Neuro-Behçet’s disease

International research project

Most people reading this 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 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 Behçet’s UK 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