International research project
Most people reading this newsletter need no introduction to Behçet’s disease. Some of us are affected by this condition and others are involved in care of those who are. We know that Behçet’s disease can affect any part of the body. Involvement of the neurological system is called neuro-Behçet’s disease and is among the most disabling complications of Behçet’s disease. Thankfully, it is not that common and affects about 10% of people with Behçet’s disease.
To introduce ourselves, we work as neurologists. We are hospital specialists, and our job is to diagnose and treat people with neurological conditions. These can affect the brain, spine, nerves and/or muscles. We deal with neurological inflammatory conditions which can occur separately or in combination with inflammation of the other organs such as eyes or skin, as is the case in neuro-Behçet’s disease.
In this article we intend to discuss neuro-Behçet’s disease and how it can affect people. We would also like to s share with you some work we have recently completed in the form of an international consensus project on developing guidance on the diagnosis and treatment of neuro-Behçet’s disease.
How does neuro-Behçet’s disease present?
Neuro-Behçet’s disease can cause inflammation of the brain/spinal tissue directly, or can cause inflammation and blockage of the blood supplying them (usually veins rather than arteries).
If there is direct inflammation of the brain tissue, people can present with one feature or a combination of features including weakness, pins and needles, loss of co-ordination, double vision, confusion, fits and meningitis. On the other hand, if the blood vessels (veins) are inflamed and blocked, patients present with symptoms of raised intracranial pressure such as headache or blurred vision. They usually have some other background Behçet’s disease symptoms such as mouth ulcers, genital ulcers or eye symptoms. Patient with Behçet’s disease commonly have headaches. Headache can occur because of neuro-Behçet’s disease per se or because of other reasons such as migraines.
It is a neurologists’ job to look into these symptoms and decide what further tests or treatments are needed.
What are the tests and treatments required?
Specialist tests such as a brain scan or lumbar puncture might be required. Treatment depends on the pattern of presentation – that is, whether the brain tissue is inflamed or blood vessels are affected. Brain tissue inflammation is more difficult to treat, can recur and results in disability. An early and strong immune therapy is needed for a longer time. Neuro-Behçet’s with blood vessel involvement requires shorter-term treatment once the initial crisis is over.
Behçet’s disease experts differ in their approach to treatment based on their experience. There were no clear guidelines on the main issues in diagnosis and treatment of neuro-Behçet’s disease, so we set out to do this project.
International Neuro-Behçet’s Recommendations Project
We planned to develop clinical and practical guidelines on the key issues in the diagnosis and treatment of neuro-Behçet’s disease to assist clinicians involved in care of these patients. As Behçet’s disease can affect different organs at the same time, it was imperative that we involved specialists from different fields to cover the treatment in a holistic way.
It took us almost 3 years to complete this project. An advisory group made of 52 international Behçet’s disease experts from 20 countries and 11 different medical specialities, including 23 neurologists and voluntary patient representatives, worked together on this project. Jan Mather was the patient group representative from the Behçet’s Syndrome Society. Most of the UK specialists involved are also BSS members. A full list of group members is given at the end of the article.
We identified important clinical issues that needed addressing under the umbrella of this project. These covered diagnosis and treatment of neuro-Behçet’s disease. This was presented at the ICBD meeting in 2010 in London.
We looked at the previously published studies thoroughly and systematically. Conclusions were drawn from the literature and were discussed and voted on. Only high scoring recommendations that passed the consensus criteria were given out as International Consensus Recommendations. These were published recently in the Journal of Neurology. The full article can be accessed here.
This project brought up clear and easily usable diagnostic criteria for neuro-Behçet’s disease. We also made 16 practical clinical recommendations for diagnosis and treatment of neuro-Behçet’s disease. We hope that these will be a useful resource to the Behçet’s disease community.
We would like to thank all members of the advisory and consensus group members who helped towards this project. Special thanks go to Jan Mather for representing the patients on the Advisory Group. Without their help this project would not have been possible.
Seema Kalra, Clinical Research Fellow and Neurology Specialist Registrar
Adnan Al-Araji, Consultant Neurologist
University Hospital of North Staffordshire NHS Trust, Stoke on Trent
List of all advisory and consensus group members in alphabetical order
Prof Gulsen Akman-Demir, Neurology, Turkey
Prof Sarmed Al Fahad, Neurology, Dubai
Dr Adnan Al-Araji, Neurology, UK
Dr Andreas Altenburg, Dermatology, Germany
Prof Ayse Altintas, Neurology, Turkey
Dr Thurayya Arayssi, Rheumatology, Qatar
Prof Marcel Arnold, Neurology, Switzerland
Prof Samir Helmy Assaad-Khalil, Internal Medicine, Egypt
Dr John Bamford, Neurology, UK
Prof Eldad Ben-Chetrit, Rheumatology, Israel
Prof Peter Berlit, Neurology, Germany
Dr Saeed Bohlega, Neurology, Saudi Arabia
Prof Panagiota Boura, Internal Medicine & Clinical Immunology, Greece
Dr Kenneth T Calamia, Rheumatology, USA
Prof Simon Carette, Rheumatology, Canada
Prof Cris S Constantinescu, Neurology, UK
Prof Khaled Elmuntaser, Internal Medicine & Rheumatology, Libya
Dr Luis R Espinoza, Rheumatology, USA
Dr Oliver Findling, Neurology, Switzerland
Dr Loic Guillevin, Internal Medicine, France
Dr Afshin Borhani Haghighi, Neurology, Iran
Prof Dorian O Haskard, Rheumatology, UK
Prof Shunsei Hirohata, Rheumatology & Internal medicine, Japan
Prof Habib Houman, Internal Medicine & Immunology, Tunisia
Dr Seema Kalra, Neurology, UK
Dr Orhun H Kantarci, Neurology, USA
Dr Mary Keogan, Immunology, UK
Dr Desmond Kidd, Neurology, UK
Prof Isabelle Kone-Paut, Paediatrics, France
Dr Murat Kurtuncu, Neurology, Turkey
Dr Loredana La Mantia, Neurology/Neuropathology, Italy
Dr Wafa Madanat, Rheumatology, Jordan
Dr Alfred Mahr, Internal Medicine, France
Dr Sergio Martínez-Yélamos, Neurology, Spain
Ms Janet Mather, Patient representative, UK
Prof Philip I. Murray, Ophthalmology, UK
Dr Alex Olivé, Rhematology, Spain
Dr Ignazio Olivieri, Rheumatology, Italy
Dr Norberto Ortego-Centeno, Internal Medicine, Spain
Prof Salih Pay, Rheumatology & Internal medicine, Turkey
Dr Cristina Ramo, Neurology, Spain
Prof Sahar N Saleem, Neuro-radiology, Egypt
Dr Carlo Salvarani, Rheumatology, Italy
Prof Neil Scolding, Neurology, UK
Prof Petros Sfikakis, Internal Medicine, Greece
Prof Alan Silman, Rheumatology, UK
Prof Aksel Siva, Neurology, Turkey
Dr Roser Solans-Laqué, Internal Medicine & Autoimmune Diseases, Spain
Dr Thomas Stache, Neurology, Germany
Prof Miles Stanford, Ophthalmology, UK
Dr Nadera J Sweiss, Medicine, USA
Dr Jan VanLaar, Internal Medicine/Immunology, Netherlands
Dr Nagagopal Venna, Neurology, USA