had been the first of its kind both locally and internationally. The clinic has been held once a week every Monday since its inception. All patients are initially evaluated by a rheumatologist, dermatologist, and ophthalmologist. The patients are referred to the departments of neurology, pulmonary disease, gastroenterology, and vascular surgery when necessary, and all management problems are discussed in a joint meeting of the various disciplines during the same day the patients are seen.

The information presented in this article is based on the following chapter:
Saip S, Akman-Demir G, Siva A. Neuro-Behçet syndrome. Handb Clin Neurol. 2014;121:1703-23.

Neurological News from Turkey – the land of EFNS/ENS 2014: Behçet’s disease was last modified: November 14th, 2018 by Editor
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